A form of CARDIAC MUSCLE disease that is characterized by ventricular dilation, VENTRICULAR DYSFUNCTION, and HEART FAILURE. Risk factors include SMOKING; ALCOHOL DRINKING; HYPERTENSION; INFECTION; PREGNANCY; and mutations in the LMNA gene encoding LAMIN TYPE A, a NUCLEAR LAMINA protein.
A form of CARDIAC MUSCLE disease, characterized by left and/or right ventricular hypertrophy (HYPERTROPHY, LEFT VENTRICULAR; HYPERTROPHY, RIGHT VENTRICULAR), frequent asymmetrical involvement of the HEART SEPTUM, and normal or reduced left ventricular volume. Risk factors include HYPERTENSION; AORTIC STENOSIS; and gene MUTATION; (FAMILIAL HYPERTROPHIC CARDIOMYOPATHY).
A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).
A form of CARDIAC MUSCLE disease in which the ventricular walls are excessively rigid, impeding ventricular filling. It is marked by reduced diastolic volume of either or both ventricles but normal or nearly normal systolic function. It may be idiopathic or associated with other diseases (ENDOMYOCARDIAL FIBROSIS or AMYLOIDOSIS) causing interstitial fibrosis.
A transient left ventricular apical dysfunction or ballooning accompanied by electrocardiographic (ECG) T wave inversions. This abnormality is associated with high levels of CATECHOLAMINES, either administered or endogenously secreted from a tumor or during extreme stress.
An autosomal dominant inherited form of HYPERTROPHIC CARDIOMYOPATHY. It results from any of more than 50 mutations involving genes encoding contractile proteins such as VENTRICULAR MYOSINS; cardiac TROPONIN T; ALPHA-TROPOMYOSIN.
A disease of the CARDIAC MUSCLE developed subsequent to the initial protozoan infection by TRYPANOSOMA CRUZI. After infection, less than 10% develop acute illness such as MYOCARDITIS (mostly in children). The disease then enters a latent phase without clinical symptoms until about 20 years later. Myocardial symptoms of advanced CHAGAS DISEASE include conduction defects (HEART BLOCK) and CARDIOMEGALY.
The muscle tissue of the HEART. It is composed of striated, involuntary muscle cells (MYOCYTES, CARDIAC) connected to form the contractile pump to generate blood flow.
Disease of CARDIAC MUSCLE resulting from chronic excessive alcohol consumption. Myocardial damage can be caused by: (1) a toxic effect of alcohol; (2) malnutrition in alcoholics such as THIAMINE DEFICIENCY; or (3) toxic effect of additives in alcoholic beverages such as COBALT. This disease is usually manifested by DYSPNEA and palpitations with CARDIOMEGALY and congestive heart failure (HEART FAILURE).
Ultrasonic recording of the size, motion, and composition of the heart and surrounding tissues. The standard approach is transthoracic.
A congenital cardiomyopathy that is characterized by infiltration of adipose and fibrous tissue into the RIGHT VENTRICLE wall and loss of myocardial cells. Primary injuries usually are at the free wall of right ventricular and right atria resulting in ventricular and supraventricular arrhythmias.
Diabetes complications in which VENTRICULAR REMODELING in the absence of CORONARY ATHEROSCLEROSIS and hypertension results in cardiac dysfunctions, typically LEFT VENTRICULAR DYSFUNCTION. The changes also result in myocardial hypertrophy, myocardial necrosis and fibrosis, and collagen deposition due to impaired glucose tolerance.
Recording of the moment-to-moment electromotive forces of the HEART as projected onto various sites on the body's surface, delineated as a scalar function of time. The recording is monitored by a tracing on slow moving chart paper or by observing it on a cardioscope, which is a CATHODE RAY TUBE DISPLAY.
The hemodynamic and electrophysiological action of the left HEART VENTRICLE. Its measurement is an important aspect of the clinical evaluation of patients with heart disease to determine the effects of the disease on cardiac performance.
Inflammatory processes of the muscular walls of the heart (MYOCARDIUM) which result in injury to the cardiac muscle cells (MYOCYTES, CARDIAC). Manifestations range from subclinical to sudden death (DEATH, SUDDEN). Myocarditis in association with cardiac dysfunction is classified as inflammatory CARDIOMYOPATHY usually caused by INFECTION, autoimmune diseases, or responses to toxic substances. Myocarditis is also a common cause of DILATED CARDIOMYOPATHY and other cardiomyopathies.
A condition in which the LEFT VENTRICLE of the heart was functionally impaired. This condition usually leads to HEART FAILURE; MYOCARDIAL INFARCTION; and other cardiovascular complications. Diagnosis is made by measuring the diminished ejection fraction and a depressed level of motility of the left ventricular wall.
The lower right and left chambers of the heart. The right ventricle pumps venous BLOOD into the LUNGS and the left ventricle pumps oxygenated blood into the systemic arterial circulation.
This structure includes the thin muscular atrial septum between the two HEART ATRIA, and the thick muscular ventricular septum between the two HEART VENTRICLES.
A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (VENTRICULAR DYSFUNCTION), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as MYOCARDIAL INFARCTION.
Occlusion of the outflow tract in either the LEFT VENTRICLE or the RIGHT VENTRICLE of the heart. This may result from CONGENITAL HEART DEFECTS, predisposing heart diseases, complications of surgery, or HEART NEOPLASMS.
Contractile activity of the MYOCARDIUM.
Striated muscle cells found in the heart. They are derived from cardiac myoblasts (MYOBLASTS, CARDIAC).
The hollow, muscular organ that maintains the circulation of the blood.
The amount of BLOOD pumped out of the HEART per beat, not to be confused with cardiac output (volume/time). It is calculated as the difference between the end-diastolic volume and the end-systolic volume.
Unexpected rapid natural death due to cardiovascular collapse within one hour of initial symptoms. It is usually caused by the worsening of existing heart diseases. The sudden onset of symptoms, such as CHEST PAIN and CARDIAC ARRHYTHMIAS, particularly VENTRICULAR TACHYCARDIA, can lead to the loss of consciousness and cardiac arrest followed by biological death. (from Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 7th ed., 2005)
Myosin type II isoforms found in cardiac muscle.
The period shortly before, during, and immediately after giving birth.
Measurement of intracardiac blood flow using an M-mode and/or two-dimensional (2-D) echocardiogram while simultaneously recording the spectrum of the audible Doppler signal (e.g., velocity, direction, amplitude, intensity, timing) reflected from the moving column of red blood cells.
Isoforms of MYOSIN TYPE II, specifically found in the ventricular muscle of the HEART. Defects in the genes encoding ventricular myosins result in FAMILIAL HYPERTROPHIC CARDIOMYOPATHY.
Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.
An abnormally rapid ventricular rhythm usually in excess of 150 beats per minute. It is generated within the ventricle below the BUNDLE OF HIS, either as autonomic impulse formation or reentrant impulse conduction. Depending on the etiology, onset of ventricular tachycardia can be paroxysmal (sudden) or nonparoxysmal, its wide QRS complexes can be uniform or polymorphic, and the ventricular beating may be independent of the atrial beating (AV dissociation).
The repeating contractile units of the MYOFIBRIL, delimited by Z bands along its length.
A condition characterized by the thickening of the ventricular ENDOCARDIUM and subendocardium (MYOCARDIUM), seen mostly in children and young adults in the TROPICAL CLIMATE. The fibrous tissue extends from the apex toward and often involves the HEART VALVES causing restrictive blood flow into the respective ventricles (CARDIOMYOPATHY, RESTRICTIVE).
The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.
A disorder of cardiac function caused by insufficient blood flow to the muscle tissue of the heart. The decreased blood flow may be due to narrowing of the coronary arteries (CORONARY ARTERY DISEASE), to obstruction by a thrombus (CORONARY THROMBOSIS), or less commonly, to diffuse narrowing of arterioles and other small vessels within the heart. Severe interruption of the blood supply to the myocardial tissue may result in necrosis of cardiac muscle (MYOCARDIAL INFARCTION).
The larger subunits of MYOSINS. The heavy chains have a molecular weight of about 230 kDa and each heavy chain is usually associated with a dissimilar pair of MYOSIN LIGHT CHAINS. The heavy chains possess actin-binding and ATPase activity.
Enlargement of the LEFT VENTRICLE of the heart. This increase in ventricular mass is attributed to sustained abnormal pressure or volume loads and is a contributor to cardiovascular morbidity and mortality.
Any disturbances of the normal rhythmic beating of the heart or MYOCARDIAL CONTRACTION. Cardiac arrhythmias can be classified by the abnormalities in HEART RATE, disorders of electrical impulse generation, or impulse conduction.
The innermost layer of the heart, comprised of endothelial cells.
Members of the armadillo family of proteins that are found in DESMOSOMES and interact with various proteins including desmocadherins; DESMOPLAKIN; ACTIN FILAMENTS; and KERATINS.
The transference of a heart from one human or animal to another.
Any detectable and heritable change in the genetic material that causes a change in the GENOTYPE and which is transmitted to daughter cells and to succeeding generations.
A subclass of developmentally regulated lamins having a neutral isoelectric point. They are found to disassociate from nuclear membranes during mitosis.
The geometric and structural changes that the HEART VENTRICLES undergo, usually following MYOCARDIAL INFARCTION. It comprises expansion of the infarct and dilatation of the healthy ventricle segments. While most prevalent in the left ventricle, it can also occur in the right ventricle.
One of the three polypeptide chains that make up the TROPONIN complex. It is a cardiac-specific protein that binds to TROPOMYOSIN. It is released from damaged or injured heart muscle cells (MYOCYTES, CARDIAC). Defects in the gene encoding troponin T result in FAMILIAL HYPERTROPHIC CARDIOMYOPATHY.
The movement and the forces involved in the movement of the blood through the CARDIOVASCULAR SYSTEM.
Regulation of the rate of contraction of the heart muscles by an artificial pacemaker.
Post-systolic relaxation of the HEART, especially the HEART VENTRICLES.
Pathological conditions involving the HEART including its structural and functional abnormalities.
Disorders or diseases associated with PUERPERIUM, the six-to-eight-week period immediately after PARTURITION in humans.
A family of transmembrane dystrophin-associated proteins that play a role in the membrane association of the DYSTROPHIN-ASSOCIATED PROTEIN COMPLEX.
Enlargement of the HEART, usually indicated by a cardiothoracic ratio above 0.50. Heart enlargement may involve the right, the left, or both HEART VENTRICLES or HEART ATRIA. Cardiomegaly is a nonspecific symptom seen in patients with chronic systolic heart failure (HEART FAILURE) or several forms of CARDIOMYOPATHIES.
Implantable devices which continuously monitor the electrical activity of the heart and automatically detect and terminate ventricular tachycardia (TACHYCARDIA, VENTRICULAR) and VENTRICULAR FIBRILLATION. They consist of an impulse generator, batteries, and electrodes.
The abrupt cessation of all vital bodily functions, manifested by the permanent loss of total cerebral, respiratory, and cardiovascular functions.
A type of imaging technique used primarily in the field of cardiology. By coordinating the fast gradient-echo MRI sequence with retrospective ECG-gating, numerous short time frames evenly spaced in the cardiac cycle are produced. These images are laced together in a cinematic display so that wall motion of the ventricles, valve motion, and blood flow patterns in the heart and great vessels can be visualized.
A CALCIUM-dependent adhesion molecule of DESMOSOMES that also plays a role in embryonic STEM CELL proliferation.
Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease.
Period of contraction of the HEART, especially of the HEART VENTRICLES.
Laboratory mice that have been produced from a genetically manipulated EGG or EMBRYO, MAMMALIAN.
Naturally occurring or experimentally induced animal diseases with pathological processes sufficiently similar to those of human diseases. They are used as study models for human diseases.
Procedures in which placement of CARDIAC CATHETERS is performed for therapeutic or diagnostic procedures.
An X-linked dominant multisystem disorder resulting in cardiomyopathy, myopathy and INTELLECTUAL DISABILITY. It is caused by mutation in the gene encoding LYSOSOMAL-ASSOCIATED MEMBRANE PROTEIN 2.
The outward appearance of the individual. It is the product of interactions between genes, and between the GENOTYPE and the environment.
A mutation in which a codon is mutated to one directing the incorporation of a different amino acid. This substitution may result in an inactive or unstable product. (From A Dictionary of Genetics, King & Stansfield, 5th ed)
Rare congenital cardiomyopathies characterized by the lack of left ventricular myocardium compaction. The noncompaction results in numerous prominent trabeculations and a loose myocardial meshwork (spongy myocardium) in the LEFT VENTRICLE. Heterogeneous clinical features include diminished systolic function sometimes associated with left ventricular dilation, that presents either neonatally or progressively. Often, the RIGHT VENTRICLE is also affected. CONGESTIVE HEART FAILURE; PULMONARY EMBOLISM; and ventricular ARRHYTHMIA are commonly seen.
The co-occurrence of pregnancy and a cardiovascular disease. The disease may precede or follow FERTILIZATION and it may or may not have a deleterious effect on the pregnant woman or FETUS.
An intermediate filament protein found predominantly in smooth, skeletal, and cardiac muscle cells. Localized at the Z line. MW 50,000 to 55,000 is species dependent.
A muscle protein localized in surface membranes which is the product of the Duchenne/Becker muscular dystrophy gene. Individuals with Duchenne muscular dystrophy usually lack dystrophin completely while those with Becker muscular dystrophy have dystrophin of an altered size. It shares features with other cytoskeletal proteins such as SPECTRIN and alpha-actinin but the precise function of dystrophin is not clear. One possible role might be to preserve the integrity and alignment of the plasma membrane to the myofibrils during muscle contraction and relaxation. MW 400 kDa.
Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.
A condition in which HEART VENTRICLES exhibit impaired function.
Drugs that bind to but do not activate beta-adrenergic receptors thereby blocking the actions of beta-adrenergic agonists. Adrenergic beta-antagonists are used for treatment of hypertension, cardiac arrhythmias, angina pectoris, glaucoma, migraine headaches, and anxiety.
The valve between the left atrium and left ventricle of the heart.
Examinations used to diagnose and treat heart conditions.
Backflow of blood from the LEFT VENTRICLE into the LEFT ATRIUM due to imperfect closure of the MITRAL VALVE. This can lead to mitral valve regurgitation.
Removal of tissue by vaporization, abrasion, or destruction. Methods used include heating tissue by hot liquids or microwave thermal heating, freezing (CRYOABLATION), chemical ablation, and photoablation with LASERS.
A guanidine analog with specific affinity for tissues of the sympathetic nervous system and related tumors. The radiolabeled forms are used as antineoplastic agents and radioactive imaging agents. (Merck Index, 12th ed) MIBG serves as a neuron-blocking agent which has a strong affinity for, and retention in, the adrenal medulla and also inhibits ADP-ribosyltransferase.
A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.
Agents that have a strengthening effect on the heart or that can increase cardiac output. They may be CARDIAC GLYCOSIDES; SYMPATHOMIMETICS; or other drugs. They are used after MYOCARDIAL INFARCT; CARDIAC SURGICAL PROCEDURES; in SHOCK; or in congestive heart failure (HEART FAILURE).
Removal of tissue with electrical current delivered via electrodes positioned at the distal end of a catheter. Energy sources are commonly direct current (DC-shock) or alternating current at radiofrequencies (usually 750 kHz). The technique is used most often to ablate the AV junction and/or accessory pathways in order to interrupt AV conduction and produce AV block in the treatment of various tachyarrhythmias.
Elements of limited time intervals, contributing to particular results or situations.
Impaired conduction of cardiac impulse that can occur anywhere along the conduction pathway, such as between the SINOATRIAL NODE and the right atrium (SA block) or between atria and ventricles (AV block). Heart blocks can be classified by the duration, frequency, or completeness of conduction block. Reversibility depends on the degree of structural or functional defects.
In screening and diagnostic tests, the probability that a person with a positive test is a true positive (i.e., has the disease), is referred to as the predictive value of a positive test; whereas, the predictive value of a negative test is the probability that the person with a negative test does not have the disease. Predictive value is related to the sensitivity and specificity of the test.
Removal and pathologic examination of specimens in the form of small pieces of tissue from the living body.
An X-linked recessive muscle disease caused by an inability to synthesize DYSTROPHIN, which is involved with maintaining the integrity of the sarcolemma. Muscle fibers undergo a process that features degeneration and regeneration. Clinical manifestations include proximal weakness in the first few years of life, pseudohypertrophy, cardiomyopathy (see MYOCARDIAL DISEASES), and an increased incidence of impaired mentation. Becker muscular dystrophy is a closely related condition featuring a later onset of disease (usually adolescence) and a slowly progressive course. (Adams et al., Principles of Neurology, 6th ed, p1415)
A transient loss of consciousness and postural tone caused by diminished blood flow to the brain (i.e., BRAIN ISCHEMIA). Presyncope refers to the sensation of lightheadedness and loss of strength that precedes a syncopal event or accompanies an incomplete syncope. (From Adams et al., Principles of Neurology, 6th ed, pp367-9)
Recording the locations and measurements of electrical activity in the EPICARDIUM by placing electrodes on the surface of the heart to analyze the patterns of activation and to locate arrhythmogenic sites.
A protein found in the thin filaments of muscle fibers. It inhibits contraction of the muscle unless its position is modified by TROPONIN.
Method in which prolonged electrocardiographic recordings are made on a portable tape recorder (Holter-type system) or solid-state device ("real-time" system), while the patient undergoes normal daily activities. It is useful in the diagnosis and management of intermittent cardiac arrhythmias and transient myocardial ischemia.
Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group.
A condition in which the RIGHT VENTRICLE of the heart was functionally impaired. This condition usually leads to HEART FAILURE or MYOCARDIAL INFARCTION, and other cardiovascular complications. Diagnosis is made by measuring the diminished ejection fraction and a depressed level of motility of the right ventricular wall.
A giant elastic protein of molecular mass ranging from 2,993 kDa (cardiac), 3,300 kDa (psoas), to 3,700 kDa (soleus) having a kinase domain. The amino- terminal is involved in a Z line binding, and the carboxy-terminal region is bound to the myosin filament with an overlap between the counter-connectin filaments at the M line.
The long cylindrical contractile organelles of STRIATED MUSCLE cells composed of ACTIN FILAMENTS; MYOSIN filaments; and other proteins organized in arrays of repeating units called SARCOMERES .
The mitochondria of the myocardium.
A form of heart block in which the electrical stimulation of HEART VENTRICLES is interrupted at either one of the branches of BUNDLE OF HIS thus preventing the simultaneous depolarization of the two ventricles.
Methods to induce and measure electrical activities at specific sites in the heart to diagnose and treat problems with the heart's electrical system.
One of the three polypeptide chains that make up the TROPONIN complex. It inhibits F-actin-myosin interactions.
A diverse superfamily of proteins that function as translocating proteins. They share the common characteristics of being able to bind ACTINS and hydrolyze MgATP. Myosins generally consist of heavy chains which are involved in locomotion, and light chains which are involved in regulation. Within the structure of myosin heavy chain are three domains: the head, the neck and the tail. The head region of the heavy chain contains the actin binding domain and MgATPase domain which provides energy for locomotion. The neck region is involved in binding the light-chains. The tail region provides the anchoring point that maintains the position of the heavy chain. The superfamily of myosins is organized into structural classes based upon the type and arrangement of the subunits they contain.
Echocardiography applying the Doppler effect, with the superposition of flow information as colors on a gray scale in a real-time image.
Abnormally rapid heartbeat, usually with a HEART RATE above 100 beats per minute for adults. Tachycardia accompanied by disturbance in the cardiac depolarization (cardiac arrhythmia) is called tachyarrhythmia.
A heterogeneous group of infections produced by coxsackieviruses, including HERPANGINA, aseptic meningitis (MENINGITIS, ASEPTIC), a common-cold-like syndrome, a non-paralytic poliomyelitis-like syndrome, epidemic pleurodynia (PLEURODYNIA, EPIDEMIC) and a serious MYOCARDITIS.
Inflammation of the PERICARDIUM that is characterized by the fibrous scarring and adhesion of both serous layers, the VISCERAL PERICARDIUM and the PARIETAL PERICARDIUM leading to the loss of pericardial cavity. The thickened pericardium severely restricts cardiac filling. Clinical signs include FATIGUE, muscle wasting, and WEIGHT LOSS.
Studies which start with the identification of persons with a disease of interest and a control (comparison, referent) group without the disease. The relationship of an attribute to the disease is examined by comparing diseased and non-diseased persons with regard to the frequency or levels of the attribute in each group.
The circulation of blood through the CORONARY VESSELS of the HEART.
AMINO ALCOHOLS containing the propanolamine (NH2CH2CHOHCH2) group and its derivatives.
Small pumps, often implantable, designed for temporarily assisting the heart, usually the LEFT VENTRICLE, to pump blood. They consist of a pumping chamber and a power source, which may be partially or totally external to the body and activated by electromagnetic motors.
Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.
A device designed to stimulate, by electric impulses, contraction of the heart muscles. It may be temporary (external) or permanent (internal or internal-external).
A PEPTIDE that is secreted by the BRAIN and the HEART ATRIA, stored mainly in cardiac ventricular MYOCARDIUM. It can cause NATRIURESIS; DIURESIS; VASODILATION; and inhibits secretion of RENIN and ALDOSTERONE. It improves heart function. It contains 32 AMINO ACIDS.
Detection of a MUTATION; GENOTYPE; KARYOTYPE; or specific ALLELES associated with genetic traits, heritable diseases, or predisposition to a disease, or that may lead to the disease in descendants. It includes prenatal genetic testing.
Echocardiography applying the Doppler effect, with velocity detection combined with range discrimination. Short bursts of ultrasound are transmitted at regular intervals and the echoes are demodulated as they return.
A group of desmosomal cadherins with cytoplasmic tails that are divergent from those of classical CADHERINS. Their intracytoplasmic domains bind PLAKOGLOBIN; PLAKOPHILINS; and DESMOPLAKINS.
Biochemical identification of mutational changes in a nucleotide sequence.
A large class of structurally-related proteins that contain one or more LIM zinc finger domains. Many of the proteins in this class are involved in intracellular signaling processes and mediate their effects via LIM domain protein-protein interactions. The name LIM is derived from the first three proteins in which the motif was found: LIN-11, Isl1 and Mec-3.
Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.
A selective adrenergic beta-1 blocking agent that is commonly used to treat ANGINA PECTORIS; HYPERTENSION; and CARDIAC ARRHYTHMIAS.
Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques.
Desmoplakins are cytoskeletal linker proteins that anchor INTERMEDIATE FILAMENTS to the PLASMA MEMBRANE at DESMOSOMES.
An impulse-conducting system composed of modified cardiac muscle, having the power of spontaneous rhythmicity and conduction more highly developed than the rest of the heart.
A genetically heterogeneous, multifaceted disorder characterized by short stature, webbed neck, ptosis, skeletal malformations, hypertelorism, hormonal imbalance, CRYPTORCHIDISM, multiple cardiac abnormalities (most commonly including PULMONARY VALVE STENOSIS), and some degree of INTELLECTUAL DISABILITY. The phenotype bears similarities to that of TURNER SYNDROME that occurs only in females and has its basis in a 45, X karyotype abnormality. Noonan syndrome occurs in both males and females with a normal karyotype (46,XX and 46,XY). Mutations in a several genes (PTPN11, KRAS, SOS1, NF1 and RAF1) have been associated the the NS phenotype. Mutations in PTPN11 are the most common. LEOPARD SYNDROME, a disorder that has clinical features overlapping those of Noonan Syndrome, is also due to mutations in PTPN11. In addition, there is overlap with the syndrome called neurofibromatosis-Noonan syndrome due to mutations in NF1.
A subclass of beta-adrenergic receptors (RECEPTORS, ADRENERGIC, BETA). The adrenergic beta-1 receptors are equally sensitive to EPINEPHRINE and NOREPINEPHRINE and bind the agonist DOBUTAMINE and the antagonist METOPROLOL with high affinity. They are found in the HEART, juxtaglomerular cells, and in the central and peripheral nervous systems.
A type of junction that attaches one cell to its neighbor. One of a number of differentiated regions which occur, for example, where the cytoplasmic membranes of adjacent epithelial cells are closely apposed. It consists of a circular region of each membrane together with associated intracellular microfilaments and an intercellular material which may include, for example, mucopolysaccharides. (From Glick, Glossary of Biochemistry and Molecular Biology, 1990; Singleton & Sainsbury, Dictionary of Microbiology and Molecular Biology, 2d ed)
The pressure within a CARDIAC VENTRICLE. Ventricular pressure waveforms can be measured in the beating heart by catheterization or estimated using imaging techniques (e.g., DOPPLER ECHOCARDIOGRAPHY). The information is useful in evaluating the function of the MYOCARDIUM; CARDIAC VALVES; and PERICARDIUM, particularly with simultaneous measurement of other (e.g., aortic or atrial) pressures.
The protein constituents of muscle, the major ones being ACTINS and MYOSINS. More than a dozen accessory proteins exist including TROPONIN; TROPOMYOSIN; and DYSTROPHIN.
Benzo-indoles similar to CARBOLINES which are pyrido-indoles. In plants, carbazoles are derived from indole and form some of the INDOLE ALKALOIDS.
Controlled physical activity which is performed in order to allow assessment of physiological functions, particularly cardiovascular and pulmonary, but also aerobic capacity. Maximal (most intense) exercise is usually required but submaximal exercise is also used.
A catecholamine derivative with specificity for BETA-1 ADRENERGIC RECEPTORS. It is commonly used as a cardiotonic agent after CARDIAC SURGERY and during DOBUTAMINE STRESS ECHOCARDIOGRAPHY.
A type of cardiac arrhythmia with premature contractions of the HEART VENTRICLES. It is characterized by the premature QRS complex on ECG that is of abnormal shape and great duration (generally >129 msec). It is the most common form of all cardiac arrhythmias. Premature ventricular complexes have no clinical significance except in concurrence with heart diseases.
A state of subnormal or depressed cardiac output at rest or during stress. It is a characteristic of CARDIOVASCULAR DISEASES, including congenital, valvular, rheumatic, hypertensive, coronary, and cardiomyopathic. The serious form of low cardiac output is characterized by marked reduction in STROKE VOLUME, and systemic vasoconstriction resulting in cold, pale, and sometimes cyanotic extremities.
Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept.
The number of times the HEART VENTRICLES contract per unit of time, usually per minute.
An individual having different alleles at one or more loci regarding a specific character.
Antineoplastic antibiotic obtained from Streptomyces peucetius. It is a hydroxy derivative of DAUNORUBICIN.
Diabetes mellitus induced experimentally by administration of various diabetogenic agents or by PANCREATECTOMY.
An autosomal recessive disease, usually of childhood onset, characterized pathologically by degeneration of the spinocerebellar tracts, posterior columns, and to a lesser extent the corticospinal tracts. Clinical manifestations include GAIT ATAXIA, pes cavus, speech impairment, lateral curvature of spine, rhythmic head tremor, kyphoscoliosis, congestive heart failure (secondary to a cardiomyopathy), and lower extremity weakness. Most forms of this condition are associated with a mutation in a gene on chromosome 9, at band q13, which codes for the mitochondrial protein frataxin. (From Adams et al., Principles of Neurology, 6th ed, p1081; N Engl J Med 1996 Oct 17;335(16):1169-75) The severity of Friedreich ataxia associated with expansion of GAA repeats in the first intron of the frataxin gene correlates with the number of trinucleotide repeats. (From Durr et al, N Engl J Med 1996 Oct 17;335(16):1169-75)
A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS.
Agents that affect the rate or intensity of cardiac contraction, blood vessel diameter, or blood volume.
The muscular structure separating the right and the left lower chambers (HEART VENTRICLES) of the heart. The ventricular septum consists of a very small membranous portion just beneath the AORTIC VALVE, and a large thick muscular portion consisting of three sections including the inlet septum, the trabecular septum, and the outlet septum.
The percent frequency with which a dominant or homozygous recessive gene or gene combination manifests itself in the phenotype of the carriers. (From Glossary of Genetics, 5th ed)
Imaging of a ventricle of the heart after the injection of a radioactive contrast medium. The technique is less invasive than cardiac catheterization and is used to assess ventricular function.
A group of sporadic, familial and/or inherited, degenerative, and infectious disease processes, linked by the common theme of abnormal protein folding and deposition of AMYLOID. As the amyloid deposits enlarge they displace normal tissue structures, causing disruption of function. Various signs and symptoms depend on the location and size of the deposits.
A group of muscle diseases associated with abnormal mitochondria function.
Calcium-transporting ATPases that catalyze the active transport of CALCIUM into the SARCOPLASMIC RETICULUM vesicles from the CYTOPLASM. They are primarily found in MUSCLE CELLS and play a role in the relaxation of MUSCLES.
Chemical substances, produced by microorganisms, inhibiting or preventing the proliferation of neoplasms.
The worsening of a disease over time. This concept is most often used for chronic and incurable diseases where the stage of the disease is an important determinant of therapy and prognosis.
A potentially lethal cardiac arrhythmia that is characterized by uncoordinated extremely rapid firing of electrical impulses (400-600/min) in HEART VENTRICLES. Such asynchronous ventricular quivering or fibrillation prevents any effective cardiac output and results in unconsciousness (SYNCOPE). It is one of the major electrocardiographic patterns seen with CARDIAC ARREST.
A localized bulging or dilatation in the muscle wall of a heart (MYOCARDIUM), usually in the LEFT VENTRICLE. Blood-filled aneurysms are dangerous because they may burst. Fibrous aneurysms interfere with the heart function through the loss of contractility. True aneurysm is bound by the vessel wall or cardiac wall. False aneurysms are HEMATOMA caused by myocardial rupture.
A potent natriuretic and vasodilatory peptide or mixture of different-sized low molecular weight PEPTIDES derived from a common precursor and secreted mainly by the HEART ATRIUM. All these peptides share a sequence of about 20 AMINO ACIDS.
Activities or games, usually involving physical effort or skill. Reasons for engagement in sports include pleasure, competition, and/or financial reward.
Agents used for the treatment or prevention of cardiac arrhythmias. They may affect the polarization-repolarization phase of the action potential, its excitability or refractoriness, or impulse conduction or membrane responsiveness within cardiac fibers. Anti-arrhythmia agents are often classed into four main groups according to their mechanism of action: sodium channel blockade, beta-adrenergic blockade, repolarization prolongation, or calcium channel blockade.
An aspect of personal behavior or lifestyle, environmental exposure, or inborn or inherited characteristic, which, on the basis of epidemiologic evidence, is known to be associated with a health-related condition considered important to prevent.
The agent of South American trypanosomiasis or CHAGAS DISEASE. Its vertebrate hosts are man and various domestic and wild animals. Insects of several species are vectors.
A species of ENTEROVIRUS infecting humans and containing 36 serotypes. It is comprised of all the echoviruses and a few coxsackieviruses, including all of those previously named coxsackievirus B.
A complex of gadolinium with a chelating agent, diethylenetriamine penta-acetic acid (DTPA see PENTETIC ACID), that is given to enhance the image in cranial and spinal MRIs. (From Martindale, The Extra Pharmacopoeia, 30th ed, p706)
Rare congenital X-linked disorder of lipid metabolism. Barth syndrome is transmitted in an X-linked recessive pattern. The syndrome is characterized by muscular weakness, growth retardation, DILATED CARDIOMYOPATHY, variable NEUTROPENIA, 3-methylglutaconic aciduria (type II) and decreases in mitochondrial CARDIOLIPIN level. Other biochemical and morphological mitochondrial abnormalities also exist.
Impaired impulse conduction from HEART ATRIA to HEART VENTRICLES. AV block can mean delayed or completely blocked impulse conduction.
A genus of the family PICORNAVIRIDAE whose members preferentially inhabit the intestinal tract of a variety of hosts. The genus contains many species. Newly described members of human enteroviruses are assigned continuous numbers with the species designated "human enterovirus".
The genetic constitution of the individual, comprising the ALLELES present at each GENETIC LOCUS.
A basic element found in nearly all organized tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.
Substances used to allow enhanced visualization of tissues.
Conical muscular projections from the walls of the cardiac ventricles, attached to the cusps of the atrioventricular valves by the chordae tendineae.
Radiography of the vascular system of the heart muscle after injection of a contrast medium.
Recording of regional electrophysiological information by analysis of surface potentials to give a complete picture of the effects of the currents from the heart on the body surface. It has been applied to the diagnosis of old inferior myocardial infarction, localization of the bypass pathway in Wolff-Parkinson-White syndrome, recognition of ventricular hypertrophy, estimation of the size of a myocardial infarct, and the effects of different interventions designed to reduce infarct size. The limiting factor at present is the complexity of the recording and analysis, which requires 100 or more electrodes, sophisticated instrumentation, and dedicated personnel. (Braunwald, Heart Disease, 4th ed)
A class of statistical procedures for estimating the survival function (function of time, starting with a population 100% well at a given time and providing the percentage of the population still well at later times). The survival analysis is then used for making inferences about the effects of treatments, prognostic factors, exposures, and other covariates on the function.
Strains of mice in which certain GENES of their GENOMES have been disrupted, or "knocked-out". To produce knockouts, using RECOMBINANT DNA technology, the normal DNA sequence of the gene being studied is altered to prevent synthesis of a normal gene product. Cloned cells in which this DNA alteration is successful are then injected into mouse EMBRYOS to produce chimeric mice. The chimeric mice are then bred to yield a strain in which all the cells of the mouse contain the disrupted gene. Knockout mice are used as EXPERIMENTAL ANIMAL MODELS for diseases (DISEASE MODELS, ANIMAL) and to clarify the functions of the genes.
Graphic registration of the heart sounds picked up as vibrations and transformed by a piezoelectric crystal microphone into a varying electrical output according to the stresses imposed by the sound waves. The electrical output is amplified by a stethograph amplifier and recorded by a device incorporated into the electrocardiograph or by a multichannel recording machine.
Transport proteins that carry specific substances in the blood or across cell membranes.
Acquired, familial, and congenital disorders of SKELETAL MUSCLE and SMOOTH MUSCLE.
Radionuclide ventriculography where scintigraphic data is acquired during repeated cardiac cycles at specific times in the cycle, using an electrocardiographic synchronizer or gating device. Analysis of right ventricular function is difficult with this technique; that is best evaluated by first-pass ventriculography (VENTRICULOGRAPHY, FIRST-PASS).
Radiography of the heart and great vessels after injection of a contrast medium.
A multi-functional catenin that is highly homologous to BETA CATENIN. Gamma catenin binds CADHERINS and helps link their cytoplasmic tails to ACTIN in the CYTOSKELETON via ALPHA CATENIN. It is also found in DESMOSOMES where it mediates the link between DESMOSOMAL CADHERINS and DESMOPLAKIN.
A strain of mice arising from a spontaneous MUTATION (mdx) in inbred C57BL mice. This mutation is X chromosome-linked and produces viable homozygous animals that lack the muscle protein DYSTROPHIN, have high serum levels of muscle ENZYMES, and possess histological lesions similar to human MUSCULAR DYSTROPHY. The histological features, linkage, and map position of mdx make these mice a worthy animal model of DUCHENNE MUSCULAR DYSTROPHY.
Gadolinium. An element of the rare earth family of metals. It has the atomic symbol Gd, atomic number 64, and atomic weight 157.25. Its oxide is used in the control rods of some nuclear reactors.
The hemodynamic and electrophysiological action of the right HEART VENTRICLE.
The chambers of the heart, to which the BLOOD returns from the circulation.
A characteristic symptom complex.
An X-linked inherited metabolic disease caused by a deficiency of lysosomal ALPHA-GALACTOSIDASE A. It is characterized by intralysosomal accumulation of globotriaosylceramide and other GLYCOSPHINGOLIPIDS in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders.
The proportion of survivors in a group, e.g., of patients, studied and followed over a period, or the proportion of persons in a specified group alive at the beginning of a time interval who survive to the end of the interval. It is often studied using life table methods.
An autosomal dominant disorder with an acronym of its seven features (LENTIGO; ELECTROCARDIOGRAM abnormalities; ocular HYPERTELORISM; PULMONARY STENOSIS; abnormal genitalia; retardation of growth; and DEAFNESS or SENSORINEURAL HEARING LOSS). This syndrome is caused by mutations of PTPN11 gene encoding the non-receptor PROTEIN TYROSINE PHOSPHATASE, type 11, and is an allelic to NOONAN SYNDROME. Features of LEOPARD syndrome overlap with those of NEUROFIBROMATOSIS 1 which is caused by mutations in the NEUROFIBROMATOSIS 1 GENES.
Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care. (Dictionary of Health Services Management, 2d ed)
An antianginal and class III antiarrhythmic drug. It increases the duration of ventricular and atrial muscle action by inhibiting POTASSIUM CHANNELS and VOLTAGE-GATED SODIUM CHANNELS. There is a resulting decrease in heart rate and in vascular resistance.
A clear, colorless liquid rapidly absorbed from the gastrointestinal tract and distributed throughout the body. It has bactericidal activity and is used often as a topical disinfectant. It is widely used as a solvent and preservative in pharmaceutical preparations as well as serving as the primary ingredient in ALCOHOLIC BEVERAGES.
A method of recording heart motion and internal structures by combining ultrasonic imaging with exercise testing (EXERCISE TEST) or pharmacologic stress.
A condition characterized by the thickening of ENDOCARDIUM due to proliferation of fibrous and elastic tissue, usually in the left ventricle leading to impaired cardiac function (CARDIOMYOPATHY, RESTRICTIVE). It is most commonly seen in young children and rarely in adults. It is often associated with congenital heart anomalies (HEART DEFECTS CONGENITAL;) INFECTION; or gene mutation. Defects in the tafazzin protein, encoded by TAZ gene, result in a form of autosomal dominant familial endocardial fibroelastosis.
One of the alpha crystallin subunits. In addition to being expressed in the lens (LENS, CRYSTALLINE), alpha-crystallin B chain has been found in a variety of tissues such as HEART; BRAIN; MUSCLE; and KIDNEY. Accumulation of the protein in the brain is associated with NEURODEGENERATIVE DISEASES such as CREUTZFELDT-JAKOB SYNDROME and ALEXANDER DISEASE.
Measurable and quantifiable biological parameters (e.g., specific enzyme concentration, specific hormone concentration, specific gene phenotype distribution in a population, presence of biological substances) which serve as indices for health- and physiology-related assessments, such as disease risk, psychiatric disorders, environmental exposure and its effects, disease diagnosis, metabolic processes, substance abuse, pregnancy, cell line development, epidemiologic studies, etc.
A conical fibro-serous sac surrounding the HEART and the roots of the great vessels (AORTA; VENAE CAVAE; PULMONARY ARTERY). Pericardium consists of two sacs: the outer fibrous pericardium and the inner serous pericardium. The latter consists of an outer parietal layer facing the fibrous pericardium, and an inner visceral layer (epicardium) resting next to the heart, and a pericardial cavity between these two layers.
The exercise capacity of an individual as measured by endurance (maximal exercise duration and/or maximal attained work load) during an EXERCISE TEST.
A family of RNA viruses that infect fungi and protozoa. There are three genera: TOTIVIRUS; GIARDIAVIRUS; and LEISHMANIAVIRUS.
A group of cardiac arrhythmias in which the cardiac contractions are not initiated at the SINOATRIAL NODE. They include both atrial and ventricular premature beats, and are also known as extra or ectopic heartbeats. Their frequency is increased in heart diseases.
Prolonged dysfunction of the myocardium after a brief episode of severe ischemia, with gradual return of contractile activity.
The measurement of an organ in volume, mass, or heaviness.
The qualitative or quantitative estimation of the likelihood of adverse effects that may result from exposure to specified health hazards or from the absence of beneficial influences. (Last, Dictionary of Epidemiology, 1988)
General increase in bulk of a part or organ due to CELL ENLARGEMENT and accumulation of FLUIDS AND SECRETIONS, not due to tumor formation, nor to an increase in the number of cells (HYPERPLASIA).
One of two major pharmacologically defined classes of adrenergic receptors. The beta adrenergic receptors play an important role in regulating CARDIAC MUSCLE contraction, SMOOTH MUSCLE relaxation, and GLYCOGENOLYSIS.
A disturbance in the prooxidant-antioxidant balance in favor of the former, leading to potential damage. Indicators of oxidative stress include damaged DNA bases, protein oxidation products, and lipid peroxidation products (Sies, Oxidative Stress, 1991, pxv-xvi).
The restoration of the sequential order of contraction and relaxation of the HEART ATRIA and HEART VENTRICLES by atrio-biventricular pacing.
A single-pass transmembrane glycoproteins that mediate CALCIUM-dependent CELL ADHESION and are core components of DESMOSOMES.
Agents that have a damaging effect on the HEART. Such damage can occur from ALKYLATING AGENTS; FREE RADICALS; or metabolites from OXIDATIVE STRESS and in some cases is countered by CARDIOTONIC AGENTS. Induction of LONG QT SYNDROME or TORSADES DE POINTES has been the reason for viewing some drugs as cardiotoxins.
The hemodynamic and electrophysiological action of the HEART VENTRICLES.
Diseases caused by abnormal function of the MITOCHONDRIA. They may be caused by mutations, acquired or inherited, in mitochondrial DNA or in nuclear genes that code for mitochondrial components. They may also be the result of acquired mitochondria dysfunction due to adverse effects of drugs, infections, or other environmental causes.

