Aortic Arch Syndromes: Conditions resulting from abnormalities in the arteries branching from the ASCENDING AORTA, the curved portion of the aorta. These syndromes are results of occlusion or abnormal blood flow to the head-neck or arm region leading to neurological defects and weakness in an arm. These syndromes are associated with vascular malformations; ATHEROSCLEROSIS; TRAUMA; and blood clots.Sleep Apnea, Obstructive: A disorder characterized by recurrent apneas during sleep despite persistent respiratory efforts. It is due to upper airway obstruction. The respiratory pauses may induce HYPERCAPNIA or HYPOXIA. Cardiac arrhythmias and elevation of systemic and pulmonary arterial pressures may occur. Frequent partial arousals occur throughout sleep, resulting in relative SLEEP DEPRIVATION and daytime tiredness. Associated conditions include OBESITY; ACROMEGALY; MYXEDEMA; micrognathia; MYOTONIC DYSTROPHY; adenotonsilar dystrophy; and NEUROMUSCULAR DISEASES. (From Adams et al., Principles of Neurology, 6th ed, p395)Sleep Apnea Syndromes: Disorders characterized by multiple cessations of respirations during sleep that induce partial arousals and interfere with the maintenance of sleep. Sleep apnea syndromes are divided into central (see SLEEP APNEA, CENTRAL), obstructive (see SLEEP APNEA, OBSTRUCTIVE), and mixed central-obstructive types.Polysomnography: Simultaneous and continuous monitoring of several parameters during sleep to study normal and abnormal sleep. The study includes monitoring of brain waves, to assess sleep stages, and other physiological variables such as breathing, eye movements, and blood oxygen levels which exhibit a disrupted pattern with sleep disturbances.Cough: A sudden, audible expulsion of air from the lungs through a partially closed glottis, preceded by inhalation. It is a protective response that serves to clear the trachea, bronchi, and/or lungs of irritants and secretions, or to prevent aspiration of foreign materials into the lungs.Continuous Positive Airway Pressure: A technique of respiratory therapy, in either spontaneously breathing or mechanically ventilated patients, in which airway pressure is maintained above atmospheric pressure throughout the respiratory cycle by pressurization of the ventilatory circuit. (On-Line Medical Dictionary [Internet]. Newcastle upon Tyne(UK): The University Dept. of Medical Oncology: The CancerWEB Project; c1997-2003 [cited 2003 Apr 17]. Available from: http://cancerweb.ncl.ac.uk/omd/)Snoring: Rough, noisy breathing during sleep, due to vibration of the uvula and soft palate.Uvula: A fleshy extension at the back of the soft palate that hangs above the opening of the throat.Vasculitis: Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body.Gastroparesis: Chronic delayed gastric emptying. Gastroparesis may be caused by motor dysfunction or paralysis of STOMACH muscles or may be associated with other systemic diseases such as DIABETES MELLITUS.Systemic Vasculitis: A heterogeneous group of diseases characterized by inflammation and necrosis of the blood vessel walls.Polyarteritis Nodosa: A form of necrotizing non-granulomatous inflammation occurring primarily in medium-sized ARTERIES, often with microaneurysms. It is characterized by muscle, joint, and abdominal pain resulting from arterial infarction and scarring in affected organs. Polyarteritis nodosa with lung involvement is called CHURG-STRAUSS SYNDROME.Churg-Strauss Syndrome: Widespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from POLYARTERITIS NODOSA.Inflammation: A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: Group of systemic vasculitis with a strong association with ANCA. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.Giant Cell Arteritis: A systemic autoimmune disorder that typically affects medium and large ARTERIES, usually leading to occlusive granulomatous vasculitis with transmural infiltrate containing multinucleated GIANT CELLS. The TEMPORAL ARTERY is commonly involved. This disorder appears primarily in people over the age of 50. Symptoms include FEVER; FATIGUE; HEADACHE; visual impairment; pain in the jaw and tongue; and aggravation of pain by cold temperatures. (From Adams et al., Principles of Neurology, 6th ed)Takayasu Arteritis: A chronic inflammatory process that affects the AORTA and its primary branches, such as the brachiocephalic artery (BRACHIOCEPHALIC TRUNK) and CAROTID ARTERIES. It results in progressive arterial stenosis, occlusion, and aneurysm formation. The pulse in the arm is hard to detect. Patients with aortitis syndrome often exhibit retinopathy.Microscopic Polyangiitis: A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.Physical Examination: Systematic and thorough inspection of the patient for physical signs of disease or abnormality.FloridaAbnormalities, Drug-Induced: Congenital abnormalities caused by medicinal substances or drugs of abuse given to or taken by the mother, or to which she is inadvertently exposed during the manufacture of such substances. The concept excludes abnormalities resulting from exposure to non-medicinal chemicals in the environment.Pregnancy: The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.Therapeutic Equipoise: Expectation of real uncertainty on the part of the investigator regarding the comparative therapeutic merits of each arm in a trial.Questionnaires: Predetermined sets of questions used to collect data - clinical data, social status, occupational group, etc. The term is often applied to a self-completed survey instrument.Pregnancy Outcome: Results of conception and ensuing pregnancy, including LIVE BIRTH; STILLBIRTH; SPONTANEOUS ABORTION; INDUCED ABORTION. The outcome may follow natural or artificial insemination or any of the various ASSISTED REPRODUCTIVE TECHNIQUES, such as EMBRYO TRANSFER or FERTILIZATION IN VITRO.Purpura, Thrombocytopenic, Idiopathic: Thrombocytopenia occurring in the absence of toxic exposure or a disease associated with decreased platelets. It is mediated by immune mechanisms, in most cases IMMUNOGLOBULIN G autoantibodies which attach to platelets and subsequently undergo destruction by macrophages. The disease is seen in acute (affecting children) and chronic (adult) forms.Purpura, Thrombocytopenic: Any form of purpura in which the PLATELET COUNT is decreased. Many forms are thought to be caused by immunological mechanisms.Wegener Granulomatosis: A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and kidneys. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against neutrophil proteinase-3 (WEGENER AUTOANTIGEN).Cholangitis, Sclerosing: Chronic inflammatory disease of the BILIARY TRACT. It is characterized by fibrosis and hardening of the intrahepatic and extrahepatic biliary ductal systems leading to bile duct strictures, CHOLESTASIS, and eventual BILIARY CIRRHOSIS.Lupus Erythematosus, Systemic: A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.Sandfly fever Naples virus: A species in the genus PHLEBOVIRUS causing PHLEBOTOMUS FEVER, an influenza-like illness. Related serotypes include Toscana virus and Tehran virus.Adenylate Cyclase: An enzyme of the lyase class that catalyzes the formation of CYCLIC AMP and pyrophosphate from ATP. EC 4.6.1.1.Phlebotomus Fever: Influenza-like febrile viral disease caused by several members of the BUNYAVIRIDAE family and transmitted mostly by the bloodsucking sandfly Phlebotomus papatasii.GTP-Binding Proteins: Regulatory proteins that act as molecular switches. They control a wide range of biological processes including: receptor signaling, intracellular signal transduction pathways, and protein synthesis. Their activity is regulated by factors that control their ability to bind to and hydrolyze GTP to GDP. EC 3.6.1.-.Antidepressive Agents: Mood-stimulating drugs used primarily in the treatment of affective disorders and related conditions. Several MONOAMINE OXIDASE INHIBITORS are useful as antidepressants apparently as a long-term consequence of their modulation of catecholamine levels. The tricyclic compounds useful as antidepressive agents (ANTIDEPRESSIVE AGENTS, TRICYCLIC) also appear to act through brain catecholamine systems. A third group (ANTIDEPRESSIVE AGENTS, SECOND-GENERATION) is a diverse group of drugs including some that act specifically on serotonergic systems.Serotonin Uptake Inhibitors: Compounds that specifically inhibit the reuptake of serotonin in the brain.Congenital Abnormalities: Malformations of organs or body parts during development in utero.Down Syndrome: A chromosome disorder associated either with an extra chromosome 21 or an effective trisomy for chromosome 21. Clinical manifestations include hypotonia, short stature, brachycephaly, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, Simian crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213)Cleft Palate: Congenital fissure of the soft and/or hard palate, due to faulty fusion.Dental Prophylaxis: Treatment for the prevention of periodontal diseases or other dental diseases by the cleaning of the teeth in the dental office using the procedures of DENTAL SCALING and DENTAL POLISHING. The treatment may include plaque detection, removal of supra- and subgingival plaque and calculus, application of caries-preventing agents, checking of restorations and prostheses and correcting overhanging margins and proximal contours of restorations, and checking for signs of food impaction.Gene Duplication: Processes occurring in various organisms by which new genes are copied. Gene duplication may result in a MULTIGENE FAMILY; supergenes or PSEUDOGENES.PhiladelphiaChromosomes, Human, 21-22 and Y: The short, acrocentric human chromosomes, called group G in the human chromosome classification. This group consists of chromosome pairs 21 and 22 and the Y chromosome.Methyl-CpG-Binding Protein 2: A DNA-binding protein that interacts with methylated CPG ISLANDS. It plays a role in repressing GENETIC TRANSCRIPTION and is frequently mutated in RETT SYNDROME.Nephrotic Syndrome: A condition characterized by severe PROTEINURIA, greater than 3.5 g/day in an average adult. The substantial loss of protein in the urine results in complications such as HYPOPROTEINEMIA; generalized EDEMA; HYPERTENSION; and HYPERLIPIDEMIAS. Diseases associated with nephrotic syndrome generally cause chronic kidney dysfunction.Prospective Studies: 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.Proteinuria: The presence of proteins in the urine, an indicator of KIDNEY DISEASES.Adrenocorticotropic Hormone: An anterior pituitary hormone that stimulates the ADRENAL CORTEX and its production of CORTICOSTEROIDS. ACTH is a 39-amino acid polypeptide of which the N-terminal 24-amino acid segment is identical in all species and contains the adrenocorticotrophic activity. Upon further tissue-specific processing, ACTH can yield ALPHA-MSH and corticotrophin-like intermediate lobe peptide (CLIP).Corticotropin-Releasing Hormone: A peptide of about 41 amino acids that stimulates the release of ADRENOCORTICOTROPIC HORMONE. CRH is synthesized by neurons in the PARAVENTRICULAR NUCLEUS of the HYPOTHALAMUS. After being released into the pituitary portal circulation, CRH stimulates the release of ACTH from the PITUITARY GLAND. CRH can also be synthesized in other tissues, such as PLACENTA; ADRENAL MEDULLA; and TESTIS.Nephrosis, Lipoid: A kidney disease with no or minimal histological glomerular changes on light microscopy and with no immune deposits. It is characterized by lipid accumulation in the epithelial cells of KIDNEY TUBULES and in the URINE. Patients usually show NEPHROTIC SYNDROME indicating the presence of PROTEINURIA with accompanying EDEMA.Syndrome: A characteristic symptom complex.