Coexistence of mitochondrial DNA and beta myosin heavy chain mutations in hypertrophic cardiomyopathy with late congestive heart failure. (1/1667)

OBJECTIVE: To investigate the possible coexistence of mitochondrial DNA (mtDNA) mutations in patients with beta myosin heavy chain (beta MHC) linked hypertrophic cardiomyopathy (HCM) who develop congestive heart failure. DESIGN: Molecular analysis of beta MHC and mtDNA gene defects in patients with HCM. SETTING: Cardiovascular molecular diagnostic and heart transplantation reference centre in north Italy. PATIENTS: Four patients with HCM who underwent heart transplantation for end stage heart failure, and after pedigree analysis of 60 relatives, eight additional affected patients and 27 unaffected relatives. A total of 111 unrelated healthy adult volunteers served as controls. Disease controls included an additional 27 patients with HCM and 102 with dilated cardiomyopathy. INTERVENTION: Molecular analysis of DNA from myocardial and skeletal muscle tissue and from peripheral blood specimens. MAIN OUTCOME MEASURES: Screening for mutations in beta MHC (exons 3-23) and mtDNA tRNA (n = 22) genes with denaturing gradient gel electrophoresis or single strand conformational polymorphism followed by automated DNA sequencing. RESULTS: One proband (kindred A) (plus seven affected relatives) had arginine 249 glutamine (Arg249Gln) beta MHC and heteroplasmic mtDNA tRNAIle A4300G mutations. Another unrelated patient (kindred B) with sporadic HCM had identical mutations. The remaining two patients (kindred C), a mother and son, had a novel beta MHC mutation (lysine 450 glutamic acid) (Lys450Glu) and a heteroplasmic missense (T9957C, phenylalanine (Phe)-->leucine (Leu)) mtDNA mutation in subunit III of the cytochrome C oxidase gene. The amount of mutant mtDNA was higher in the myocardium than in skeletal muscle or peripheral blood and in affected patients than in asymptomatic relatives. Mutations were absent in the controls. Pathological and biochemical characteristics of patients with mutations Arg249Gln plus A4300G (kindreds A and B) were identical, but different from those of the two patients with Lys450Glu plus T9957C(Phe-->Leu) mutations (kindred C). Cytochrome C oxidase activity and histoenzymatic staining were severely decreased in the two patients in kindreds A and B, but were unaffected in the two in kindred C. CONCLUSIONS: beta MHC gene and mtDNA mutations may coexist in patients with HCM and end stage congestive heart failure. Although beta MHC gene mutations seem to be the true determinants of HCM, both mtDNA mutations in these patients have known prerequisites for pathogenicity. Coexistence of other genetic abnormalities in beta MHC linked HCM, such as mtDNA mutations, may contribute to variable phenotypic expression and explain the heterogeneous behaviour of HCM.  (+info)

Altered crossbridge kinetics in the alphaMHC403/+ mouse model of familial hypertrophic cardiomyopathy. (2/1667)

A mutation in the cardiac beta-myosin heavy chain, Arg403Gln (R403Q), causes a severe form of familial hypertrophic cardiomyopathy (FHC) in humans. We used small-amplitude (0.25%) length-perturbation analysis to examine the mechanical properties of skinned left ventricular papillary muscle strips from mouse hearts bearing the R403Q mutation in the alpha-myosin heavy chain (alphaMHC403/+). Myofibrillar disarray with variable penetrance occurred in the left ventricular free wall of the alphaMHC403/+ hearts. In resting strips (pCa 8), dynamic stiffness was approximately 40% greater than in wild-type strips, consistent with elevated diastolic stiffness reported for murine hearts with FHC. At pCa 6 (submaximal activation), strip isometric tension was approximately 3 times higher than for wild-type strips, whereas at pCa 5 (maximal activation), tension was marginally lower. At submaximal calcium activation the characteristic frequencies of the work-producing (b) and work-absorbing (c) steps of the crossbridge were less in alphaMHC403/+ strips than in wild-type strips (b=11+/-1 versus 15+/-1 Hz; c= 58+/-3 versus 66+/-3 Hz; 27 degrees C). At maximal calcium activation, strip oscillatory power was reduced (0. 53+/-0.25 versus 1.03+/-0.18 mW/mm3; 27 degrees C), which is partly attributable to the reduced frequency b, at which crossbridge work is maximum. The results are consistent with the hypothesis that the R403Q mutation reduces the strong binding affinity of myosin for actin. Myosin heads may accumulate in a preforce state that promotes cooperative activation of the thin filament at submaximal calcium but blunts maximal tension and oscillatory power output at maximal calcium. The calcium-dependent effect of the mutation (whether facilitating or debilitating), together with a variable degree of fibrosis and myofibrillar disorder, may contribute to the diversity of clinical symptoms observed in murine FHC.  (+info)

Altered cardiac excitation-contraction coupling in mutant mice with familial hypertrophic cardiomyopathy. (3/1667)

Excitation-contraction coupling in cardiac muscle of familial hypertrophic cardiomyopathy (FHC) remains poorly understood, despite the fact that the genetic alterations are well defined. We characterized calcium cycling and contractile activation in trabeculae from a mutant mouse model of FHC (Arg403Gln knockin, alpha-myosin heavy chain). Wild-type mice of the same strain and age ( approximately 20 weeks old) served as controls. During twitch contractions, peak intracellular Ca2+ ([Ca2+]i) was higher in mutant muscles than in the wild-type (P < 0.05), but force development was equivalent in the two groups. Ca2+ transient amplitude increased dramatically in both groups as stimulation rate increased from 0.2 to 4 Hz. Nevertheless, developed force fell at the higher stimulation rates in the mutants but not in controls (P < 0.05). The steady-state force-[Ca2+]i relationship was less steep in mutants (Hill coefficient, 2.94 +/- 0.27 vs. 5.28 +/- 0.64; P > 0.003), with no changes in the [Ca2+]i required for 50% activation or maximal Ca2+-activated force. Thus, calcium cycling and myofilament properties are both altered in FHC mutant mice: more Ca2+ is mobilized to generate force, but this does not suffice to maintain contractility at high stimulation rates.  (+info)

Sudden death in hypertrophic cardiomyopathy: potential importance of altered autonomic control of vasculature. (4/1667)

Current evidence suggests that alterations in the autonomic function and abnormal vascular control play a significant role either as independent triggers themselves or as modifiers of ischaemia and tolerance to to arrhythmias. A combination of several factors--that is, arrhythmia, hypotension, altered autonomic function including vascular control, and ischaemia are therefore likely to act as triggers for sudden death. The relative contribution of each of these factors needs further detailed study.  (+info)

Effects of permanent dual-chamber pacing on mitral regurgitation in hypertrophic obstructive cardiomyopathy. (5/1667)

AIMS: To assess the effects of chronic dual-chamber pacing on mitral regurgitation in hypertrophic obstructive cardiomyopathy. METHODS AND RESULTS: Twenty-three patients with hypertrophic obstructive cardiomyopathy and mitral regurgitation. treated with DDD pacing for 16 +/- 14 months, were included in the study. Mitral regurgitation was assessed by Doppler-echocardiography using semi-quantitative analysis (grades I-IV) and by measuring the maximum regurgitant jet area/left atrial area ratio. At the end of follow-up, DDD pacing reduced the outflow gradient from 93 +/- 37 mmHg to 31 +/- 30 mmHg (P<0.0001). Nine of the 14 patients who initially had > or =grade II mitral regurgitation improved by at least one grade, two of them exhibiting dramatic improvement (from grade IV and III to grade I). The regurgitant jet area/left atrial area ratio was reduced with DDD pacing from 20 +/- 13% to 11 +/- 6% (P<0.0001). Patients who had significant mitral regurgitation despite pacing were those whose outflow gradient remained high or those with mitral valve organic abnormalities (mitral annulus calcification or mitral valve prolapse). In the absence of organic abnormalities other than leaflet elongation, there was a significant correlation between the gradient value achieved with DDD pacing and the extent of mitral regurgitation (P<0.05). CONCLUSION: In the absence of organic mitral valve abnormalities, DDD pacing reduces in parallel mitral regurgitation and left ventricular outflow gradient. In such patients therefore, significant mitral regurgitation is not a contraindication to pacing.  (+info)

Rapid progression of cardiomyopathy in mitochondrial diabetes. (6/1667)

Cardiac involvement and its clinical course in a diabetic patient with a mitochondrial tRNA(Leu)(UUR) mutation at position 3243 is reported in a 54-year-old man with no history of hypertension. At age 46, an electrocardiogram showed just T wave abnormalities. At age 49, it fulfilled SV1 + RV5 or 6>35 mm with strain pattern. At age 52, echocardiography revealed definite left ventricular (LV) hypertrophy, and abnormally increased mitochondria were shown in biopsied endomyocardial specimens. He was diagnosed as having developed hypertrophic cardiomyopathy associated with the mutation. However, at age 54, SV1 and RV5,6 voltages were decreased, and echocardiography showed diffuse decreased LV wall motion and LV dilatation. Because he had mitochondrial diabetes, the patient's heart rapidly developed hypertrophic cardiomyopathy, and then it seemed to be changing to a dilated LV with systolic dysfunction. Rapid progression of cardiomyopathy can occur in mitochondrial diabetes.  (+info)

A patient with hypertrophic cardiomyopathy accompanied by right ventricular dilation of unknown cause. (7/1667)

Hypertrophic cardiomyopathy (HCM) is a disease characterized by an unknown cause of hypertrophy in the left or right ventricle. The dilated phase of HCM shows disease conditions resembling dilated cardiomyopathy, such as ventricular dilation, thin ventricular wall, and reduction of the ejection fraction. A patient presented with left ventricular concentric hypertrophy accompanied by right ventricular dilatation of unknown cause. Right ventricular endomyocardial biopsy specimens showed characteristic myocardial disarray. Therefore, there is the possibility that the patient had right and left ventricular HCM in the process toward the dilated phase, in which dilatation first occurred in the right ventricle.  (+info)

Ca2+ sensitization and potentiation of the maximum level of myofibrillar ATPase activity caused by mutations of troponin T found in familial hypertrophic cardiomyopathy. (8/1667)

Human wild-type cardiac troponin T, I, C and five troponin T mutants (I79N, R92Q, F110I, E244D, and R278C) causing familial hypertrophic cardiomyopathy were expressed in Escherichia coli, and then were purified and incorporated into rabbit cardiac myofibrils using a troponin exchange technique. The Ca2+-sensitive ATPase activity of these myofibrillar preparations was measured in order to examine the functional consequences of these troponin mutations. An I79N troponin T mutation was found to cause a definite increase in Ca2+ sensitivity of the myofibrillar ATPase activity without inducing any significant change in the maximum level of ATPase activity. A detailed analysis indicated the inhibitory action of troponin I to be impaired by the I79N troponin T mutation. Two more troponin T mutations (R92Q and R278C) were also found to have a Ca2+-sensitizing effect without inducing any change in maximum ATPase activity. Two other troponin T mutations (F110I and E244D) had no Ca2+-sensitizing effects on the ATPase activity, but remarkably potentiated the maximum level of ATPase activity. These findings indicate that hypertrophic cardiomyopathy-linked troponin T mutations have at least two different effects on the Ca2+-sensitive ATPase activity, Ca2+-sensitization and potentiation of the maximum level of the ATPase activity.  (+info)