Surgical treatment of nonaneurysmal aortic arch lesions in patients with systemic embolization. (1/58)

PURPOSE: Atherosclerotic lesions of the aortic arch are potential sources of arterial embolism. Here we investigate whether surgery, with the necessary circulatory supports, can be proposed as a good option for treatment of this problem. Study of these lesions on a national scale in France has made possible the assessment for future indications of techniques and results of the surgical management of aortic arch lesions, which retrospectively proved to be embolic. METHODS: Thirty-eight patients, (19 men and 19 women) underwent surgery between 1976 and 1996 in 17 French cardiovascular surgical centers. The average age at the time of surgery was 49 +/- 12 years (range, 31 to 82 years). Atherosclerotic lesions were detected with transesophagial echocardiography (n = 19), angiography of the aortic arch (n = 16), computed tomography (n = 9), and magnetic resonance imaging (n = 10). Surgery consisted of thrombectomy and endarterectomy (n = 22), aortic resection and graft replacement (n = 10), and patch aortoplasty (n = 5; one thrombus disappeared spontaneously before surgery was performed). RESULTS: The average postoperative period was 30 months (range, 3 to 82 months). Contact was lost with four patients after a follow-up period of 12 months. On pathologic specimens obtained at surgery, an atherosclerotic plaque was found in 73% of the cases (n = 28). In 15% of the cases, the aorta appeared normal (n = 6) and four other types of lesion were identified: angiosarcoma (n = 1), ectasia at the insertion of the remains of the ductus arteriosus (n = 1), rupture of tunica intima (n = 1), and a fibroblastic plaque (n = 1). A thrombus was identified in 26 cases, attached to the arterial wall in 18 cases. When transesophagial echocardiographic results showed mobile lesions (n = 22), histopathologic examination of specimens allowed the detection of a thrombus in 18 cases and an atherosclerotic plaque with a mobile projection in four cases. The postoperative mortality rate was 2.6%. The morbidity rate (28.9%; n = 11) was related to neurologic complications (n = 6), vascular complications (n = 4), and infection (n = 1). Four cases (12%) were reoperated. CONCLUSION: Nonaneurysmal aortic arch lesions are a frequent and still underestimated source of stroke and peripheral embolization. Surgery with circulatory support can be recommended in good operative candidates with recurrent critical events despite medical management and with high embolic potential (young patients with no calcified plaques).  (+info)