The angiographic features of the left ventricle were examined in patients with idiopathic hypertrophic subaortic stenosis who had clinical and hemodynamic evidence of obstruction. Of 36 combined hemodynamic and angiographic studies considered to be technically satisfactory, 33 showed a characteristic combination of abnormalities. In the frontal projection in systole, a linear radiolucent area extended across the left ventricular outflow tract 2 to 2.5 cm below the aortic annulus, at a level corresponding to the site of intraventricular pressure change. In the left oblique and lateral projections, the mitral leaflets did not swing posteriorly in a normal fashion, but projected into the outflow tract during mid and late systole. The radiolucent line, seen in the frontal views, was considered to represent contact of the leading edge of the leaflet with the hypertrophied muscular interventricular septum. The jet of mitral regurgitation, when present, was seen immediately below the anterior mitral ...
Only 10 years have elapsed since the concept of functional obstruction to left ventricular ejection was introduced to clinical medicine (1), and in the interim the features of obstructive cardiomyopathy, or idiopathic hypertrophic subaortic stenosis, have become generally recognized. It seems certain that the increasingly frequent recognition of this disease has resulted largely from wider application of left heart catheterization techniques; nevertheless, while the diagnosis initially was based on hemodynamic features peculiar to this disease, it has become possible with increasing experience to recognize and assess the severity of this lesion on clinical grounds alone (2).. Work originating in a ...
Introduction: Hypertrophic Obstructive Cardiomyopathy (HOCM) is associated with systolic anterior motion of the mitral valve, frequently leading to mitral regurgitation (MR). We hypothesized that after septal reduction with alcohol septal ablation (ASA), left ventricular outflow tract gradients would be reduced and mitral regurgitation would improve.. Methods: We reviewed echocardiograms at baseline and 3 month post ASA for 210 consecutive patients treated at the Medical University of South Carolina between 2000 and 2007 for whom complete echo data was available. For each echocardiogram the left atrial end-systolic volume index (LAESVI), resting left ventricular outflow tract (LVOT) gradient, degree of mitral regurgitation and mitral regurgitation jet area were assessed.. Results: Mitral regurgitation was seen in 70% of patients at baseline (Figure 1A). ASA significantly reduced regurgitation, with only 41% having mild or greater MR at three months (70% vs 41%, p=,0.001), and nearly eliminated ...
Although it is often considered a disease of the young, hypertrophic obstructive cardiomyopathy is commonly diagnosed in the elderly. Studies have also indicated that this condition may be more common in older patients than is generally recognised, supporting the notion that the elderly may be a large and neglected subset of patients with hypertrophic obstructive cardiomyopathy. Whiting and colleagues35 reported that 32% of patients presenting with hypertrophic cardiomyopathy were , 60 years of age, and in a community hospital based series published by Petrin and Tavel,36 83% of patients were , 50 years of age. Krasnow and Stein37 described 23 new cases of hypertrophic cardiomyopathy with 20 of the patients over the age of 50 years and 15 of these patients over the age of 60 years. Likewise, in a study by Lever and colleagues27 hypertrophic cardiomyopathy was more commonly diagnosed in patients ⩾ 65 years of age than in those , 40 years of age. Nevertheless, most information about the ...
Hypertrophic cardiomyopathy occurs as an autosomal dominant familial disorder or as a sporadic disease without familial involvement. While missense mutations in the beta cardiac myosin heavy chain (MHC) gene account for approximately half of all cases of familial hypertrophic cardiomyopathy, the molecular causes of sporadic hypertrophic cardiomyopathy are unknown. To determine whether beta cardiac MHC mutations are also associated with sporadic disease, we screened this gene in seven individuals with sporadic hypertrophic cardiomyopathy. Mutations in the beta cardiac MHC genes were identified in two probands with sporadic disease. In that their parents were neither clinically nor genetically affected, we conclude that mutations in each proband arose de novo. Transmission of the mutation and disease to an offspring occurred in one pedigree, predicting that these are germline mutations. The demonstration of hypertrophic cardiomyopathy arising within a pedigree coincident with the appearance of a ...
TY - JOUR. T1 - Apical myectomy for patients with hypertrophic cardiomyopathy and advanced heart failure. AU - Nguyen, Anita. AU - Schaff, Hartzell V. AU - Nishimura, Rick A.. AU - Geske, Jeffrey B.. AU - Dearani, Joseph A.. AU - King, Katherine S.. AU - Ommen, Steve R.. PY - 2019/1/1. Y1 - 2019/1/1. N2 - Objective: In patients with apical hypertrophic cardiomyopathy, extensive apical hypertrophy may reduce left ventricular end-diastolic volume and contribute to diastolic dysfunction, angina, and ventricular arrhythmias. Transapical myectomy to augment left ventricular cavity size can increase stroke volume and decrease left ventricular end-diastolic pressure. In this study, we describe early outcomes of patients with apical hypertrophic cardiomyopathy after transapical myectomy and compare survival with that of patients with hypertrophic cardiomyopathy listed for heart transplantation. Methods: Between September 1993 and March 2017, 113 symptomatic patients with apical hypertrophic ...
TY - JOUR. T1 - Clinical and echocardiographic predictors of outcomes in patients with apical hypertrophic cardiomyopathy. AU - Moon, Jeonggeun. AU - Shim, Chi Young. AU - Ha, Jong Won. AU - Cho, In Jeong. AU - Kang, Min Kyung. AU - Yang, Woo In. AU - Jang, Yangsoo. AU - Chung, Namsik. AU - Cho, Seung Yun. PY - 2011/12/1. Y1 - 2011/12/1. N2 - Apical hypertrophic cardiomyopathy (HC) is considered to have a favorable prognosis, but recent observations have suggested less benign clinical courses. We investigated the outcomes in patients with apical HC and evaluated the predictors. All 454 patients with apical HC (316 men, age 61 ± 11 years) were recruited. Major cardiovascular events (MACE) were defined as unplanned hospitalization because of heart failure, stroke, or cardiovascular mortality. The patients were divided into 2 groups: group 1 with MACE and group 2 without MACE. During the follow-up period (43 ± 20 months), the all-cause mortality rate was 9% (39 of 454), and 110 patients (25%) had ...
Sakamoto T, Tei C, Murayama M, Ichiyasu H, Hada Y. Giant T wave inversion as a manifestation of asymmetrical apical hypertrophy (AAH) of the left ventricle: echocardiographic and ultrasono-cardiotomographic study. Jpn Heart J. 1976;17(5):611-29. PMID: 136532. DOI: https://doi.org/10.1536/ihj.17.611 Yamaguchi H, Ishimura T, Nishiyama S, Nagasaki F, Nakanishi S, Takatsu F, et al. Hypertrophic nonobstructive cardiomyopathy with giant negative T waves (apical hypertrophy): Ventriculographic and echocardiographic features in 30 patients. Am J Cardiol. 1979;44(3):401- 12. PMID: 573056. DOI: https://doi.org/10.1016/0002-9149(79)90388-6 Eriksson MJ, Sonnenberg B, Woo A, Rakowski P, Parker TG, Wigle ED, et al. Long-term outcome in patients with apical hypertropic cardiomyopathy. J Am Cardiol. 2002;39(4):638- 45. PMID: 11849863. DOI: https://doi.org/10.1016/s0735-1097(01)01778-8 Kitaoka H, Doi Y, Casey SA, Hitomi M, Furuno T, Maron BJ. Comparision of prevalence of apical hypertrophic cardiomyopathy in ...
Intestinal angina is characterized by recurrent postprandial abdominal pain and anorexia. Commonly, these symptoms are caused by severe stenosis of at least two vessels among the celiac and mesenteric arteries. However, intestinal perfusion is affected not only by the degree of arterial stenosis but also by systemic perfusion. We experienced a unique case of intestinal angina caused by relatively mild stenosis of the abdominal arteries complicated with hypertrophic obstructive cardiomyopathy. We report an 86-year old Japanese man with hypertrophic obstructive cardiomyopathy and advanced atrioventricular block who was diagnosed with intestinal angina. Computed tomography showed mild stenosis of the celiac artery and severe stenosis of the inferior mesenteric artery, and these lesions were relatively mild compared with other reports. A dual-chamber pacemaker with right ventricular apical pacing was implanted to improve the obstruction of the left ventricular outflow tract. After implantation, the patient
TY - JOUR. T1 - Surgical myectomy improves pulmonary hypertension in obstructive hypertrophic cardiomyopathy. AU - Geske, Jeffrey B.. AU - Konecny, Tomas. AU - Ommen, Steve R.. AU - Nishimura, Rick A.. AU - Sorajja, Paul. AU - Schaff, Hartzell V.. AU - Ackerman, Michael J.. AU - Gersh, Bernard J.. N1 - Copyright: Copyright 2015 Elsevier B.V., All rights reserved.. PY - 2014/8/7. Y1 - 2014/8/7. N2 - Aims: Characterization of pulmonary hypertension (PH) and the effects of myectomy in hypertrophic cardiomyopathy (HCM) remain poorly defined. The aim of the study was to investigate the effect of myectomy on PH in HCM. Methods and results: This is a retrospective analysis of 306 consecutive symptomatic HCM patients (70% NYHA class III-IV) with evaluation of echocardiographic right ventricular systolic pressure (RVSP) both preceding (median 3 days) and following (median 4 days) myectomy. Compared with patients without PH (RVSP ,35 mmHg, n = 145, 47%), patients with moderate or severe PH (RVSP ≥50 ...
Implantation of dual chamber pacemakers for symptoms associated with hypertrophic obstructive cardiomyopathy is being evaluated as an alternative treatment. The effectiveness of this intervention involves programming pacemaker parameters specific to the individual patient. We present a case of a patient diagnosed with hypertrophic obstructive cardiomyopathy who underwent dual chamber pacemaker implantation for symptoms refractory to medical therapy. ...
RATIONALE: Most sarcomere gene mutations that cause hypertrophic cardiomyopathy are missense alleles that encode dominant negative proteins. The potential exceptions are mutations in the MYBPC3 gene (encoding cardiac myosin-binding protein-C [MyBP-C]), which frequently encode truncated proteins. OBJECTIVE: We sought to determine whether there was evidence of haploinsufficiency in hypertrophic cardiomyopathy caused by MYBPC3 mutations by comparing left ventricular muscle from patients undergoing surgical myectomy with samples from donor hearts. METHODS AND RESULTS: MyBP-C protein and mRNA levels were quantitated using immunoblotting and RT-PCR. Nine of 37 myectomy samples had mutations in MYBPC3: 2 missense alleles (Glu258Lys, Arg502Trp) and 7 premature terminations. No specific truncated MyBP-C peptides were detected in whole muscle homogenates of hypertrophic cardiomyopathy tissue. However, the overall level of MyBP-C in myofibrils was significantly reduced (P|0.0005) in tissue containing either a
Idiopathic hypertrophic subaortic stenosis (IHSS). also called hypertrophic obstructive cardiomyopathy (HOCM), is a genetic form of hypertrophic cardiomyopathy (HCM) Characterized by marked hypertrophy of the interventricular septum:. Reduces the diameter of the left ventricular outflow tract, leading to outflow tract obstruction. Overtime it leads to:. LVH and increased diastolic filling pressure. It is the most common (Mendelian) genetic heart disease, occurring at rate of approximately 1 in 500. Autosomal dominant pattern of inheritance with variable penetrance leading to a high degree of phenotypic heterogeneity, which makes genetic testing implausible Monogenic disease where in 1 of the 13 genes lead to a single amino acid change in proteins that compose the sarcomere complex Presents after puberty (Average in mid-20s, but increasingly seen in 40-50s). Common in young athletes. May present with symptoms or sudden cardiac death (SCD). Ordinarily, muscle cells line up linearly In IHSS, ...
Obstructive hypertrophic cardiomyopathy (HCOM) is known as a familial genetic disorder. The most potent risk factor in the development of hypertrophic cardiomyopathy aregenetic mutations in Beta-myosin heavy chain, Myosin binding protein C, and Cardiac troponin T. Genes involved in the pathogenesis of hypertrophic cardiomyopathy include but not limited to MYH7, TNNT2, TPM1. However, hypertension, thyroid disease, diabetes, and obesity also play a role in non obstructive forms of hypertrophic cardiomyopathy. This is in response to chronic effects of abnormal pressure and volumes on the myocardium and is different from apical hypertrophy (Yamaguchi syndrome). ...
TY - JOUR. T1 - Hypertrophic obstructive cardiomyopathy. T2 - The Mayo Clinic experience. AU - Kotkar, Kunal D.. AU - Said, Sameh M.. AU - Dearani, Joseph A.. AU - Schaff, Hartzell V.. PY - 2017/7/1. Y1 - 2017/7/1. N2 - Background: Hypertrophic cardiomyopathy (HCM) is a primary myocardial disease characterized by left ventricular hypertrophy in the absence of other etiologies. Clinical presentation may vary from asymptomatic to sudden cardiac death. Medical treatment is the first-line therapy for symptomatic patients. Extended left ventricular septal myectomy is the procedure of choice if medical treatment is unsuccessful or intolerable. Mayo Clinic experience: More than 3,000 patients have had septal myectomy for HCM at the Mayo Clinic (MN, USA) from 1993 to 2016. Risk of hospital death after isolated septal myectomy for obstructive HCM is , 1% and is similar to the risk of operation for elective mitral valve repair. Complications, such as complete heart block requiring permanent pacemaker, are ...
Three novel beta cardiac myosin heavy chain (MHC) gene missense mutations, Phe513Cys, Gly716Arg, and Arg719Trp, which cause familial hypertrophic cardiomyopathy (FHC) are described. One mutation in exon 15 (Phe513Cys) does not alter the charge of the encoded amino acid, and affected family members have a near normal life expectancy. The Gly716Arg mutation (exon 19; charge change of +1) causes FHC in three family members, one of whom underwent transplantation for heart failure. The Arg719Trp mutation (exon 19; charge change of -1) was found in four unrelated FHC families with a high incidence of premature death and an average life expectancy in affected individuals of 38 yr. A comparable high frequency of disease-related deaths in four families with the Arg719Trp mutation suggests that this specific gene defect directly accounts for the observed malignant phenotype. Further, the significantly different life expectancies associated with the Arg719Trp vs. Phe513Cys mutation (P | 0.001) support the
TY - JOUR. T1 - Benefits of intraoperative echocardiography in the surgical management of hypertrophic cardiomyopathy. AU - Marwick, Thomas H.. AU - Stewart, William J.. AU - Lever, Harry M.. AU - Lytle, Bruce W.. AU - Rosenkranz, Eliot. AU - Duffy, Carol I.. AU - Salcedo, Ernesto E.. PY - 1992/11/1. Y1 - 1992/11/1. N2 - Objectives. The purpose of this study was to determine the role of intraoperative echocardiography in planning the site and extent of myectomy and in ensuring adequate control of the left ventricular outflow tract gradient. Background. Although intraoperative echocardiography has been found to be beneficial in patients undergoing valve repair, its impact on surgical decisions in patients undergoing septal myectomy for hypertrophic cardiomyopathy has not been described. Methods. In 50 patients undergoing septal myectomy over a 5-year period, epicardial echocardiography was performed before cardiopulmonary bypass to establish the extent of outflow tract obstruction, locate its ...
Familial hypertrophic cardiomyopathy is a genetically heterogeneous disease with variable clinical features that is inherited as autosomal dominant with variable penetrance. Recent developments in genetics of hereditary cardiomyopathy have not only enlightened many points about pathogenesis, but have also provided great benefit to diagnostic approaches of clinicians. Heterozygous mutation of c3691-3692insTTCA in MYBPC3 gene was identified in a pediatric patient with diagnosis of hypertrophic cardiomyopathy at clinic. Hypertrophy was observed in sister and father of the patient in echocardiography screening, and it was subsequently determined that they also had same mutation. This mutation has not previously been defined and reported previously in the literature as cause of hypertrophic cardiomyopathy.. Keywords: Echocardiography, hypertrophic cardiomyopathy, molecular genetics, MYBPC ...
TY - JOUR. T1 - Multidetector computerized tomography can guide and document alcohol septal ablation in hypertrophic obstructive cardiomyopathy. AU - Ghersin, Eduard. AU - Soto, Victor. AU - Heldman, Alan W.. PY - 2011/1/18. Y1 - 2011/1/18. UR - http://www.scopus.com/inward/record.url?scp=78751605593&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=78751605593&partnerID=8YFLogxK. U2 - 10.1161/CIRCULATIONAHA.110.975599. DO - 10.1161/CIRCULATIONAHA.110.975599. M3 - Article. C2 - 21242503. AN - SCOPUS:78751605593. VL - 123. JO - Circulation. JF - Circulation. SN - 0009-7322. IS - 2. ER - ...
The short-term results of PTSMA are excellent for the reduction of LVOT obstruction (13,17,32). Previous studies report that mean resting PGs significantly reduced from 60 mm Hg to 14 mm Hg after PTSMA (13,17,32), and other studies with longer follow-up (16,18,33)do not show any recurrence of obstruction over time. One- to two-year follow-up shows continued improvement in symptoms with a mean increase of over one NYHA functional class (16,18,33). More importantly, objective tests show increases of exercise time around 40% over follow-up (18). Recently, Lakkis et al. (18)reported results of a one-year follow-up study in 50 patients. In his study, resting PGs dropped significantly from average 74 ± 23 mm Hg to 6 ± 18 mm Hg, and dobutamine-induced gradient decreased from 84 ± 28 mm Hg to 30 ± 33 mm Hg. The exercise duration increased by 2 min at one year. Similar results were also found in our study, with both resting and provokable PGs significantly reduced immediately after the PTSMA and ...
Myocardial disarray, also known as myocyte disarray, is a term to describe the loss of the normal parallel alignment of myocytes (the muscle cells of the heart). Instead, the myocytes usually form circles around foci of connective tissue. Myocardial disarray is associated with myocardial fibrosis (the replacement of the myocytes with non-contractile scar tissue). Myocardial disarray can be seen in a number of disease states, including: Aortic stenosis Congenital heart disease Hypertensive heart disease Hypertrophic cardiomyopathy The common factor amongst all these diseases is that they all cause varying degrees of remodelling (myocardial fibrosis) of the ventricles. Myocardial disarray. A critical review Information from the Stanford Hypertrophic Cardiomyopathy ...
OBJECTIVE: Angina and the presence of myocardial ischaemia are common in hypertrophic cardiomyopathy. Dual chamber pacing results in clinical improvement in these patients. This study evaluates the effects of permanent dual chamber pacing on absolute regional myocardial perfusion and perfusion reserve. SETTING: University hospital. PATIENTS AND DESIGN: Six patients with hypertrophic cardiomyopathy and severe symptoms of angina received a dual chamber pacemaker. Absolute myocardial regional perfusion and perfusion reserve (dipyridamole 0.56 mg/kg) were measured by dynamic positron emission tomography with 13N-ammonia both during sinus rhythm and 3 months after pacemaker insertion. Results were compared with those from 28 healthy volunteers. RESULTS: Pacing resulted in a reduction of anginal complaints and a reduction in intraventricular pressure gradient from 65 (SD 30) mm Hg to 19 (10) mm Hg. During sinus rhythm, baseline perfusion was higher in patients with hypertrophic cardiomyopathy than ...
TY - JOUR. T1 - Dual chamber pacing relieves obstruction in japanese-variant hypertrophic cardiomyopathy. AU - Wever-Pinzon, Omar. AU - Romero, Jorge E.. AU - Cordova, Juan P.. PY - 2013/9. Y1 - 2013/9. N2 - Japanese-variant or apical hypertrophic cardiomyopathy (HCM) is a specific type of HCM, first described in Japan and initially thought to carry a benign prognosis. However, current evidence suggests that these patients experience severe symptoms and are at increased risk of ventricular arrhythmias and death, especially in the presence of an apical akinetic chamber. The management of patients who do not respond to medical therapy is challenging. We describe a patient with Japanesevariant HCM, with an apical akinetic chamber and severe symptoms who failed medical therapy. The use of dual chamber pacing relieved obstruction and significantly improved the patients symptoms.. AB - Japanese-variant or apical hypertrophic cardiomyopathy (HCM) is a specific type of HCM, first described in Japan and ...
This trial will investigate efficacy, safety, tolerability and dose ranging of MYK-461 in patients with symptomatic non-obstructive hypertrophic cardiomyopathy
Steve R. Ommen: The hypertrophic cardiomyopathy is a condition that has been under-diagnosed and overly feared throughout the world. In the United States alone, there are over a half a million people that have hypertrophic cardiomyopathy, many of whom are completely asymptomatic and unaware of their diagnosis. Some people can die suddenly. Sudden cardiac death occurs randomly without warning.. Hartzell V. Schaff: Over 2/3 of the patients will have obstruction. And the obstruction to the left ventricular outflow tract is an indication for operation in patients that have symptoms. So we know now that 2/3 of the patients with hypertrophic cardiomyopathy and obstruction are candidates for surgery.. Steve R. Ommen: Hypertrophic cardiomyopathy is the most common inherited cardiomyopathy or heart muscle disease. People are born with the genetics for it, but the hypertrophy doesnt appear to start developing until adolescence, growth spurts, or beyond. It is possible for infants to born with thick heart ...
Steve R. Ommen: The hypertrophic cardiomyopathy is a condition that has been under-diagnosed and overly feared throughout the world. In the United States alone, there are over a half a million people that have hypertrophic cardiomyopathy, many of whom are completely asymptomatic and unaware of their diagnosis. Some people can die suddenly. Sudden cardiac death occurs randomly without warning.. Hartzell V. Schaff: Over 2/3 of the patients will have obstruction. And the obstruction to the left ventricular outflow tract is an indication for operation in patients that have symptoms. So we know now that 2/3 of the patients with hypertrophic cardiomyopathy and obstruction are candidates for surgery.. Steve R. Ommen: Hypertrophic cardiomyopathy is the most common inherited cardiomyopathy or heart muscle disease. People are born with the genetics for it, but the hypertrophy doesnt appear to start developing until adolescence, growth spurts, or beyond. It is possible for infants to born with thick heart ...
The study is the most extensive one so far to analyse the genetic background and outcome of hypertrophic cardiomyopathy in a nationwide cohort.. Hypertophic cardiomyopathy is estimated to occur in one in 500 adults. Among young people and athletes, it is the most common cause of sudden cardiac deaths. According to the newly published study, the prognosis of patients diagnosed with and treated for hypertrophic cardiomyopathy in Finland is good. In a seven-year follow-up, their mortality rate was low, although significantly higher than the mortality rate of the age- and sex-controlled population.. The researchers also analysed the National Cause of Death Register to explore the role of hypertrophic cardiomyopathy in all deaths occurring in Finland. Over a ten-year period, almost 600 Finns died of hypertrophic cardiomyopathy, and one third of these deaths were sudden cardiac deaths.. According to Professor Kuusisto, the high prevalence of sudden cardiac deaths indicates that cardiomyopathy remains ...
We described a patient with familial non-obstructive hypertrophic cardiomyopathy and complete atrioventricular block. A 27-year-old male was admitted to our institution with syncope. Electrocardiography demonstrated complete atrioventricular block. Two-dimensional echocardiography revealed non-obstructive hypertrophic cardiomyopathy. A temporary transvenous ventricular pacemaker was inserted urgently, and subsequently replaced by a permanent dual-chamber pacemaker. Meanwhile, non-obstructive hypertrophic cardiomyopathy was diagnosed in the mother, the aunt and one of the brothers of the patient in the screening of the family, but atrioventricular conduction block was not detected in them. In the electrophysiological study of the mother, inducible ventricular tachycardia was detected. The reason for diversity of the arrhythmias in the members of the same family with hypertrophic cardiomyopathy may be explained by penetrance. The phenotype of the familial hypertrophic cardiomyopathy is influenced ...
TY - JOUR. T1 - Exercise gas exchange analysis in obstructive hypertrophic cardiomyopathy before and after myectomy (cardiopulmonary exercise test combined with exercise-echocardiography in HCM). AU - Bandera, F.. AU - Generati, G.. AU - Pellegrino, M.. AU - Secchi, F.. AU - Menicanti, L.. AU - Guazzi, M.. PY - 2015/1/15. Y1 - 2015/1/15. KW - Cardiopulmonary exercise test. KW - Exercise intolerance. KW - Exercise-echocardiography. KW - Hypertrophic cardiomyopathy. KW - Septal resection. UR - http://www.scopus.com/inward/record.url?scp=84916213770&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84916213770&partnerID=8YFLogxK. U2 - 10.1016/j.ijcard.2014.11.104. DO - 10.1016/j.ijcard.2014.11.104. M3 - Article. C2 - 25465307. AN - SCOPUS:84916213770. VL - 178. SP - 282. EP - 283. JO - International Journal of Cardiology. JF - International Journal of Cardiology. SN - 0167-5273. ER - ...
DISCUSSION. Recently, the advances in cardiopulmonary bypass and myocardial protection associated with the improvement of the surgical technique, and the postoperative cardiovascular surgery has caused the morbidity and mortality of septal myectomy to become progressively decreased. Currently, assistance units with extensive experience present a mortality rate between 1% and 2%, being able to reach zero in most favorable situations (9,12,13). In our experience, we had an in-hospital death (2.9%) and a survival rate of 87.9% with a mean follow-up of 9.6 years, equivalent to that observed in the international experience, with an 5-year overall survival ranging from 86% up to 96% and in 10 years between 70% and 90% [9,10,12,13]. The recent consensus of American and European Societies of Cardiology have highlighted the surgical septal myectomy as the gold standard to reduce the left ventricular outflow obstruction tract and to relief the symptoms in patients with hypertrophic obstructive ...
Hypertrophic cardiomyopathy is a genetic disease in which the heart muscle grows abnormally, making the heart muscle thicken. Hypertrophic cardiomyopathy is described as either obstructive or nonobstructive. Nonobstructive hypertrophic cardiomyopathy. The heart muscle is abnormally thick but not to...
Presented by: Angelos G. Rigopoulos,1,2 Hubert Seggewiss,1 Ioannis C. Rizos2. 11st Department of Internal Medicine, Leopoldina Hospital, Schweinfurt, Germany. 22nd Department of Cardiology, University of Athens Medical School, Athens, Greece. Alcohol septal reduction in hypertrophic obstructive cardiomyopathy (HOCM) is indicated for patients with drug refractory symptoms or drug intolerance who have significant left ventricular obstruction.(1) On the other hand, several structural and functional changes including low-normal left ventricular (LV) ejection fraction, moderate to severe diastolic dysfunction, marked atrial dilatation, thinning of the LV walls, onset of atrial fibrillation, spontaneous reduction or loss of LV outflow obstruction, and LV apical aneurysms characterise an advanced stage of progression in the physical history of the disease which signifies adverse remodelling and clinical deterioration.(2) The benefit derived from septal reduction treatments in this stage is largely ...
TY - JOUR. T1 - Apical hypertrophic cardiomyopathy. AU - Usry, Courtney R.. AU - Javaheri, Sean Payman. PY - 2015/12/1. Y1 - 2015/12/1. UR - http://www.scopus.com/inward/record.url?scp=84954560667&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=84954560667&partnerID=8YFLogxK. M3 - Article. AN - SCOPUS:84954560667. VL - 55. JO - Consultant. JF - Consultant. SN - 0010-7069. IS - 12. ER - ...
BACKGROUND: Electrocardiographic (ECG) fusion with intrinsic QRS could reduce the benefit of atrial synchronous biventricular pacing (AS-BiVP) in patients with hypertrophic obstructive cardiomyopathy (HOCM). OBJECTIVES: The purpose of this study was to assess the benefit of AS-BiVP and the influence of ECG fusion for reduction of left ventricular outflow tract gradient (LVOTG) in these patients. METHODS: Twenty-one symptomatic HOCM patients with severe LVOTG were included. Twelve patients were evaluated retrospectively for the prevalence of fusion and its influence on outcomes after AS-BiVP. Eleven patients (2 of the first population were also evaluated retrospectively) were prospectively included to evaluate the benefit of performing atrioventricular node ablation (AVNA) to achieve full ventricular capture if fusion was present during AS-BiVP. RESULTS: Seven of the first 12 patients (58%) had ECG fusion. After 54 +/- 24 months of AS-BiVP, the presence of fusion was associated with lower values ...
Store Levitra Super Active+ at room temperature, between 68 and 77 degrees F (20 and 25 degrees C). Store away from heat, moisture, and light. Keep Levitra Super Active+ out of the reach of children and away from pets.. Warnings/Precautions. Levitra Super Active+ has vasodilator properties which may result in mild and transient decreases in blood pressure. Patients with left ventricular outflow obstruction, e.g., aortic stenosis and idiopathic hypertrophic subaortic stenosis, can be sensitive to the action of vasodilators including Type 5 phosphodiesterase inhibitors ...
Global Hypertrophic Cardiomyopathy Therapeutics Market - Key Trends With heart diseases emerging as one of the most common causes of mortality among men and women worldwide, Transparency Market Research (TMR) expects the demand for hypertrophic cardiomyopathy (HCM) therapeutics to surge considerably. Furthermore, the market is expected to gain significant impetus from successful government interventions aimed at spreading awareness about hypertrophic cardiomyopathy.. View Report-. https://www.transparencymarketresearch.com/hypertrophic-cardiomyopathy-therapeutics-market.html. TMR projects the global HCM therapeutics market to expand at a moderate 1.4% CAGR between 2015 and 2023. Despite witnessing positive opportunities, the rising demand for advanced medical devices could threaten the markets growth to an extent. Nevertheless, since the majority of HCM drugs are yet to get approved, the hypertrophic cardiomyopathy therapeutics market is likely to gain momentum post their approval in the near ...
0002] Hypertrophic cardiomyopathy (HCM) is an often fatal but manageable disease. The incidence is reported to be about 1/400 (approximately 750,000) in the general U.S. population. The variable expressivity of this disease suggests it may be higher, making HCM the most common monogenic cardiac disorder in the U.S. Macon and McKenna et al., ACC/ESC Expert Consensus Document on Hypertrophic Cardiomyopathy, J of American College of Cardiology (2003) 42: 1-27. In addition, it is the most frequent cause of unexpected sudden death in teenagers and young adults. Elliott, Poloniecki et al., Sudden death in hypertrophic cardiomyopathy: Identification of high risk patients, J of American College of Cardiology (2000) 36: 2212-2218. The disease is characterized by a thickening of the heart muscle (hypertrophy) in the absence of hypertension or any other apparent cause. HCM is difficult to diagnose. Clinical presentation and progression of HCM varies widely among affected patients and the symptoms ...
IVS hypertrophy can occur as part of generalized LV hypertrophy, or it can occur in isolation, such as in asymmetric septal hypertrophy (ASH).5 In ASH, the ratio of the IVS thickness to the inferolateral (posterior) wall thickness is ,1.3. Although ASH is a characteristic feature of hypertrophic cardiomyopathy, it can also occur in the elderly due to hypertension or aortic stenosis. Excessive thickening can lead to LVOT obstruction in systole: this condition is termed hypertrophic obstructive cardiomyopathy.5 This obstruction can be further worsened with concomitant systolic anterior motion of the anterior leaflet of the mitral valve.. Surgical repair of hypertrophic cardiomyopathy with septal myectomy is generally performed for patients with severe LVOT obstruction: a gradient at rest ,30 mm Hg, IVS thickness ,18 mm, and evidence of systolic anterior motion.6 During septal myectomy, TEE can be used to evaluate the extent of resection required, determine the presence and severity of mitral ...
Large septal Q waves may be present reflective of the septal hypertrophy. In the Yamaguchi variant of apical hypertrophic cardiomyopathy, there may be deeply inverted T waves in precordial leads V2-V6 and II, III, aVL (see example). Shown below is an example of a variant of apical hypertrophic cardiomyopathy with deeply inverted T waves in precordial leads V2-V6 and II, III, aVL. ...
Indications for Drugs ::. Mild to moderate hypertension, Chronic stable and vasospastic angina, Raynauds disease, Coronary Artery Disease, HTN, Stroke prevention. Drug Dose ::. Adult: PO Stable angina; HTN; Prinzmetals angina Initial: 5 mg once daily; If the desired therapeutic effect cannot be achieved within 2-4 weeks, the dose may be increased to a maximum dose of 10 mg once daily. Child: >12 yr Initially, 2.5 mg once daily, increased to 5 mg once daily if necessary. Elderly: Initially, 2.5 mg once daily. Hepatic impairment: Initially, 2.5 mg once daily.. Contraindication ::. Hypersensitivity to Amlodipine or other calcium channel antagonist, Severe hypertension.. Drug Precautions ::. Impaired liver or renal function, CHF, sick-sinus syndrome, severe ventricular dysfunction, hypertrophic cardiomyopathy, severe aortic stenosis. Caution when used in patients with idiopathic hypertrophic subaortic stenosis. Elderly, children. Pregnancy, lactation. Use in renal failure: Although Amlodipine is ...
Indications for Drugs ::. Mild to moderate hypertension, Chronic stable and vasospastic angina, Raynauds disease, Coronary Artery Disease, HTN, Stroke prevention. Drug Dose ::. Adult: PO Stable angina; HTN; Prinzmetals angina Initial: 5 mg once daily; If the desired therapeutic effect cannot be achieved within 2-4 weeks, the dose may be increased to a maximum dose of 10 mg once daily. Child: >12 yr Initially, 2.5 mg once daily, increased to 5 mg once daily if necessary. Elderly: Initially, 2.5 mg once daily. Hepatic impairment: Initially, 2.5 mg once daily.. Contraindication ::. Hypersensitivity to Amlodipine or other calcium channel antagonist, Severe hypertension.. Drug Precautions ::. Impaired liver or renal function, CHF, sick-sinus syndrome, severe ventricular dysfunction, hypertrophic cardiomyopathy, severe aortic stenosis. Caution when used in patients with idiopathic hypertrophic subaortic stenosis. Elderly, children. Pregnancy, lactation. Use in renal failure: Although Amlodipine is ...
Compared to men, women with a hereditary heart condition called hypertrophic cardiomyopathy are substantially more likely to be diagnosed later in life and with more severe symptoms, an Italian study indicates. This occurs despite the fact that hypertrophic cardiomyopathy should theoretically be present in males and females equally, Dr. Iacopo Olivotto commented to Reuters Health, because it is a genetic disease with an inheritance pattern that requires only one parent to have the condition. People with hypertrophic cardiomyopathy (HCM) suffer from progressive weakening of the heart, which becomes enlarged in an attempt to compensate ...
Patients with hypertrophic cardiomyopathy are not immune from nationwide problems of inactivity and obesity. This study suggests that these issues may in fact be exaggerated in patients with hypertrophic cardiomyopathy. This may be due to apprehension regarding exercise on the part of patients, as well as by exercise restrictions imposed by physicians. Well-designed, likely multicenter, prospective studies will be necessary to determine the risks and benefits of different exercise regimens on hypertrophic cardiomyopathy disease progression and complications.. ...
Hypertrophic obstructive cardiomyopathy (HOCM) answers are found in the Diagnosaurus powered by Unbound Medicine. Available for iPhone, iPad, Android, and Web.
Familial Hypertrophic Cardiomyopathy Type 13 (Hypertrophic Cardiomyopathy 13): Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis.
OBJECTIVES: The purpose of this study was to determine the spectrum of left ventricular hypertrophy and ventricular morphology in adults with hypertrophic cardiomyopathy due to mutations of the beta-myosin heavy-chain gene. BACKGROUND: Although echocardiography is an important test in diagnosing hypertrophic cardiomyopathy, the lack of an independent diagnostic criterion has been an obstacle in determining the full echocardiographic spectrum of this disease. Mutations in the beta-myosin heavy chain gene occur in approximately 50% of familial cases; in members of families with a known mutation, the diagnosis can be made with certainty. METHODS: Echocardiograms from 39 genetically affected and 30 genetically unaffected adult family members over age 16 years from 10 families were analyzed. Left ventricular wall thickness was measured at 10 separate locations, and the presence of systolic anterior motion of the mitral valve, right ventricular hypertrophy and left ventricular morphology was evaluated
Comment from Dr. Jennifer Johnson (Pittsburgh, PA), Section Editor of Pediatric Cardiology Journal Watch: This is a single center retrospective chart review of all pediatric hypertrophic cardiomyopathy patients who underwent exercise stress echocardiography to determine if exercise stress echocardiography can be useful in risk stratifying hypertrophic cardiomyopathy subgroups and if LVOT obstruction occurs in the patients with nonobstructive hypertrophic cardiomyopathy.. Methods: Data was collected on all pediatric hypertrophic cardiomyopathy patient who underwent exercise stress echocardiography from 2007-2018 at Boston Childrens Hospital. Subjects were assigned to one of three categories based on left ventricular outflow tract gradients: group 1: ,30 mm Hg at rest and exercise; group 2: ,30 mm Hg at rest and ≥30 mm Hg with exercise; and group 3: ≥ 30 mm Hg at rest and exercise. The composite adverse endpoints on follow-up included heart transplant, aborted cardiac arrest, and sudden ...
Looking for online definition of Feline hypertrophic cardiomyopathy in the Medical Dictionary? Feline hypertrophic cardiomyopathy explanation free. What is Feline hypertrophic cardiomyopathy? Meaning of Feline hypertrophic cardiomyopathy medical term. What does Feline hypertrophic cardiomyopathy mean?
Decreased expression of tumor necrosis factor-α and regression of hypertrophy after nonsurgical septal reduction therapy for patients with hypertrophic obstructive cardiomyopathy Academic Article ...
TY - JOUR. T1 - Aborted sudden cardiac death in a young soldier with concomitant hypertrophic cardiomyopathy and Wolff-Parkinson-White syndrome. AU - Kim, Sunwon. AU - Song, Woo Hyuk. AU - Kim, Sung Soon. PY - 2019/2/1. Y1 - 2019/2/1. N2 - We present a young soldier presenting with aborted sudden cardiac death, who was found to have concomitant hypertrophic cardiomyopathy and Wolff-Parkinson-White syndrome. Along with pathological haemodynamic features of hypertrophic cardiomyopathy, an easily-inducible re-entrant tachycardia was clearly documented in our patient. Given the fatal potential of supraventricular tachycardia in hypertrophic cardiomyopathy, we postulated that his tachyarrhythmia could potentially trigger the event. Upon his refusal to receive implantable cardioverter/defibrillator therapy, we ablated anatomical arrhythmogenic substrate instead, and he remained uneventfully over 3 years on β-blocker.. AB - We present a young soldier presenting with aborted sudden cardiac death, who ...
This paper describes the case of a former professional athlete who developed an apical hypertrophic cardiomyopathy (aHMC) apparently after a severe traumatic event at the age of 46 years. The aHCM can phenotypically appear in a relatively advanced age and the typical electrocardiographic abnormalities usually become visible before hypertrophy is evident at ultrasound or magnetic resonance imaging. Several studies show associations between various forms of surgical stress or trauma and myocardial lesions or cardiovascular disease, suggesting the role of sudden catecholaminergic stimulation in aHCM development in genetically predisposed subjects. Late onset of aHCM and late mean age of presentation may be an example of gene-environment interaction needed to reveal latent genotype manifestations.. ...
{ consumer: Some people who have hypertrophic cardiomyopathy are at high risk for sudden death. It can occur at any age, but it is most shocking when it happens to young adults or athletes. While the media often highlight these tragic deaths, sudden death is rare. It occurs in about 1 out of 100 adults with hypertrophic..., clinical: Some people who have hypertrophic cardiomyopathy are at high risk for sudden death. It can occur at any age, but it is most shocking when it happens to young adults or athletes. While the media often highlight these tragic deaths, sudden death is rare. It occurs in about 1 out of 100 adults with hypertrophic... } Region 6, Nebraska
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Also called hypertrophic cardiomyopathy, asymmetric septal hypertrophy is a condition that occurs when heart muscles cells enlarge, causing the walls of the lower heart chambers (typically the left ventricle) to become thick and stiff. This makes it difficult for the heart to relax and for a sufficient amount of blood to fill the heart chambers. While the heart squeezes normally, the limited filling prevents the heart from pumping enough blood, especially during physical activity. Children with asymmetric septal hypertrophy are not allowed to play competitive sports because of the possibility of a sudden collapse or increased heart failure.. ...
Cardiac myosin binding protein C phosphorylation affects cross-bridge cycles elementary steps in a site-specific manner.: Based on our recent finding that card
definition of ASH, what does ASH mean?, meaning of ASH, Asymmetric Septal Hypertrophy, ASH stands for Asymmetric Septal Hypertrophy
TY - JOUR. T1 - Preventative therapeutic approaches for hypertrophic cardiomyopathy. AU - Solomon, Tanya. AU - Filipovska, Aleksandra. AU - Hool, Livia. AU - Viola, Helena. PY - 2020/8/21. Y1 - 2020/8/21. N2 - Sarcomeric gene mutations are associated with the development of hypertrophic cardiomyopathy (HCM). Current drug therapeutics for HCM patients are effective in relieving symptoms, but do not prevent or reverse disease progression. Moreover, due to heterogeneity in the clinical manifestations of the disease, patients experience variable outcomes in response to therapeutics. Mechanistically, alterations in calcium handling, sarcomeric disorganization, energy metabolism and contractility participate in HCM disease progression. While some similarities exist, each mutation appears to lead to mutation-specific pathophysiology. Furthermore, these alterations may precede or proceed development of the pathology. This review assesses the efficacy of HCM therapeutics from studies performed in animal ...
Left ventricular outflow tract obstruction (LVOTO) is an important source of morbidity and mortality after repair of atrioventricular septal defect (AVSD). The intrinsic anatomy of the left ventricular outflow tract in AVSD is complex and predisposes
TY - JOUR. T1 - Implications of arrhythmias and prevention of sudden death in hypertrophic cardiomyopathy. AU - Adabag, A. Selcuk. AU - Maron, Barry J.. N1 - Copyright: Copyright 2011 Elsevier B.V., All rights reserved.. PY - 2007/4. Y1 - 2007/4. KW - Arrhythmia. KW - Holter monitoring. KW - Hypertrophic cardiomyopathy. KW - Sudden death. UR - http://www.scopus.com/inward/record.url?scp=34250205997&partnerID=8YFLogxK. UR - http://www.scopus.com/inward/citedby.url?scp=34250205997&partnerID=8YFLogxK. U2 - 10.1111/j.1542-474X.2007.00157.x. DO - 10.1111/j.1542-474X.2007.00157.x. M3 - Review article. C2 - 17593187. AN - SCOPUS:34250205997. VL - 12. SP - 171. EP - 180. JO - Annals of Noninvasive Electrocardiology. JF - Annals of Noninvasive Electrocardiology. SN - 1082-720X. IS - 2. ER - ...
U.S., June 13 -- ClinicalTrials.gov registry received information related to the study (NCT03178357) titled Cardiac Rehabilitation in Patients With HCM Without LV Outflow Tract Obstruction With Preserved EF - Pilot Study on June 5. Brief Summary: Hypertrophic cardiomyopathy (HCM) is the most common hereditary disease characterized by left ventricular hypertrophy and consequently left ventricular diastolic function. Its prevalence is estimated at around 0.2% in the general population. HCM is the most common cause of sudden cardiac death due to cardiovascular disease in young athletes, accounting for one third of deaths. HCM patients often have symptoms of heart failure. The ESC recommendations for heart failure (HF) from 2016 recommend exercise training regardless of ejection fraction to improve exercise capacity, quality of life, and reduction in hospitalizations due to HF. Meanwhile, for many years, HCM was equivalent to exercise training limitation. According to the 2014 ESC guidelines, it ...
Take Home Points: Hypertrophic Cardiomyopathy in the Elderly + Review of Intracardiac Pressures Hypertrophic Cardiomopathy - Not one size fits all! Definition - a genetically determined disease of the myocardium that causes different types of LV hypertrophy Types of HCM - though we may associate HCM with LV outflow tract (LVOT) obstruction or sudden cardiac…
INTRODUCTION: The implantation of a cardioverter-defibrillator (ICD) is an established method of sudden cardiac death (SCD) prevention. The value of ICD therapy in secondary prevention of SCD is unquestionable. Precise identification of high-risk patients and ICD use for primary prevention of SCD, especially in patients with hypertrophic cardiomyopathy (HCM), remain controversial. Problems include the high prevalence of complications associated with ICD implantation and optimal selection of ICDs.. AIM: To estimate the frequency and type of complications after ICD implantations in HCM patients in a long-term follow-up.. METHOD: The efficacy and safety of ICD therapy were estimated in 46 HCM patients with devices implanted for a secondary (n-18) or primary prevention (n-28) of SCD.. RESULTS: During the mean follow-up period of 28.2+/-26.1 months (from 2 to 68) appropriate ICD interventions occurred in 10 (55%) patients of the secondary prevention group and in 3 (10%) patients of the primary ...
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Medical definition of hypertrophic cardiomyopathy: cardiomyopathy that is characterized by ventricular hypertrophy especially of the left ventricle…
Autori: Dermengiu D, Ceausu M, Rusu MC, Dermengiu S, Curca GC, Hostiuc S. Editorial: Rom J Leg Med , 18(1), p.3-12, 2010.. Rezumat:. We report a case of sudden death in a 20 years old male who colapsed just minues after the beggining of a football training session. The autopsy evidenced the presence of a unique combination of coronary abnormalities: myocardial bridging at the level of both branches of the LCA; abnormal origin of the right coronary artery: 1 mm above the left semilunar valve of aorta; the initial segment of the RCA coursing within the aortic wall (0,7 cm); myocardial bridging at the level of LCX; sinoatrial node artery originated from the LCX. Histological examination revealed the presence of Hypertrophic Cardiomyopathy markers within the left ventricle and interventricular septum and the cumulative effects of the coronary cardiac anomalies on the myocardial blood flow: extensive interstitial and perivascular sclerolipomatosis, dissecting fibrosis at the level of the sinoatrial ...