PARADOXES OF TAKAYASU'S DISEASE. (2/58)

Takayasu's disease (or arteritis) has been defined as an "idiopathic aortitis usually affecting young women." It can come to light from very spectacular and often quite puzzling clinical manifestations. Six cases of Takayasu's disease were investigated at the UCLA Hospital in the years 1961-1962, and signs and symptoms of central nervous system involvement were found in five of the patients. This relatively high incidence of neurological deficit prompted a review of case reports in the literature and this in turn led to a series of "unexpected" findings in the historical evolution of the illness as well as in its anatomopathological aspects. The study indicated that Takayasu's disease is frequently associated with neurological manifestations, at times very severe. In addition, the disease appears to be far more extensive than its classical description suggests. New criteria for the diagnosis of Takayasu's disease must include, among other things, special emphasis on the disseminated nature of the disease.  (+info)

Common variable immunodeficiency syndrome with right aortic arch: a case report. (3/58)

BACKGROUND: Common variable immunodeficiency syndrome predominantly affects adults. It is characterized by low production of all the major classes of immunoglobulins. We report a case of common variable immunodeficiency syndrome with right aortic arch. An association of right-sided arch and common variable immunodeficiency syndrome has not been previously reported. CASE PRESENTATION: A 41-year-old female patient presented with a history of recurrent pneumonia, sinusitis, otitis media, diarrhoea, cystitis since childhood. Biochemical and immunocytochemical analysis revealed common variable immunodeficiency syndrome and radiological evaluation confirmed right aortic arch and aberrant left subclavian artery. CONCLUSION: Common variable immunodeficiency syndrome syndrome is a clinical entity that should be kept in mind in patients with recurrent infections of different sites.  (+info)

Subclavian steal in Takayasu's arteritis. A hemodynamic study by means of ultrasonic Doppler flowmetry. (4/58)

Blood flow in the vertebral artery and the upper extremity was studied in five cases of Takayasu's arteritis with subclavian steal by use of ultrasonic Doppler flowmetry and finger plethysmography. The diagnosis of subclavian steal was made by observation of flow reversal in the vertebral artery on the subclavian steal side during grip exercise and, in addition, the vertebral flow change with brachial artery occlusion. The blood flow increase of both internal cartotid and non-affected (non-subclavian steal side) vertebral arteries during a common carotid compression was almost normal in patients with Takayasu's arteritis in this study. During carotid compression on the side of the subclavian steal, ipsilateral vertebral blood flow greatly decreased, and the amplitude the ipsilateral finger plethysmogram decreased slightly or moderately. It is suggested that there are significantly important factors in suppressing sumptoms of vertebrobasilar ischemia in these patients with Takayasu's arteritis with subclavian steal. These factors are believed to be (1) good function of the circle of Willis, (2) good blood supply to the brain stem, and (3) collateral circulation to the distal subclavian artery not via the vertebral artery.  (+info)

Major vascular anomalies in Turner syndrome: prevalence and magnetic resonance angiographic features. (5/58)