Background: ICDs efficiently terminate life-threatening arrhythmias, but complications occur during long-term follow-up. Patients own perspective is largely unknown. The aim of the study was to describe experiences of hypertrophic cardiomyopathy (HCM) patients with implantable defibrillators (ICDs).. Methods: We analyzed 26 Swedish patient interviews using hermeneutics and latent content analysis.. Results: Patients (aged 27-76 years) were limited by HCM especially if it deteriorates into heart failure. The ICD implies safety, gratitude, and is accepted as a part of the body even when inappropriate ICD shocks are encountered. Nobody regretted the implant. Both the disease and the ICD affected professional life and leisure time activities, especially at younger ages. Family support was usually strong, but sometimes resulted in overprotection, whereas health care focused on medical issues. Despite limitations, patients adapted, accepted, and managed challenges.. Conclusion: HCM patients with ICDs ...
Light-chain (AL) amyloidosis is the most common type of amyloidosis; cardiac involvement is rare but has a poor prognosis. Biventricular hypertrophic cardiomyopathy is an exceptional finding in amyloidosis and its association with obstructive right ventricular gradient is even rarer. We report the case of a male patient with biventricular hypertrophy suggesting amyloidosis, with an obstructive gradient in the right ventricle.. ...
The results show that hypertrophic cardiomyopathy was an uncommon cause of death in these young competitive athletes and suggest that the identification and disqualification of affected athletes at screening before participation in competitive sports may have prevented sudden death.
Fitness gains are possible without exercise pains. This website is designed to show you how to maintain heart health through step-by-step videos, key medical information and a proven exercise program. Blending the best in cardiac medicine with the latest fitness practices, we have designed a safe and effective series of exercises that can be done individually or as part of a program. Created specifically for hypertrophic cardiomyopathy (HCM) patients, our Beginner, Intermediate or Advanced HCM programs can be followed by anyone seeking to improve their overall fitness. We invite you to try our holistic approach to exercise created to promote well-being, resilience to stress and a happy, healthy heart.. ...
TY - JOUR. T1 - The metabolome in finnish carriers of the MYBPC3-Q1061X mutation for hypertrophic cardiomyopathy. AU - Jørgenrud, Benedicte. AU - Jalanko, Mikko. AU - Heliö, Tiina. AU - Jääskeläinen, Pertti. AU - Laine, Mika. AU - Hilvo, Mika. AU - Nieminen, Markku S.. AU - Laakso, Markku. AU - Hyötyläinen, Tuulia. AU - Oresic, Matej. AU - Kuusisto, Johanna. N1 - CO:U Hormone Laboratory, Aker Hospital, Oslo University Hospital, Oslo, Norway CO:U Division of Women and Childrens Health, Department of Pediatric Research, Oslo University Hospital, Oslo, Norway CO:K Helsinki University Central Hospital, Department of Cardiology, Helsinki, Finland CO:K Kuopio University Hospital, Heart Center, Kuopio, Finland CO:K University of Eastern Finland, Kuopio University Hospital, Department of Medicine, Kuopio, Finland CO:U Steno Diabetes Center, Gentofte, Denmark CA2: BA3112 CA2: BA116 AU2: Hilvo, Mika AU2: Hyötyläinen, Tuulia AU2: Oresic, Matej ISI: MULTIDISCIPLINARY SCIENCES. PY - 2015. Y1 - ...
Hypertrophic cardiomyopathy (HCM) is a primary myocardial disorder with an autosomal dominant pattern of inheritance that is characterized by hypertrophy of the left ventricles with histological features of myocyte hypertrophy, myfibrillar disarray, and interstitial fibrosis. HCM is one of the most common inherited cardiac disorders, with a prevalence in young adults of 1 in 500. Hundreds of mutations in the genes that encode protein constituents of the sarcomere have been identified in HCM. These mutations increase the Ca2+ sensitivity of cardiac myofilaments. Increased myofilament Ca2+ sensitivity is expected to increase the ATP utilization by actomyosin at submaximal Ca2+ concentrations, which might cause an imbalance in energy supply and demand in the heart under severe stress. The inefficient use of ATP suggests that an inability to maintain normal ATP levels could be the central abnormality. This theory might be supported by the discovery of the role of a mutant PRKAG2 gene in HCM, which ...
Myosin light chain kinase (MLCK)-dependent phosphorylation of the regulatory light chain (RLC) of cardiac myosin is known to play a beneficial role in heart disease, but the idea of a phosphorylation-mediated reversal of a hypertrophic cardiomyopathy (HCM) phenotype is novel. Our previous studies on transgenic (Tg) HCM-RLC mice revealed that the D166V (Aspartate166 →Valine) mutation-induced changes in heart morphology and function coincided with largely reduced RLC phosphorylation in situ. In this paper, we hypothesized that the introduction of a constitutively phosphorylated Serine15 (S15D) into the hearts of D166V mice would prevent the development of a deleterious HCM phenotype. In support of this notion, MLCK-induced phosphorylation of D166V-mutated hearts was found to rescue some of their abnormal contractile properties. Tg-S15D-D166V mice were generated with the human cardiac RLC-S15D-D166V construct substituted for mouse cardiac RLC and were subjected to functional, structural, and ...
Use to determine etiology of hypertrophic cardiomyopathy (HCM) in symptomatic individuals. Useful for presymptomatic testing in individuals with a family history of HCM or sudden cardiac death.
We report three families presenting with hypertrophic cardiomyopathy, lactic acidosis, and multiple defects of mitochondrial respiratory chain (MRC) activities. By direct sequencing of the candidate gene MTO1, encoding the mitochondrial-tRNA modifier 1, or whole exome sequencing analysis, we identified novel missense mutations. All MTO1 mutations were predicted to be deleterious on MTO1 function. Their pathogenic role was experimentally validated in a recombinant yeast model, by assessing oxidative growth, respiratory activity, mitochondrial protein synthesis, and complex IV activity. In one case, we also demonstrated that expression of wt MTO1 could rescue the respiratory defect in mutant fibroblasts. The severity of the yeast respiratory phenotypes partly correlated with the different clinical presentations observed in MTO1 mutant patients, although the clinical outcome was highly variable in patients with the same mutation and seemed also to depend on timely start of pharmacological ...
Rapidly determining the complex genetic basis of Hypertrophic cardiomyopathy (HCM) is vital to better understanding and optimally managing this common polygenetic cardiovascular disease. A rapid custom Ion-amplicon-resequencing assay, covering 30 commonly affected genes of HCM, was developed and validated in 120 unrelated patients with HCM to facilitate genetic diagnosis of this disease. With this HCM-specific panel and only 20 ng of input genomic DNA, physicians can, for the first time, go from blood samples to variants within a single day. On average, this approach gained 595628 mapped reads per sample, 95.51% reads on target (64.06 kb), 490-fold base coverage depth and 93.24% uniformity of base coverage in CDS regions of the 30 HCM genes. After validation, we detected underlying pathogenic variants in 87% (104 of 120) samples. Tested seven randomly selected HCM genes in eight samples by Sanger sequencing, the sensitivity and false-positive-rate of this HCM panel was 100% and 5%, respectively. This
Hypertrophic Cardiomyopathy; Springer UK; https://www.amazon.co.uk/Hypertrophic-Cardiomyopathy-Srihari-Naidu/dp/1447149556 ... Hypertrophic cardiomyopathy is the most common cause of sudden cardiac death among athletes, and a cause of heart failure at ... A co-author on the 2011 ACCF/AHA National Guideline on the Diagnosis and Management of Hypertrophic Cardiomyopathy, he is ... Naidu is Director of the Cardiac Cath Lab and Hypertrophic Cardiomyopathy Center - with offices in Long Island and Westchester ...
Primary disease of the muscle of the heart that cause LVH are known as hypertrophic cardiomyopathies, which can lead into heart ... "Hypertrophic cardiomyopathy". Lancet. Elsevier BV. 381 (9862): 242-255. doi:10.1016/s0140-6736(12)60397-3. ISSN 0140-6736. PMID ... LVH is used in the staging and risk stratification of Non-ischemic cardiomyopathies such as Fabry's Disease. Patients with LVH ...
"Feline Hypertrophic Cardiomyopathy (HCM)". MedVet Medical & Cancer Centers for Pets. 2 December 2016. Retrieved 31 October 2019 ... The breed does have instances of the genetic disorder hypertrophic cardiomyopathy (HCM). Other domestic cat breeds prone to HCM ... "Hypertrophic Cardiomyopathy". Cornell University College of Veterinary Medicine. January 2014. Retrieved 11 January 2020. ... Kittleson, Mark D.; Meurs, Kathryn M.; Harris, Samantha P. (December 2015). "The Genetic Basis of Hypertrophic Cardiomyopathy ...
Kraft Theresia; Montag Judith; Radocaj Ante; Brenner Bernhard (2016-10-14). "Hypertrophic Cardiomyopathy". Circulation Research ... he started to study mutations in cardiac myosin related to hypertrophic cardiomyopathy. He died in 2017, from cancer. Yu, L.C ...
"Dilated Cardiomyopathy". The Lecturio Medical Concept Library. Retrieved 25 August 2021. "Hypertrophic Cardiomyopathy". The ... Hypertrophic cardiomyopathy is typically an inherited condition. Treatments for cardiomegaly include a combination of ... Types...Hypertrophic cardiomyopathy, Left ventricular hypertrophy (LVH), Intense, prolonged athletic training Hershberger, Ray ... Maron, Barry J; Maron, Martin S (January 2013). "Hypertrophic cardiomyopathy". The Lancet. 381 (9862): 242-255. doi:10.1016/ ...
Hypertrophic cardiomyopathy (HCM) is a major concern in the Bengal cat breed. This is a disease in which the heart muscle ( ... "Genetics: Bengal Cat Hypertrophic Cardiomyopathy Study". CVM.NCSU.edu. College of Veterinary Medicine, North Carolina State ... "Hypertrophic cardiomyopathy". MayoClinic.org. Mayo Clinic. Archived from the original on September 15, 2018. Retrieved January ... Bengal cats which are used for breeding should be screened annually to ensure that no hypertrophic cardiomyopathy is present. ...
"Familial hypertrophic cardiomyopathy". Genetics Home Reference. U.S. National Library of Medicine. This article incorporates ... and hypertrophic cardiomyopathy. A patient with a 4269A>G mutation in MT-TI was found with the deficiency. Anderson S, Bankier ... causing hypertrophic cardiomyopathy". Human Mutation. 8 (3): 216-22. doi:10.1002/(SICI)1098-1004(1996)8:3. 3.0.CO;2-7. PMID ... "A homoplasmic mitochondrial transfer ribonucleic acid mutation as a cause of maternally inherited hypertrophic cardiomyopathy ...
Hypertrophic cardiomyopathy is a condition of the cardiac muscle which grows abnormally thick in the absence of a ... Hypertrophic cardiomyopathy". In Raja, Shahzad G. (ed.). Cardiac Surgery: A Complete Guide. Switzerland: Springer. pp. 735-748 ... February 2005). "Hypertrophic obstructive cardiomyopathy: comparison of outcomes after myectomy or alcohol ablation adjusted by ... In a large subset of patients with hypertrophic obstructive cardiomyopathy, thickening of the heart muscle in a particular part ...
Mutations in the MT-TG gene has also been associated with familial hypertrophic cardiomyopathy. Familial hypertrophic ... "Familial hypertrophic cardiomyopathy". Genetics Home Reference. U.S. National Library of Medicine. Merante F, Tein I, Benson L ... A family with a transition mutation of 9997T>C in the MT-TG gene exhibited familial hypertrophic cardiomyopathy. Anderson S, ... "Maternally inherited hypertrophic cardiomyopathy due to a novel T-to-C transition at nucleotide 9997 in the mitochondrial tRNA( ...
"Feline Hypertrophic Cardiomyopathy". Cat Fanciers' Association. Archived from the original on 13 May 2008. Retrieved 24 ... "Hypertrophic Cardiomyopathy Genetic Mutation Testing Service for Cats". Washington State University. Archived from the original ... The most severe threat is feline hypertrophic cardiomyopathy (HCM), the most common heart disease seen in cats, whether ... "Frequently Asked Questions about the test for the Hypertrophic Cardiomyopathy Mutation". Washington State University. Archived ...
Clinical course of hypertrophic cardiomyopathy in a regional United States cohort. Maron BJ, Casey SA, Poliac LC, Gohman TE, ... Hypertrophic obstructive cardiomyopathy. Veselka J, Anavekar NS, Charron P Lancet. 2017;389(10075):1253. Epub 2016 Nov 30. ... Hypertrophic obstructive cardiomyopathy. Veselka J, Anavekar NS, Charron P. Lancet. 2017;389(10075):1253. Epub 2016 Nov 30. ... Outcome of patients with hypertrophic cardiomyopathy and a normal electrocardiogram. McLeod CJ, Ackerman MJ, Nishimura RA, ...
Hypertrophic cardiomyopathy". In Raja, Shahzad G. (ed.). Cardiac Surgery: A Complete Guide. Switzerland: Springer. pp. 735-748 ... Septal myectomy is a cardiac surgery treatment for hypertrophic cardiomyopathy (HCM). The open-heart surgery entails removing a ... "Hypertrophic obstructive cardiomyopathy: comparison of outcomes after myectomy or alcohol ablation adjusted by propensity score ... "Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy". J Am Coll ...
... hypertrophic cardiomyopathy (HCM) also referred to as hypertrophic obstructive cardiomyopathy (HOCM) is more likely to happen ... "Hypertrophic cardiomyopathy - Medical Encyclopedia". Medline Plus. National Library of Medicine. Retrieved 2015-10-03. Meet the ... Castro, M (2006). "Mitochondrial DNA haplogroups in Spanish patients with hypertrophic cardiomyopathy". Int J Cardiol. 112 (2 ...
... hypertrophic cardiomyopathy/obstructive cardiomyopathy). It necessitated hospitalization during his tenure as Defense Secretary ...
A patient with hypertrophic cardiomyopathy. Jung Bo-min as Yang Seon-ae Yoon Mi-sun's daughter. Kim Dae-geon as Do Jin-woo Yang ...
"Hypertrophic cardiomyopathy: MedlinePlus Medical Encyclopedia". Nlm.nih.gov. Retrieved March 8, 2015. The two part video can be ... On the night of December 25, 2011, he died from complications of hypertrophic cardiomyopathy at the age of 18. Ben Breedlove ... Hypertrophic Cardiomyopathy Association OurAdvice4You at YouTube.com BreedloveTV at YouTube.com TotalRandomness512 at YouTube. ... "HCMA Remembers Ben Breedlove and Encourages You to Get the Facts on Hypertrophic Cardiomyopathy (HCM)". Market Watch. January 5 ...
... and Their Association with Hypertrophic Cardiomyopathy" (PDF). Vetogene.it. Retrieved 15 December 2017. "Hypertrophic ... Hypertrophic cardiomyopathy (HCM) is a common heart disease in all cats and is most commonly genetic in cause. The disease ... cardiomyopathy (HCM) in cats". Fabcats.org. Retrieved 15 December 2017. Wikimedia Commons has media related to Ragdoll cats. ...
The E101K missense mutation has been associated with Hypertrophic Cardiomyopathy and Left Ventricular Noncompaction. Another ... "Inherited and de novo mutations in the cardiac actin gene cause hypertrophic cardiomyopathy". Journal of Molecular and Cellular ... "Mutation in the alpha-cardiac actin gene associated with apical hypertrophic cardiomyopathy, left ventricular non-compaction, ... "Gene mutations in apical hypertrophic cardiomyopathy". Circulation. 112 (18): 2805-11. doi:10.1161/CIRCULATIONAHA.105.547448. ...
... which may be caused by diseases such as hypertensive cardiomyopathy, hypertrophic cardiomyopathy, restrictive cardiomyopathy, ... This includes patients with cardiomyopathy (hypertensive, hypertrophic, restrictive) and elderly individuals. Other factors ... in hypertrophic cardiomyopathy". Am. J. Cardiol. 70 (18): 1507-11. doi:10.1016/0002-9149(92)90313-N. PMID 1442632. Theodorakis ...
"Gregory M. Hirsch Hypertrophic Cardiomyopathy Center". Archived from the original on January 27, 2011. Retrieved November 9, ... "Postrenal biopsy AVM leading to severe hypertension and dilated cardiomyopathy". Pediatr. Nephrol. 24 (12): 2459-62. doi: ...
Kowey, P. R.; Eisenberg, R.; Engel, T. R. (1984-06-14). "Sustained arrhythmias in hypertrophic obstructive cardiomyopathy". The ... "Electrophysiological Testing for Sustained Arrhythmias in Patients with Hypertrophic Obstructive Cardiomyopathy". N Engl J Med ... "Sustained Arrhythmias in Hypertrophic Obstructive Cardiomyopathy". New England Journal of Medicine. 310 (24): 1566-1569. doi: ...
... hypertrophic cardiomyopathy), abnormally large (dilated cardiomyopathy), or abnormally stiff (restrictive cardiomyopathy). Some ... Liew, Alphonsus C.; Vassiliou, Vassilios S.; Cooper, Robert; Raphael, Claire E. (2017-12-12). "Hypertrophic Cardiomyopathy-Past ... Diseases of the heart muscle known as cardiomyopathies are of major importance. These include ischemic conditions caused by a ... Diseases affecting cardiac muscle, known as cardiomyopathies, are the leading cause of death in developed countries. The most ...
... and proteasomal activities were also depressed in patients with hypertrophic cardiomyopathy or dilated cardiomyopathy. Skinned ... MYBPC3 was thus the fourth gene for hypertrophic cardiomyopathy, following MYH7, encoding β-myosin heavy chain, TNNT2 and TPM1 ... Gruen M, Gautel M (Feb 1999). "Mutations in beta-myosin S2 that cause familial hypertrophic cardiomyopathy (FHC) abolish the ... Behrens-Gawlik V, Mearini G, Gedicke-Hornung C, Richard P, Carrier L (Feb 2014). "MYBPC3 in hypertrophic cardiomyopathy: from ...
Marian AJ, Roberts R (April 2001). "The molecular genetic basis for hypertrophic cardiomyopathy". Journal of Molecular and ...
... hypertrophic cardiomyopathy). He had been suffering from prostate cancer and related kidney problems but had seemed to be ...
Hypertrophic cardiomyopathy screening may also be considered in adolescents as part of a sports physical out of concern for ... Corrado, D.; Basso, C.; Schiavon, M.; Thiene, G. (6 August 1998). "Screening for hypertrophic cardiomyopathy in young athletes ...
... and progressive hypertrophic cardiomyopathy at 6 months. These data indicate that NM-IIB functions in ensuring the proper ...
September 1990). "Familial hypertrophic cardiomyopathy is a genetically heterogeneous disease". The Journal of Clinical ... For instance, it has been associated with retinitis pigmentosa, hypertrophic cardiomyopathy, osteogenesis imperfecta, and ...
Sigwart, U. (22 July 1995). "Non-surgical myocardial reduction for hypertrophic obstructive cardiomyopathy". Lancet. 346 (8969 ... a non-surgical method for the treatment of hypertrophic obstructive cardiomyopathy, as an alternative to open heart surgery. In ... a non-surgical method for the treatment of hypertrophic obstructive cardiomyopathy, as an alternative to open heart surgery. ... alcohol septal ablation for the treatment of hypertrophic obstructive cardiomyopathy. Sigwart received his medical degree in ...
Hypertrophic cardiomyopathy is diagnosed at birth in half. Death in the first year is usually due to cardiac failure. Marked ... A thickened heart muscle impairs its pumping ability (hypertrophic cardiomyopathy). The generalized muscle weakness can cause ... Sengers syndrome is a rare autosomal recessive condition characterised by congenital cataract, hypertrophic cardiomyopathy, ... which has stable cardiomyopathy and myopathy with normal intellect. Other reported features include nystagmus, strabismus, ...
... signs of hypertrophic obstructive cardiomyopathy (HOCM), and signs of arrhythmogenic right ventricular dysplasia (ARVD/C).[31][ ... some of the most important are hypertrophic cardiomyopathy, acute aortic dissection, pericardial tamponade, pulmonary embolism ... Aortic dissection (a tear in the aorta) and cardiomyopathy can also result in syncope.[19] ...
Hypertrophic cardiomyopathy. *Immunodeficiency virus. *Infectious peritonitis. *Leukemia virus. *Lower urinary tract disease ...
... hypertrophic cardiomyopathy and arrhythmogenic right ventricular dysplasia.[17][18] Fetal arrhythmia[edit]. Arrhythmias may ...
Catecholaminergic polymorphic ventricular tachycardia and hypertrophic cardiomyopathy and arrhythmogenic right ventricular ...
... cardiomyopathy). insulin receptor (Rabson-Mendenhall syndrome) · Insulin resistance ... Dermatomyositis · Hypertrophic osteoarthropathy. Mucocutaneous. reactive erythema: Erythema gyratum repens · Necrolytic ...
Cardiomyopathy *Dilated *Alcoholic. *Hypertrophic. *Tachycardia-induced. *Restrictive. *Loeffler endocarditis. *Cardiac ...
NOS 425 Cardiomyopathy 425.0 Endomyocardial fibrosis 425.1 Hypertrophic obstructive cardiomyopathy 425.2 Obscure cardiomyopathy ... primary cardiomyopathies 425.5 Alcoholic cardiomyopathy 425.7 Nutritional and metabolic cardiomyopathy 425.8 Cardiomyopathy in ... other diseases classified elsewhere 425.9 Secondary cardiomyopathy unspecified 426 Conduction disorders 426.0 Atrioventricular ...
"Ubiquitin proteasome dysfunction in human hypertrophic and dilated cardiomyopathies". Circulation. 121 (8): 997-1004. doi: ...
... a cardiac surgery treatment for hypertrophic obstructive cardiomyopathy Earth-boring dung beetle, or dor beetles, of the family ...
... hypertrophic cardiomyopathy Ronald McNair (1950 - 1986), graduate of North Carolina A&T State University, one of the astronauts ...
Cardiomyopathy due to anthracyclines Cardiomyopathy hearing loss type t RNA lysine gene mutation Hypertrophic cardiomyopathy: ... Cardiomyopathy dilated with conduction defect type 1, Cardiomyopathy dilated with conduction defect type 2, Cardiomyopathy, ... familial Cardiomyopathy hypogonadism metabolic anomalies Cardiomyopathy spherocytosis Cardiomyopathy, fatal fetal, due to ... right ventricular cardiomyopathy Cardiomyopathic lentiginosis Cardiomyopathy cataract hip spine disease Cardiomyopathy diabetes ...
were the first to identify the causative mutation Arg403Gln for hypertrophic cardiomyopathy (HCM) in the MYH7 gene. Studies ... Jaaskelainen P, Miettinen R, Karkkainen P, Toivonen L, Laakso M, Kuusisto J (2004). "Genetics of hypertrophic cardiomyopathy in ... "A molecular basis for familial hypertrophic cardiomyopathy: A beta cardiac myosin heavy chain gene missense mutation". Cell. 62 ... at COPaKB Archived 4 March 2016 at the Wayback Machine GeneReviews/NIH/NCBI/UW entry on Familial Hypertrophic Cardiomyopathy ...
The cause of his death was a genetic heart defect, hypertrophic cardiomyopathy, a leading cause of sudden cardiac death in ... said that the official cause of death remains an enlarged heart and a condition called hypertrophic myocardium, a rare genetic ...
"Ubiquitin proteasome dysfunction in human hypertrophic and dilated cardiomyopathies". Circulation. 121 (8): 997-1004. doi: ...
Hypertrophic cardiomyopathy is a disease in which the myocardium is thickened and can cause blood flow obstruction. If ...
... together with hypertrophic cardiomyopathy and coronary arteriosclerosis. The death was ruled a natural death. As to the ... "Hypertrophic Cardiomyopathy With Coronary Arteriosclerosis" listed as other conditions contributing but not related to the ...
Recently, it has been suggested that TRIM63/MuRF1 is associated with an autosomal-recessive form of hypertrophic cardiomyopathy ... "Mutations in TRIM63 cause an autosomal-recessive form of hypertrophic cardiomyopathy". Heart. 106 (17): 1342-1348. doi:10.1136/ ...
The differential diagnosis of this condition consists of the following: Hypertrophic cardiomyopathy Beckwith-Wiedemann syndrome ...
... assistant professor of medicine at the University of Minnesota and research investigator with the Hypertrophic Cardiomyopathy ...
... and hypertrophic cardiomyopathy. A patient with a 12316G>A mutation in MT-TL2 was found with the deficiency. Anderson S, ... In addition, multiple individuals with a T12297C substitution showed signs of cardiomyopathy accompanied with varying degrees. ... and is associated with dilated cardiomyopathy". European Journal of Human Genetics. 9 (4): 311-5. doi:10.1038/sj.ejhg.5200622. ...
His subspecialty interests include valvular heart disease, hypertrophic cardiomyopathy and pericardial disease. [1] Mike ...
"ELAC2 mutations cause a mitochondrial RNA processing defect associated with hypertrophic cardiomyopathy". Am. J. Hum. Genet. 93 ... a rare autosomal recessive disorder of mitochondrial functions characterized by severe hypertrophic cardiomyopathy. GRCh38: ...
... hypertrophic cardiomyopathy. Al Copeland, 64, American restaurateur, founder of Popeyes Chicken, salivary gland cancer. Hugo ...
... cardiac myosin-binding protein C gene associated with dilated phase of hypertrophic cardiomyopathy and dilated cardiomyopathy ... an important hypertrophic cardiomyopathy gene". Environmental and Molecular Mutagenesis. 52 (2): 161-4. doi:10.1002/em.20596. ... type 1 and hypertrophic cardiomyopathy, respectively. GRCh38: Ensembl release 89: ENSG00000086967 - Ensembl, May 2017 GRCm38: ... of novel interactions between domains of Myosin binding protein-C that are modulated by hypertrophic cardiomyopathy missense ...
"Ubiquitin proteasome dysfunction in human hypertrophic and dilated cardiomyopathies". Circulation. 121 (8): 997-1004. doi: ...
... the hypertrophic growth response and cardiac conductance. Another role for miRNA in cardiovascular diseases is to use their ... "Targeted deletion of Dicer in the heart leads to dilated cardiomyopathy and heart failure". Proceedings of the National Academy ... pointing to their involvement in cardiomyopathies. Furthermore, animal studies on specific miRNAs identified distinct roles for ...
... sequencing for clinical diagnostics-principles and application to targeted resequencing for hypertrophic cardiomyopathy: a ...
Hypertrophic Cardiomyopathy: Do New Markers Enhance Current Risk-Stratification Models?. Tom Kai Ming Wang, MBChB, MD; Milind ... Hypertrophic cardiomyopathy (HCM) is the most common form of hereditary heart disease, affecting 1 in 200-500 people. Of all ... Cite this: Hypertrophic Cardiomyopathy: Do New Markers Enhance Current Risk-Stratification Models? - Medscape - Oct 05, 2021. ... and the HCM Risk-SCD model in the ESC Guidelines on Diagnosis and Management of Hypertrophic Cardiomyopathy. The AHA/ACC ...
Hypertrophic cardiomyopathy (HCM) is a condition in which the heart muscle becomes thick. Often, only one part of the heart is ... In some cases, the condition may develop into dilated cardiomyopathy. People with hypertrophic cardiomyopathy are at higher ... Cardiomyopathy - hypertrophic (HCM); IHSS; Idiopathic hypertrophic subaortic stenosis; Asymmetric septal hypertrophy; ASH; HOCM ... Hypertrophic cardiomyopathy (HCM) is a condition in which the heart muscle becomes thick. Often, only one part of the heart is ...
Hypertrophic cardiomyopathy (HCM) (see the image below) is a genetic disorder that is typically inherited in an autosomal ... encoded search term (Hypertrophic Cardiomyopathy) and Hypertrophic Cardiomyopathy What to Read Next on Medscape ... Hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis: a ... Hypertrophic Cardiomyopathy Differential Diagnoses. Updated: Apr 29, 2022 * Author: Sandy N Shah, DO, MBA, FACC, FACP, FACOI; ...
What is Hypertrophic Cardiomyopathy?. Hypertrophic cardiomyopathy is a common genetic disorder that affects about 1 in 5001 ... Hypertrophic Cardiomyopathy (HCM), American Heart Association. Accessed August 15, 2022.. *Hypertrophic Cardiomyopathy, ... Genotype and Lifetime Burden of Disease in Hypertrophic Cardiomyopathy: Insights From the Sarcomeric Human Cardiomyopathy ... Cardiomyopathy UK. Accessed August 15, 2022.. *Maron BJ. Clinical Course and Management of Hypertrophic Cardiomyopathy. New ...
Hypertrophic cardiomyopathy at Curlie GeneReviews/NIH/NCBI/UW entry on Familial Hypertrophic Cardiomyopathy Overview National ... 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy Colan SD (October 2010). "Hypertrophic ... A diagnosis of hypertrophic cardiomyopathy is based upon a number of features of the disease process. While there is use of ... Familial hypertrophic cardiomyopathy is inherited as an autosomal dominant trait and is attributed to mutations in one of a ...
An Expanding List of Tier 1 Genomic Applications: Evidence-based Guidelines for Hypertrophic Cardiomyopathy and Public Health. ... The CDC Tier-Classified Guideline Database includes three Tier 1 guidelines on hypertrophic cardiomyopathy (HCM). A 2014 ... Hypertrophic Cardiomyopathy - Genomics and Precision Health Blog ...
Rare familial disorder with hypertrophic obstructive cardiomyopathy. Rare familial disorder with hypertrophic subaortic ... Rare familial disorder with hypertrophic cardiomyopathy. Go to external page http://www.orpha.net/ORDO/Orphanet_99739 Copy ...
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Hypertrophic cardiomyopathy is a common inherited cardiomyopathy, occurring in about 1 in 500 individuals.1 The first gene ... Hypertrophic cardiomyopathy: diagnosis, risk stratification and treatment. Daniel L. Jacoby, Eugene C. DePasquale, William J. ... Hypertrophic cardiomyopathy: diagnosis, risk stratification and treatment. Daniel L. Jacoby, Eugene C. DePasquale, William J. ... Sudden death in hypertrophic cardiomyopathy: identification of high risk patients. J Am Coll Cardiol 2000;36:2212-8. ...
... is one of just a few medical centers in California designated as a Center of Excellence by the Hypertrophic Cardiomyopathy ... About Hypertrophic Cardiomyopathy. Hypertrophic cardiomyopathy (HCM) is a genetic heart condition affecting an estimated 1 in ... How is Hypertrophic Cardiomyopathy Treated?. The goal of hypertrophic cardiomyopathy treatment is to reduce symptoms and ... How is Hypertrophic Cardiomyopathy Diagnosed?. Hypertrophic cardiomyopathy symptoms may be non-specific. Heart problems in ...
Hypertrophic cardiomyopathy (adult). Mayo Clinic; 2021.. *Hypertrophic cardiomyopathy (HCM). American College of Cardiology. ... www.heart.org/en/health-topics/cardiomyopathy/what-is-cardiomyopathy-in-adults/hypertrophic-cardiomyopathy. Accessed Dec. 9, ... Steve R. Ommen: Hypertrophic cardiomyopathy is the most common inherited cardiomyopathy or heart muscle disease. People are ... The goals of hypertrophic cardiomyopathy treatment are to relieve symptoms and prevent sudden cardiac death in people at high ...
This is one of several forms of heart muscle disease which commonly affects cats. Although the condition is rare in dogs, the German Shepherd Dog is cited as being the breed most likely to be afflicted with this disease. The cause is unknown.. Characterised by the gross thickening of the muscle in the walls of the heart which makes the walls stiff and less compliant, resulting in poor filling of the heart chambers with blood during the diastolic phase of contraction, and inadequate output of blood during systole. The clinical signs of this condition are those of heart failure. ...
Hypertrophic cardiomyopathy is an inherited heart muscle disease with variable clinical expression and natural history. ... Toronto Hosts the Inaugural Hypertrophic Cardiomyopathy Summit Hypertrophic cardiomyopathy affects 1 in 500 Canadians. Symptoms ... Normal Life Expectancy Seen in Patients With Hypertrophic Cardiomyopathy. A study has revealed that patients with hypertrophic ... In Children With Hypertrophic Cardiomyopathy Risk Factors Help Predict Outcomes. The risk of death or need for immediate ...
Hypertrophic cardiomyopathy - Homo sapiens (human) [ Pathway menu , Organism menu , Pathway entry , Download KGML , Show ... Hypertrophic cardiomyopathy (HCM) is a primary myocardial disorder with an autosomal dominant pattern of inheritance that is ...
16 Studies found for: Active, not recruiting Studies , Cardiomyopathy, Hypertrophic. Also searched for Hypertrophic ...
Hypertrophic Cardiomyopathy Association. The HCMA is the preeminent organization improving the lives of those with hypertrophic ... All content is the property of the Hypertrophic Cardiomyopathy Association for reprint permission contact the HCMA ... cardiomyopathy, HCM, preventing untimely deaths and advancing global understanding. Founded in 1996 we are committed to ...
... Hypertrophic cardiomyopathyClassification & external resources ICD-10 I42.1-I42.2 ICD-9 425.4 ... Differentiating hypertrophic cardiomyopathy and valvular aortic stenosis Aortic stenosis Hypertrophic cardiomyopathy ... Myocarditis - Cardiomyopathy (Dilated cardiomyopathy, Hypertrophic cardiomyopathy, Loeffler endocarditis, Restrictive ... and hypertrophic obstructive cardiomyopathy (HOCM). A non-obstructive variant of HCM is apical hypertrophic cardiomyopathy [8] ...
... help physicians weigh the benefits and risks of a particular diagnostic or therapeutic procedure on Hypertrophic Cardiomyopathy ...
Hypertrophic Cardiomyopathy news articles. The latest Hypertrophic Cardiomyopathy stories, articles, research, discoveries, ... Current Hypertrophic Cardiomyopathy News and Events. Current Hypertrophic Cardiomyopathy News and Events, Hypertrophic ... Hypertrophic cardiomyopathy guideline encourages shared decision-making. The American Heart Association and the American ... Study reinforces drugs potential to treat hypertrophic cardiomyopathy. WSU research sheds new light on a molecule that may be ...
... surgery or ablation were the only treatments for obstructive hypertrophic cardiomyopathy (HCM). Now, a newly FDA-approved ... suspected that John was developing hypertrophic cardiomyopathy (HCM). Its a congenital disease that causes the heart muscle to ...
Oftentimes, those with hypertrophic obstructive cardiomyopathy(HOCM, oHCM), apical hypertrophic cardiomyopathy, or asymmetric ... Hypertrophic Cardiomyopathy Association. The HCMA is the preeminent organization improving the lives of those with hypertrophic ... Hypertrophic cardiomyopathy (HCM) is a common genetic disorder that affects people regardless of gender, ethnicity, age or ... This website will expand your knowledge of hypertrophic cardiomyopathy as well as issues important to our community by ...
DDD pacing in hypertrophic cardiomyopathy: a multicentre clinical experience. Message subject: (Your Name) has forwarded a page ...
Hypertrophic Cardiomyopathy. To understand the pathology of HCM, a brief lesson in heart anatomy is in order. The heart has ... Hypertrophic cardiomyopathy can progress rapidly in some cats, while in others, the condition remains relatively static for ... both boys were suffering from a troublesome cardiac condition called hypertrophic cardiomyopathy (HCM). ...
The spectrum of the etiology and management of hypertrophic cardiomyopathy in infancy have been updated in the past several ... ABSTRACT Hypertrophic cardiomyopathy is a rare disorder in infancy. Signs of myocardial ischemia and cardiomegaly are the ... Hypertrophic cardiomyopathy in infants of diabetic mothers is usually benign and transient, and treatment is not needed unless ... Hypertrophic cardiomyopathy is a rare disorder in infancy. Signs of myocardial ischemia and cardiomegaly are the predominate ...
Hypertrophic Cardiomyopathy News * Psychological Health - Good or Bad - Plays an Important Role in Heart Disease Minding your ...
Use this URL to cite or link to this record in EThOS: ...
... This professionally illustrated 16″ x ... poster is designed to help clinicians diagnose hypertrophic cardiomyopathy (HCM).. The poster outlines the use of ...
... improved left ventricular outflow tract gradient and other metrics in patients with symptomatic obstructive hypertrophic ... cardiomyopathy, according to data from the PIONEER-HCM study presented at the Heart Failure Society of American Annual ...
In hypertrophic cardiomyopathy, the heart muscles become abnormally thick. This makes it harder for blood to be pumped out of ... Hypertrophic Cardiomyopathy. In hypertrophic cardiomyopathy, the heart muscles become abnormally thick. The thickening either ... Also, patients with hypertrophic cardiomyopathy are at a higher risk for sudden death, which can occur at any age. ... If youre experiencing signs or symptoms of hypertrophic cardiomyopathy, schedule an appointment or call 800-TEMPLE-MED (800- ...
  • Hypertrophic cardiomyopathy (HCM, or HOCM when obstructive) is a condition in which the heart becomes thickened without an obvious cause. (wikipedia.org)
  • HCM is also known as idiopathic hypertrophic subaortic stenosis ( IHSS ) and hypertrophic obstructive cardiomyopathy ( HOCM ). (bionity.com)
  • Oftentimes, those with hypertrophic obstructive cardiomyopathy(HOCM, oHCM), apical hypertrophic cardiomyopathy, or asymmetric septal hypertrophic cardiomyopathy think this is a completely different disease. (4hcm.org)
  • Hypertrophic obstructive cardiomyopathy (HOCM), historically referred to as idiopathic hypertrophic subaortic stenosis, is a relatively common disorder. (statpearls.com)
  • Hypertrophic obstructive cardiomyopathy (HOCM) is a relatively common disorder. (statpearls.com)
  • Treatment depends on whether there is obstruction of blood flow (hypertrophic obstructive cardiomyopathy or HOCM) and whether there are symptoms. (hypertrophiccardiomyopathy.com)
  • Furthermore, nomenclature that was popular in the 1960s and 1970s, such as ihss idiopathic hypertrophic subaortic stenosis or hocm hypertrophic obstructive cardiomyopathy, is potentially confusing by virtue of the inference that lvot is an invariable and obligatory component of the disease. (firebaseapp.com)
  • Blood Passage Contraction: Thickening leads to contraction of the blood passages and thus causes hypertrophic obstructive cardiomyopathy (HOCM). (reeleye.com)
  • This case study demonstrates that although the CT Coronary Angiogram was within normal limits, the perfusion scan provided additional, clinically significant information in regards to the haemodynamics within the myocardium of a patient with Hypertrophic Obstructive Cardio Myopathy (HOCM). (waset.org)
  • A Persian cat aged 21 years was diagnosed with Dynamic Hypertrophic Obstructive Cardiomyopathy (HOCM) and treated with PCSO-524® (Antinol®) for 45 days. (antinolstudies.com)
  • A patient with hypertrophic obstructive cardiomyopathy (HOCM) was admitted with acute heart failure. (kssg.ch)
  • Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): health status analysis of a randomised, double-blind, placebo-controlled, phase 3 trial. (medscape.com)
  • Projecting the long-term clinical value of mavacamten for the treatment of obstructive hypertrophic cardiomyopathy in the United States: an assessment of net health benefit. (medscape.com)
  • The aim of the present study was to determine the long-term effects of percutaneous transluminal septal myocardial ablation (PTSMA) on systolic and diastolic left ventricular (LV) functions in patients with obstructive hypertrophic cardiomyopathy (HC). (eur.nl)
  • Patients with severe obstructive hypertrophic cardiomyopathy and associated symptoms may require catheter-based on surgical interventions to relieve the obstruction. (hypertrophiccardiomyopathy.com)
  • The heart muscle in non-obstructive hypertrophic cardiomyopathy is thickened, but it doesn't block blood flow out of the heart. (statcardiologist.com)
  • If this happens, the condition is called obstructive hypertrophic cardiomyopathy. (firebaseapp.com)
  • Thus, HCM is a disease of the myofilaments, whose alterations in … I'm trying to figure out why nitrates are contraindicated in obstructive hypertrophic cardiomyopathy. (reeleye.com)
  • Catecholamine response to exercise in patients with non-obstructive hypertrophic cardiomyopathy. (harvard.edu)
  • Management strategy in 249 consecutive patients with obstructive hypertrophic cardiomyopathy referred to a dedicated program. (harvard.edu)
  • 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). (medscape.com)
  • The spectrum of the etiology and management of hypertrophic cardiomyopathy in infancy have been updated in the past several decades. (uwi.edu)
  • In 2011, the American College of Cardiology & American Heart Association along with several other leading organizations, developed guidelines for the management of hypertrophic cardiomyopathy. (hypertrophiccardiomyopathy.com)
  • Diagnosis and management of hypertrophic cardiomyopathy. (firebaseapp.com)
  • Global Hypertrophic Cardiomyopathy treatment Market 2021 research report delivers a systematic and comprehensive market research study, along with the facts and figures associated with any subject in the field of marketing. (medium.com)
  • 2021) Valsartan in early-stage hypertrophic cardiomyopathy: a randomized phase 2 trial. (babyanyone.com)
  • Familial hypertrophic cardiomyopathy is inherited as an autosomal dominant trait and is attributed to mutations in one of a number of genes that encode for the sarcomere proteins. (wikipedia.org)
  • A familial hypertrophic cardiomyopathy locus maps to chromosome 15q2. (ox.ac.uk)
  • The prevalence of HCM is about 0.2% to 0.5% of the general population.Myosin heavy chain mutations are associated with development of familial hypertrophic cardiomyopathy. (theworldnews.in)
  • Cardiomyopathy, Hypertrophic, Familial" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (musc.edu)
  • This graph shows the total number of publications written about "Cardiomyopathy, Hypertrophic, Familial" by people in this website by year, and whether "Cardiomyopathy, Hypertrophic, Familial" was a major or minor topic of these publications. (musc.edu)
  • Below are the most recent publications written about "Cardiomyopathy, Hypertrophic, Familial" by people in Profiles. (musc.edu)
  • Neonatal gene transfer of Serca2a delays onset of hypertrophic remodeling and improves function in familial hypertrophic cardiomyopathy. (musc.edu)
  • Genetic evaluation of familial cardiomyopathy. (musc.edu)
  • Frazier A, Judge DP, Schulman SP, Johnson N, Holmes KW, Murphy AM. Familial hypertrophic cardiomyopathy associated with cardiac beta-myosin heavy chain and troponin I mutations. (musc.edu)
  • Knollmann BC, Kirchhof P, Sirenko SG, Degen H, Greene AE, Schober T, Mackow JC, Fabritz L, Potter JD, Morad M. Familial hypertrophic cardiomyopathy-linked mutant troponin T causes stress-induced ventricular tachycardia and Ca2+-dependent action potential remodeling. (musc.edu)
  • Familial hypertrophic cardiomyopathy is a common inherited cardiovascular disorder in people. (biomedcentral.com)
  • Mutations in the ALMS1 gene are associated with the development of Alstrom syndrome, a multisystem familial disease that can include cardiomyopathy (dilated, restrictive). (biomedcentral.com)
  • Here we describe the association of familial hypertrophic cardiomyopathy in Sphynx cats with a novel ALMS1 mutation. (biomedcentral.com)
  • Our findings suggest that variants in genes involved with cardiac development and cell regulation, like the ALMS1 gene, may deserve further consideration for association with familial hypertrophic cardiomyopathy. (biomedcentral.com)
  • Familial hypertrophic cardiomyopathy is a very common inherited cardiovascular disorder in people, with a prevalence of 1:250 to 500 [ 1 ]. (biomedcentral.com)
  • This study describes the discovery of a mutation in the ALMS1 gene associated with the development of familial hypertrophic cardiomyopathy in the domestic cat, an excellent model of human hypertrophic cardiomyopathy due to many similarities, including the familial nature, clinical presentation and pathologic findings [ 9 , 10 ]. (biomedcentral.com)
  • Beta blockers are used in both cases, but treatment with diuretics, a mainstay of CHF treatment, will exacerbate symptoms in hypertrophic obstructive cardiomyopathy by decreasing ventricular preload volume and thereby increasing outflow resistance (less blood to push aside the thickened obstructing tissue). (wikipedia.org)
  • Hypertrophic cardiomyopathy is a congenital or acquired disorder characterized by marked ventricular hypertrophy with diastolic dysfunction (eg, due to valvular aortic stenosis, coarctation of the aorta, systemic hypertension). (merckmanuals.com)
  • The Gsk3b-/- embryos had a double outlet RV, ventricular septal defects, and hypertrophic myopathy, with near obliteration of the ventricular cavities. (jci.org)
  • Hypertrophic cardiomyopathy (HCM) is an inherited disease of the cardiac sarcomere that results in left ventricular hypertrophy, hyperdynamic function, microvascular dysfunction, impaired relaxation, and myocardial fibrosis. (acc.org)
  • Hypertrophic cardiomyopathy (HCM) is a genetic disease of the heart characterized by an increase in left ventricular wall thickness ( ≥ 15 mm ) in the absence of abnormal loading conditions (hypertension, congenital heart disease, or valvular disease) or other inherited or acquired diseases capable of producing such degree of hypertrophy. (medscape.com)
  • Basically, hypertrophic obstructive cardiomyopathy involves an abnormal relationship between the ventricular septum (enlarged and obstructive) and the mitral valve. (hypertrophiccardiomyopathy.com)
  • Kohli U, Saarel EV, Shah M. Extreme Left Ventricular Hypertrophy in Pediatric Hypertrophic Cardiomyopathy: Good News or Bad News? (uchicago.edu)
  • Steen H, Giusca S, Montenbruck M, Patel AR, Pieske B, Florian A, Erley J, Kelle S, Korosoglou G. Left and right ventricular strain using fast strain-encoded cardiovascular magnetic resonance for the diagnostic classification of patients with chronic non-ischemic heart failure due to dilated, hypertrophic cardiomyopathy or cardiac amyloidosis. (uchicago.edu)
  • To treat with diuretics (a mainstay of CHF treatment) will exacerbate symptoms in hypertrophic cardiomyopathy by decreasing ventricular volume and increasing outflow resistance. (theworldnews.in)
  • The most common causes of left ventricular hypertrophy are aortic stenosis, aortic regurgitation insufficiency, hypertension, cardiomyopathy and coarctation of the. (firebaseapp.com)
  • Dilated cardiomyopathy dcm is a myocardial disease characterised by ventricular dilatation and global myocardial dysfunction ejection fraction 35 mm. (firebaseapp.com)
  • Hypertrophic cardiomyopathy (HCM) is characterized by increased left ventricular wall thickness that can lead to devastating conditions such as heart failure and sudden cardiac death. (uea.ac.uk)
  • In addition, the journal provides information about the role rhythm plays in hypertrophic cardiomyopathy and ventricular arrhythmias. (bartleby.com)
  • Hypertrophic cardiomyopathy (HCM) is a genetic disorder of the heart muscle, characterized by a small left ventricular cavity and marked hypertrophy of the myocardium with myocyte disarray. (reeleye.com)
  • Dilated cardiomyopathy is a progressive disease of heart muscle that is characterized by ventricular chamber enlargement and contractile dysfunction. (medscape.com)
  • BACKGROUND AND AIMS: Both myectomy and alcohol septal ablation (ASA) can substantially reduce left ventricular (LV) outflow obstruction, relieve symptoms, and improve outcomes in hypertrophic cardiomyopathy (HCM). (units.it)
  • Apical hypertrophic cardiomyopathy (HCM) is an uncommon variant of HCM characterized by hypertrophy located in the left ventricular apex that occurs at a rate of about 30% in the Japanese population. (viamedica.pl)
  • Of 51 patients with hypertrophic cardiomyopathy who had episodes of ventricular tachycardia detected during ambulatory electrocardiographic monitoring only two had clinical sustained uniform ventricular tachycardia that required medical treatment because of worsening symptoms. (uea.ac.uk)
  • Significance: Hypertrophic cardiomyopathy (HCM) is a cardiac genetic disease characterized by left ventricular hypertrophy, diastolic dysfunction, and myocardial disarray. (vumc.nl)
  • Routine clinical echocardiographic data were obtained on 34 subjects at the Mayo Clinic (10 normal subjects, 10 patients with amyloid heart disease, 8 patients with hypertrophic cardiomyopathy and 6 patients with left ventricular hypertrophy due to hypertension). (edu.au)
  • The texture of the myocardium in hypertensive left ventricular hypertrophy not associated with amyloid or hypertrophic cardiomyopathy was in general not significantly different from that of normal myocardium. (edu.au)
  • Effects of losartan on left ventricular hypertrophy and fibrosis in patients with nonobstructive hypertrophic cardiomyopathy. (harvard.edu)
  • You have any symptoms of hypertrophic cardiomyopathy. (medlineplus.gov)
  • If you're experiencing signs or symptoms of hypertrophic cardiomyopathy, schedule an appointment or call 800-TEMPLE-MED (800-836-7536) today. (templehealth.org)
  • The symptoms of hypertrophic cardiomyopathy vary depending on how far the condition has advanced. (ameliaheartcenter.com)
  • Expanding the indications for septal myectomy in patients with hypertrophic cardiomyopathy: Results of operation in patients with latent obstruction. (medscape.com)
  • Dr. Naidu is an internationally-known expert in the treatment of HCM, and is involved in national clinical trials of new drugs, publishes the international textbook Hypertrophic Cardiomyopathy, and co-runs the live alcohol septal ablation proctoring course annually in Detroit. (westchestermedicalcenter.org)
  • In selected patients, Hypertrophic Obstructive Cardiomyopathy can be treated by alcohol septal ablation by causing necrosis of the obstructive muscle. (hypertrophiccardiomyopathy.com)
  • Predictors of outcome after alcohol septal ablation therapy in patients with hypertrophic obstructive cardiomyopathy. (musc.edu)
  • Pathological effects of alcohol septal ablation for hypertrophic obstructive cardiomyopathy. (harvard.edu)
  • Hypertrophic cardiomyopathy is a common genetic disorder that affects about 1 in 500 1 people and causes the heart muscle to become thicker and stiff. (cdc.gov)
  • Hypertrophic cardiomyopathy (HCM) is a genetic heart condition affecting an estimated 1 in 250 people. (ucsd.edu)
  • Hypertrophic cardiomyopathy (HCM) is a common genetic disorder that affects people regardless of gender, ethnicity, age or geographic location. (4hcm.org)