BACKGROUND: Turner syndrome (TS) is associated with aortic coarctation and dissection; hence, echocardiographic evaluation of all patients is currently recommended. X-ray angiography in clinically symptomatic patients has suggested a range of other vascular anomalies, but the true prevalence of such lesions in TS is unknown. To better understand the prevalence and pathogenesis of cardiovascular defects in TS, we prospectively evaluated a group of asymptomatic adult volunteers with TS using magnetic resonance (MR) angiography. METHODS AND RESULTS: A total of 85 adults with TS and 27 normal female adult volunteers underwent gadolinium-enhanced 3D MR angiography. A high prevalence of aortic anomalies was seen in women with TS, including elongation of the transverse arch (49%), aortic coarctation (12%), and aberrant right subclavian artery (8%). Venous anomalies were also prominent, including persistent left superior vena cava (13%) and partial anomalous pulmonary venous return (13%). None of these anomalies were found in healthy female controls. The constellation of elongation of the transverse arch, aortic coarctation, and persistent left superior vena cava was significantly associated with women with TS. Neck webbing and increased thoracic anterior-to-posterior dimension diameters were strong predictors for arterial and venous anomalies. CONCLUSIONS: Thoracic vascular anomalies are common in TS, occurring in approximately 50% of a group not preselected for cardiovascular disease. The highly significant association between neck webbing, increased chest diameter, and these vascular anomalies suggests that in utero, centrally localized lymphatic obstruction may contribute to these cardiovascular deformities in TS. Improved recognition of these often-undetected vascular lesions may be important for identification of patients in need of closer cardiovascular monitoring.  (+info)

Aortic root replacement with a freestyle stentless valve for aortitis syndrome with ascending aortic aneurysm and aortic regurgitation. (6/58)

A 47-year-old woman who had been diagnosed as having aortitis syndrome underwent aortic root replacement for an ascending aortic aneurysm and aortic regurgitation. Because the patient has been treated with steroids for more than 20 years, a Freestyle stentless valve was used to avoid the risk of valve detachment. There were no complications observed during the postoperative course. Although long-term follow-up will be necessary to observe the valve durability, the Freestyle stentless valve seems to be useful for aortic root replacement in patients at high risk of valve detachment due to aortitis syndrome.  (+info)

Protruding aortic arch thrombus: treatment with minimally invasive surgical approach. (7/58)

BACKGROUND: Protruding aortic arch thrombus is associated clinically with life-threatening emboli. Definitive treatment for aortic arch thrombus removal has demanded complicated vascular surgical procedures, with high morbidity and mortality. METHODS AND RESULTS: Transesophageal echocardiography (TEE) enabled diagnosis of a protruding thrombus at the aortic arch in 5 patients, and a simultaneous lesion in the descending aorta in 1 patient. Four patients had visceral emboli, coinciding with peripheral emboli in 2 patients, and the fifth patient had peripheral and cerebral emboli. One patient had had ischemic stroke and femoral emboli a few months previously. Mean patient age was 51 years. None had clinical evidence of coronary or peripheral atherosclerotic occlusive disease. Risk factors included hypertension (n = 2), smoking (n = 4), and preexisting thrombophilia (n = 4). Five patients underwent TEE-guided aortic balloon thrombectomy from the arch with a 34-mm occluding balloon catheter. One patient also underwent balloon thrombectomy from the descending aorta with a 14F Foley catheter. Access into the aorta was obtained through the iliac artery (n = 4) during laparotomy because of visceral ischemia or through the transfemoral approach (n = 2). Previous procedures included superior mesenteric embolectomy (n = 3), segmental bowel resection (n = 1), splenectomy (n = 1), and peripheral arterial embolectomy n = 3). Real-time intraoperative TEE enabled visualization of the protruding thrombus and assisted with maneuvering of the balloon catheter. At completion peripheral thrombectomy thrombus material was retrieved in 4 patients. Postoperatively there were no clinically proved new procedure-related visceral emboli, and all patients received anticoagulant therapy thereafter. Follow-up TEE within 2 weeks and up to 7 years revealed no recurrent aortic arch thrombus. CONCLUSIONS: TEE-guided aortic balloon thrombectomy used in 6 procedures was effectively completed without visceral or peripheral ischemic complications. It enabled removal of the life-threatening source of emboli from the proximal aorta, thereby averting the need of major aortic surgery.  (+info)

Unusual vascular ring anomaly in a foal. (8/58)

A 2.5-month-old filly was presented with signs of esophageal obstruction. The filly was euthanized and postmortem examination revealed a vascular ring anomaly. The vascular ring anomaly was not caused by a persistent right aortic arch, which is the only vascular ring anomaly reported to occur in horses.  (+info)