  • 1. Pelaqita Persians' extends the health/genetic guarantee through the age of six years for ONLY HCM (Hypertrophic Cardiomyopathy) [until _________] . (pelaqitapersians.com)
  • BACKGROUND: Genetic testing for families with hypertrophic cardiomyopathy (HCM) provides a significant opportunity to improve care. (ox.ac.uk)
  • Calling awareness to sudden cardiac arrest is one of the cornerstones of the HCMA, the national resource for patients who have Hypertrophic Cardiomyopathy (HCM), the most common genetic heart disease. (theworldnews.in)
  • Hypertrophic cardiomyopathy is a genetic disease. (sicknessfinder.com)
  • Genetic testing for hypertrophic cardiomyopathy (HCM) can provide an important clinical marker for disease outcome and family screening. (elsevier.com)
  • An individual with one parent with this disease has a higher chance of genetic mutation for hypertrophic cardiomyopathy. (statcardiologist.com)
  • Compiled consensus on the most important diagnostic modalities and genetic testing tools for the treatment of hypertrophic cardiomyopathy hcm. (firebaseapp.com)
  • Hypertrophic cardiomyopathy hcm is a genetic cardiac disease with a heterogeneous phenotypic expression. (firebaseapp.com)
  • Hypertrophic cardiomyopathy (HCM) is a common, serious, genetic heart disorder. (northwestern.edu)
  • myomectomy) Hypertrophic cardiomyopathy, HCM is a cardiac disorder with a genetic etiology, characterized by hypertrophy of the myocardium It is an important cause of morbidity and mortality in the affected population. (bartleby.com)
  • Hypertrophic cardiomyopathy (HCM) (see the image below) is a genetic disorder that is typically inherited in an autosomal dominant fashion with variable penetrance and variable expressivity. (reeleye.com)
  • Hypertrophic cardiomyopathy is generally caused by abnormal genetic mutations that cause the heart muscle to grow abnormally thick. (netmeds.com)
  • If one of the parent has hypertrophic cardiomyopathy, then you may have a 50% chance of having the genetic mutation for the disease. (netmeds.com)
  • Hypertrophic cardiomyopathy: Most children with hypertrophic cardiomyopathy have a genetic mutation that changes the structure of an important muscle cell protein. (kidshealth.org)
  • Dilated cardiomyopathy: Genetic mutations may cause dilated cardiomyopathy. (kidshealth.org)
  • Restrictive cardiomyopathy: Genetic mutations can cause this type, as can scarring of the heart muscle, tumors of the heart muscle, and other problems. (kidshealth.org)
  • Despite increased knowledge of causal mutations, the exact path from genetic defect leading to cardiomyopathy is complex and involves additional disease hits. (vumc.nl)
  • Fast Five Quiz: Hypertrophic Cardiomyopathy Workup - Medscape - Apr 13, 2022. (medscape.com)
  • Some people with hypertrophic cardiomyopathy may not have symptoms and will have normal lifespan. (medlineplus.gov)
  • People with hypertrophic cardiomyopathy are at higher risk for sudden death than people without the condition. (medlineplus.gov)
  • This study aims to find out how common it is for people with hypertrophic cardiomyopathy to also have sleep apnea. (nih.gov)
  • However, in very few people with hypertrophic cardiomyopathy, it can cause shortness of breath, chest pain, defects in the heart's electrical signalling, which may result in lethal abnormal heart rhythms (arrhythmias) or sudden death. (netmeds.com)
  • Hypertrophic cardiomyopathy (HCM) is the most common form of hereditary heart disease, affecting 1 in 200-500 people . (medscape.com)
  • HCM can be distinguished from other inherited causes of cardiomyopathy by its autosomal dominant pattern, whereas Fabry disease is X-linked and Friedreich's Ataxia is inherited in an autosomal recessive pattern. (wikipedia.org)
  • Given the frequency of hypertrophic cardiomyopathy and the development of additional diagnostic and prognostic strategies, practitioners require a reasonable evidence-based approach to diagnose, assess and treat this disease. (cmaj.ca)
  • Studies of the clinical prevalence of hypertrophic cardiomyopathy indicate that clinical recognition of disease may occur earlier in men than women. (cmaj.ca)
  • Hypertrophic cardiomyopathy , or HCM , is a disease of the myocardium (the muscle of the heart ) in which a portion of the myocardium is hypertrophied (thickened) without any obvious cause. (bionity.com)
  • A cardiomyopathy is any disease that primarily affects the muscle of the heart. (bionity.com)
  • So-called Takotsubo cardiomyopathy is a rare but life-threatening disease of the heart that can occur during extreme stress events. (brightsurf.com)
  • The growth in awareness programs carried out by various organizations regarding the lethality of the disease would boost the industry of Hypertrophic Cardiomyopathy Therapeutics in the forecast period. (alliedmarketresearch.com)
  • A literal translation of the term cardiomyopathy is 'disease of the heart muscle' and this is exactly what the medical condition is characterized by. (pethealthandcare.com)
  • The cat heart disease 'cardiomyopathy' is a very serious and fatal condition, if not treated fast and effectively. (pethealthandcare.com)
  • Hypertrophic cardiomyopathy (HCM) is an inherited disease that may appear without an obvious family history, making the diagnosis often difficult - patients may have lived with the disease for years and decades prior to a diagnosis being made. (westchestermedicalcenter.org)
  • Cardiac Disease in Pregnancy: Hypertrophic Obstructive Cardiomyopathy and Pulmonic Stenosis. (uchicago.edu)
  • Younger people are likely to have a more severe form of hypertrophic cardiomyopathy HCM is frequently asymptomatic until sudden cardiac death, and for this reason some suggest routinely screening certain populations for this disease. (theworldnews.in)
  • Cardiomyopathy is defined as a primary disease of the heart muscle (hereinafter referred to as the myocardium), which are guided primarily impaired cardiac function. (sicknessfinder.com)
  • Hypertrophic Cardiomyopathy is a disease in which the patient's heart muscle becomes thickened and enlarged. (statcardiologist.com)
  • Hypertrophic cardiomyopathy is a disease that causes the heart muscle to thicken. (nih.gov)
  • To date, the ecg remains an irreplaceable first step when evaluating patients with hypertrophic cardiomyopathy hcm and an abnormal ecg may be the only manifestation of disease at an early stage. (firebaseapp.com)
  • Feline hypertrophic cardiomyopathy (hcm) is a disease affecting cats in which the walls of the heart become increasingly enlarged. (thedossiers.net)
  • Hypertrophic cardiomyopathy (HCM) is a disease of muscle tissue in the heart that affects as many as 1 in 500 people in the United States, making it the most common inherited cardiomyopathy. (myheartdiseaseteam.com)
  • Patients with restrictive cardiomyopathy (RCM) often present at an advanced stage of disease with the pronounced cardiopulmonary symptoms of CHF. (bartleby.com)
  • Approximately, 1 of every 500 people is affected with hypertrophic cardiomyopathy, it is important for patients to understand the dynamics of the disease as it could potentially be life threatening. (bartleby.com)
  • Restrictive cardiomyopathy (RCM) is a rare disease of the myocardium and is the least common of the 3 clinically recognized and described cardiomyopathies. (reeleye.com)
  • Hypertrophic cardiomyopathy (HCM) is a genetically determined heart muscle disease most often (60 to 70 percent) caused by mutations in one of several sarcomere genes which encode components of the contractile apparatus. (reeleye.com)
  • Multiple causes of dilated cardiomyopathy exist, one or more of which may be responsible for an individual case of the disease (see Etiology ). (medscape.com)
  • For those patients with more serious symptoms or disease progression, hypertrophic cardiomyopathy may be treated with a pacemaker to optimize heart function. (congenital.org)
  • Hypertrophic cardiomyopathy (HCM) is an inherited disease of the heart muscle. (d4-pharma.com)
  • Conclusion: CT coronary angiography with adenosine stress myocardial CTP was utilised in this case to specifically exclude coronary artery disease in conjunction with accessing perfusion within the hypertrophic myocardium. (waset.org)
  • Adenosine stress myocardial CTP demonstrated the reduced myocardial blood flow within the hypertrophic myocardium, but the coronary arteries did not show any obstructive disease. (waset.org)
  • Valsartan, an angiotensin II receptor blocker drug, delayed disease progression and improved cardiac structure and function in patients with early-stage hypertrophic cardiomyopathy, according to results from a recent clinical trial published in Nature Medicine. (news-medical.net)
  • Background - The common intronic deletion, MYBPC3Δ25, detected in 4-8% of South Asian populations, is reported to be associated with cardiomyopathy, with ~7-fold increased risk of disease in variant carriers. (ox.ac.uk)
  • The most common etiologies of MR include MV prolapse (MVP), rheumatic heart disease, infective endocarditis, annular calcification, cardiomyopathy, and ischemic heart disease. (medscape.com)
  • Hypertrophic cardiomyopathy (HCM) is an inherited disease of the heart muscle characterized by otherwise unexplained thickening of the left ventricle. (arizona.edu)
  • One of the primary causes of LBBB is dilated cardiomyopathy , a heart disease where a weakened heart can't contract normally. (verywellhealth.com)
  • The effects of focal hypertrophy on geometry of the left ventricle and systolic function have not been studied in patients with hypertrophic cardiomyopathy (HCM), despite the fact that the former is the most prominent disease characteristic. (medscimonit.com)
  • It should come as too big of a surprise that "cardiomyopathy" refers to disease that affect the heart muscle. (indulgeyourpet.com)
  • Dilated cardiomyopathy (DCM) in dogs is a disease that is characterized by an enlarged heart. (indulgeyourpet.com)
  • Hypertrophic cardiomyopathy is a rare disease affecting dogs, so this is not a condition that many folks will have to deal with. (indulgeyourpet.com)
  • The death certificate and the autopsy listed "hypertrophic cardiomyopathy" as the cause of death with "arteriosclerotic coronary artery disease" as a contributing condition. (cdc.gov)
  • Medical treatments for hypertrophic cardiomyopathy include beta-blockers or calcium channel blockers to help relax the hypertrophied heart muscle and to slow the heart rate which allows for better heart function. (hypertrophiccardiomyopathy.com)
  • There are various treatments for hypertrophic cardiomyopathy. (congenital.org)
  • The treatment of hypertrophic cardiomyopathy usually depends on arrhythmias, outflow obstruction, activity level, and heart functioning changes. (statcardiologist.com)
  • EKGs can help detect hypertrophic cardiomyopathy and certain kinds of arrhythmias . (jdch.com)
  • The myocardium is abnormal with cellular and myofibrillar disarray, although this finding is not specific for hypertrophic cardiomyopathy. (merckmanuals.com)
  • Cardiomyopathy also can lead to a life-threatening arrhythmia (abnormal heartbeat), heart valve problems, and blood clots. (kidshealth.org)
  • An EKG can help detect certain abnormal heart rhythms and hypertrophic cardiomyopathy - a condition linked to sudden death in athletes. (jdch.com)
  • The method has been applied to the analysis of the local deformation patterns in a set of patients affected by hypertrophic cardiomyopathy in order to find the agreement between hyperenhanced zones in late enhancement images and areas in the myocardium with abnormal tensor values (both the radial and the circumferential components as well as the shearing component have been accounted for). (uva.es)
  • Hypertrophic cardiomyopathy is characterized by an abnormal thickening of the heart muscle. (babyanyone.com)
  • Blood thinners such as warfarin (Jantoven), dabigatran (Pradaxa), rivaroxaban (Xarelto) or apixaban (Eliquis) to prevent blood clots if you have atrial fibrillation or the apical type of hypertrophic cardiomyopathy, which can increase the risk of sudden cardiac death. (mayoclinic.org)
  • Current treatment guidelines recommend anticoagulation for hypertrophic cardiomyopathy (HCM) with atrial fibrillation (AF) regardless of the CHA 2 DS 2 -VASc score. (aging-us.com)
  • Clinical profile and consequences of atrial fibrillation in hypertrophic cardiomyopathy. (drsvenkatesan.com)
  • Learn more about our doctors and care team who diagnose and treat hypertrophic cardiomyopathy. (templehealth.org)
  • Some medications, lifestyle changes, and medical procedures help to treat hypertrophic cardiomyopathy. (statcardiologist.com)
  • The medications that help to treat Hypertrophic Cardiomyopathy may include calcium channel blockers, beta-blockers, diuretics, and antiarrhythmics. (statcardiologist.com)
  • For the first time, medication that impacts heart muscle thickness and function, rather than addressing symptoms, is found to treat hypertrophic cardiomyopathy. (d4-pharma.com)
  • A non-obstructive variant of HCM is apical hypertrophic cardiomyopathy [8] , which is also known as nonobstructive hypertrophic cardiomyopathy and Japanese variant hypertrophic cardiomyopathy (since the first cases described were all in individuals of Japanese descent). (bionity.com)
  • IMSEAR at SEARO: Apical hypertrophic cardiomyopathy. (who.int)
  • Your provider will likely order tests to diagnose hypertrophic cardiomyopathy (HCM) or rule out other conditions that can cause similar symptoms. (mayoclinic.org)
  • An echocardiogram is commonly used to diagnose hypertrophic cardiomyopathy. (mayoclinic.org)
  • This professionally illustrated 16″ x 20″ poster is designed to help clinicians diagnose hypertrophic cardiomyopathy (HCM). (asecho.org)
  • There are many ways for the doctor to diagnose hypertrophic cardiomyopathy. (ameliaheartcenter.com)
  • If there's no significant blocking of blood flow, the condition is called nonobstructive hypertrophic cardiomyopathy. (netmeds.com)
  • The mitral valve in hypertrophic cardiomyopathy: old versus new concepts. (musc.edu)
  • Clinical implications of midventricular obstruction in patients with hypertrophic cardiomyopathy. (medscape.com)
  • DDD pacing in hypertrophic cardiomyopathy: a multicentre clinical experience. (bmj.com)
  • This study aimed to determine the prevalence of physical inactivity and perceived barriers to physical activity among individuals with hypertrophic cardiomyopathy (HCM), and to determine potential demographic, clinical and health-related factors influencing likelihood of meeting physical activity guidelines. (theactigraph.com)
  • Evaluating the Clinical Validity of Hypertrophic Cardiomyopathy Genes. (ox.ac.uk)
  • Use of genetics in the clinical evaluation of cardiomyopathy. (musc.edu)
  • Among the 31 cases with complete clinical data, initiation of bystander-administered cardiopulmonary resuscitation and an underlying diagnosis other than hypertrophic cardiomyopathy were the strongest predictors of survival. (nih.gov)
  • Progression of myocardial fibrosis in hypertrophic cardiomyopathy: Mechanisms and clinical implications. (ox.ac.uk)
  • In the current clinical trial, more than 170 participants between the ages of 8 and 45 years old who were diagnosed with early-stage hypertrophic cardiomyopathy were randomized to receive either valsartan or a placebo for two years. (babyanyone.com)
  • Further prospective studies of this technique are needed to establish its utility in identifying the etiology of clinical cardiomyopathies. (edu.au)
  • Clinical Correlates and Prognostic Value of Elevated Right Atrial Pressure in Patients With Hypertrophic Cardiomyopathy. (harvard.edu)
  • 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. (medscape.com)
  • 2011 ACCF/AHA Guideline for the Diagnosis and Treatment of Hypertrophic Cardiomyopathy. (hypertrophiccardiomyopathy.com)
  • The present study tested the hypothesis that quantitative echocardiographic texture analysis, a method of evaluating the spatial pattern of echoes in echocardiographic images, would differentiate amyloid and hypertrophic cardiomyopathy from normal myocardium. (edu.au)
  • Cardiomyopathy encompasses a group of diseases in which the myocardium (heart muscle) is unable to contract, leading to cardiac dysfunction. (khanacademy.org)
  • Outline the typical presentation for a patient with hypertrophic obstructive cardiomyopathy. (statpearls.com)
  • Having a healthy heart can reduce the chances of a patient with hypertrophic cardiomyopathy developing any problems. (heartsense.in)
  • An irregular heartbeat can be life threatening in a patient with hypertrophic cardiomyopathy. (heartsense.in)
  • To evaluate a patient with hypertrophic cardiomyopathy for the risk of a life-threatening arrhythmia using the ECG score of Ostman-Smith et al. (medicalalgorithms.com)
  • Ostman-Smith et al developed a score for identifying a patient with hypertrophic cardiomyopathy who is at increased risk for sudden death. (medicalalgorithms.com)
  • Current perspectives on sudden cardiac death in hypertrophic cardiomyopathy. (medscape.com)
  • abstract = "FHL1 gene mutations are associated with reducing body myopathy, X-linked myopathy with postural muscle atrophy, scapuloperoneal myopathy, Emery-Dreifuss muscular dystrophy, and isolated hypertrophic cardiomyopathy. (monash.edu)
  • abstract = "Purpose: To employ four-dimensional (4D) flow MRI to investigate associations between hemodynamic parameters with systolic anterior motion (SAM), mitral regurgitation (MR), stroke volume, and cardiac mass in patients with hypertrophic cardiomyopathy (HCM). (northwestern.edu)
  • abstract = "Introduction: Risk assessment for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) remains complex. (arizona.edu)
  • Clinico-histopathological correlation of symptomatic left main coronary artery electrocardiographic ischemia pattern in a young child with hypertrophic cardiomyopathy. (uchicago.edu)
  • Sadly, after a battery of tests that included cardiac ultrasound, the cause of Michael and Riley's murmurs were revealed: both boys were suffering from a troublesome cardiac condition called hypertrophic cardiomyopathy (HCM). (manhattancats.com)
  • Dilated cardiomyopathy is 1 of the 3 traditional classes of cardiomyopathy, along with hypertrophic and restrictive cardiomyopathy. (medscape.com)
  • Hypertrophic cardiomyopathy is a common inherited cardiomyopathy, occurring in about 1 in 500 individuals. (cmaj.ca)
  • Hypertrophic cardiomyopathy is one of the most common inherited cardiomyopathies, it may be passed from a parent to child in some families. (ucsd.edu)
  • Hypertrophic cardiomyopathy hcm is one of the most common inherited cardiomyopathy. (firebaseapp.com)
  • Mar 16, 2019 hypertrophic cardiomyopathy hcm is one of the most common inherited cardiac disorders affecting 1 in 500 people and is the number one cause of sudden cardiac death in young athletes. (firebaseapp.com)
  • BACKGROUND: There is limited research on hypertrophic cardiomyopathy (HCM), which is the most common inherited cardiac disorder, in diverse populations, including Black individuals. (nyu.edu)
  • The impact of ischemia assessed by magnetic resonance on functional, arrhythmic, and imaging features of hypertrophic cardiomyopathy. (medscape.com)
  • Subsequent histologic analysis showed increased cardiomyocyte cross-sectional area and regions of myocyte disarray and fibrosis, classic features of hypertrophic cardiomyopathy (HCM). (wustl.edu)
  • Differential diagnosis of hypertrophic cardiomyopathy from congenital heart defects is important for subsequent management. (uwi.edu)
  • Firstly , it is important to remember that despite having a diagnosis of hypertrophic cardiomyopathy, most patients can leave a completely normal life. (heartsense.in)
  • The diagnosis of hypertrophic cardiomyopathy by cardiovascular magnetic resonance. (musc.edu)
  • The diagnosis of Hypertrophic Cardiomyopathy is generally based on the patient's family history, medical history, physical exam, and diagnostic test results. (statcardiologist.com)
  • The diagnosis of hypertrophic cardiomyopathy involves several types of tests. (firebaseapp.com)
  • We also included seminal articles that have made important contributions to our knowledge of hypertrophic cardiomyopathy, even if such articles were not identified in our search. (cmaj.ca)
  • This website will expand your knowledge of hypertrophic cardiomyopathy as well as issues important to our community by providing clinically reviewed information, support options and member services. (4hcm.org)
  • The CDC Tier-Classified Guideline Database includes three Tier 1 guidelines on hypertrophic cardiomyopathy (HCM). (cdc.gov)
  • Prevalence of hypertrophic cardiomyopathy in an outpatient population referred for echocardiographic study. (medscape.com)
  • Prevalence of hypertrophic cardiomyopathy in a general population of young adults. (medscape.com)
  • New perspectives on the prevalence of hypertrophic cardiomyopathy. (firebaseapp.com)
  • BACKGROUND Predictors of risk of lethal arrhythmic events (LAE) is poorly understood and may differ from adults in children with hypertrophic cardiomyopathy (HCM). (uni-koeln.de)
  • The mean age of individuals with hypertrophic cardiomyopathy with asymmetry was less than that of those with severe atherosclerosis (24 vs 32 years, p = 0.03). (nih.gov)
  • Thus exercise precipitates sudden cardiac death in younger individuals with hypertrophic cardiomyopathy. (nih.gov)
  • Focus on echocardiography in hypertrophic cardiomyopathy - fourth in series. (wikem.org)
  • Echocardiography in patients with hypertrophic cardiomyopathy. (firebaseapp.com)
  • Purpose: To compare patterns of cardiovascular magnetic resonance tissue tracking (CMR-TT) for CA and hypertrophic cardiomyopathy (HCM) and to assess the feasibility of CMR-TT to distinguish between these diseases without administration of gadolinium-contrast. (elsevier.com)
  • Do We Need Specific Exercise Recommendation Guidelines For Pediatric Hypertrophic Cardiomyopathy? (ctimeetingtech.com)
  • AimsEchocardiographic strain imaging with two-dimensional speckle tracking is an emerging tool for defining cardiac function and identifying preclinical hypertrophic cardiomyopathy (HCM). (elsevier.com)
  • Hypertrophic cardiomyopathy (HCM) is a primary myocardial disorder with an autosomal dominant pattern of inheritance that is characterized by hypertrophy of the left ventricles with histological features of myocyte hypertrophy, myfibrillar disarray, and interstitial fibrosis. (genome.jp)
  • Hypertrophic cardiomyopathy is a rare disorder in infancy. (uwi.edu)
  • Hypertrophic cardiomyopathy (HCM) is a disorder where the heart muscle becomes abnormally thick or hypertrophied. (alliedmarketresearch.com)
  • Jun 19, 20 cardiomyopathy cardiomyopathy is a disorder of the muscle of the heart. (firebaseapp.com)
  • Historically, it has been referred to as idiopathic hypertrophic subaortic stenosis. (statpearls.com)
  • We performed a PubMed search using the term "hypertrophic cardiomyopathy. (cmaj.ca)
  • The HCMA is the preeminent organization improving the lives of those with hypertrophic cardiomyopathy, HCM, preventing untimely deaths and advancing global understanding. (4hcm.org)
  • Hypertrophic cardiomyopathy is inherited as an autosomal dominant trait and is attributed to mutations in one of a number of genes that encode for one of the sarcomere proteins including beta-cardiac myosin heavy chain (the first gene identified), cardiac actin, cardiac troponin T, alpha-tropomyosin, cardiac troponin I, cardiac myosin-binding protein C, and the myosin light chains. (bionity.com)
  • DOI https://doi.org/10.15212/CVIA.2019.0588, Yukuan Chen, Xiaohui Wu, Danchun Hu and Wei Wang, from the Shantou University Medical College, Shantou, China and Second Affiliated Hospital of Shantou University Medical College, Shantou, China consider the importance of mitochondrial-related genes in dilated cardiomyopathy. (brightsurf.com)
  • A significant number of causal mutations have been identified, particularly in sarcomeric or sarcomeric associated genes, but it has been estimated that about 40% of cases are due to mutations in genes in which the association with cardiomyopathy has yet to be identified, genes referred to as the "missing causal genes" [ 2 , 3 ]. (biomedcentral.com)
  • Contact the doctor immediately if someone notices shortness of breath, irregular Heartbeat, and chest pain, or if he has any family history of Hypertrophic Cardiomyopathy. (statcardiologist.com)