Management of Aortic stenosis is decided by its severity and location of narrowing.. Urgent treatment is required in severe aortic obstruction.. Medical follow-up is required most of the times in mild-moderate aortic obstruction.. In Valvular variety: aortic valve balloon dilatation is management of choice now a day.. (For detailed procedure, kindly refer to intervention section of balloon dilatation of aortic valve on www.childrenheartcare.com). ...
DISCUSSION. It is known that the majority of thoracic aortic diseases are degenerative and occur in association with risk factors for atherosclerosis such as smoking, hypertension and hypercholesterolemia[10] and all of those had a high prevalence in our sample.. One of the factors that impact morbimortality after aortic interventions are neurologic events and progression of aortic disease[11]. TEVAR is a safe and effective procedure to treat both aneurysms and dissections involving the descending aorta with relative low risk[12,13]. The landing zone was soon extended proximally in order to treat more extensive segments of the aorta, reducing the morbimortality when compared with conventional techniques[14-16].. New totally endovascular techniques for treatment of complex aortic arch diseases are available and include stenting of the supra-aortic vessels (parallel techniques), fenestrated or branched endografts. Those are promising, but experience in aortic arch repair are very limited[17] and ...
The U.S. Food and Drug Administration (FDA) recently approved the NuMED Cheatham Platinum (CP) Stent System for the prevention and/or treatment of aortic wall injury (AWI) in patients with coarctation of the aorta involving a compliant aortic isthmus or first segment of the descending aorta where there is adequate size and patency of at least one femoral artery and balloon angioplasty is contraindicated or predicted to be ineffective.. Stent therapy for coarctation of the aorta offers a non-surgical alternative to traditional operative repair of the aortic obstruction with similar results and complication rates. Stent treatment also has a lower rate of recurrence and aortic wall injury compared to balloon dilation alone.. Covered stent therapy provides similar outcomes and allows for simultaneous treatment of aortic wall injuries, such as aneurysm and pseudo-aneurysm that might be present, related to previous surgical or catheter based therapy of the coarctation. In the COAST II clinical trial, ...
In an attempt to improve myocardial performance in acute myocardial infarction with shock, increments of coronary perfusion pressure were achieved by partial obstruction of the abdominal aorta with a balloon catheter introduced via a femoral artery in 28 dogs with plastic sphere coronary embolization. Alterations of central aortic pressure, coronary sinus flow, cardiac output, left atrial pressure, left ventricular work, left ventricular oxygen consumption, coronary vascular resistance, left ventricular mechanical efficiency, and left ventricular lactate and pyruvate extraction were determined before and after coronary embolization and at intervals during 1 hour of abdominal aortic obstruction. After coronary embolization, aortic pressure, ...
In the early stages of fetal development, two aortic arches come from the heart, ascend upward and then descend behind the heart merging together to become a single aorta. As the heart develops normally, the right-sided arch disappears, leaving the left-sided arch to ascend upward and continue to the descending aorta behind the heart. The normally developed left-sided aorta lies in front of the trachea, or breathing tube, and esophagus, or swallowing tube.. A vascular ring is a defect where the arch vessels encircle the breathing and swallowing tubes.This is caused by abnormal development of the aortic arches. If both arches stay open it is called a double aortic arch. The two arches surround the breathing and swallowing tubes and may cause narrowing or compression of these structures and lead to breathing and/or feeding difficulties. In another form of a vascular ring, the left-sided arch disappears, and the right-sided arch stays open. If this is associated with an abnormal origin of the blood ...
Aberrant right subclavian arteries are the commonest of the aortic arch anomalies. A bulbous enlargement of the proximal segment of the aberrant right subclavian artery at its origin from the aortic arch is also demonstrated suggestive of Kommer...
Crouzon syndrome is an autosomal dominant genetic disorder known as a branchial arch syndrome. Specifically, this syndrome affects the first branchial (or pharyngeal) arch, which is the precursor of the maxilla and mandible. Since the branchial arches are important developmental features in a growing embryo, disturbances in their development create lasting and widespread effects. This syndrome is named after Octave Crouzon, a French physician who first described this disorder. He noted the affected patients were a mother and her daughter, implying a genetic basis. First called "craniofacial dysostosis", the disorder was characterized by a number of clinical features. This syndrome is caused by a mutation in the fibroblast growth factor receptor II, located on chromosome 10. Breaking down the name, "craniofacial" refers to the skull and face, and "dysostosis" refers to malformation of bone. Now known as Crouzon syndrome, the characteristics can be described by the rudimentary meanings of its ...
Kutsche and Van Mierop4 described CORSA in 1984. They found this anomaly in 4 of 21 infants (19%) studied for aortic arch interruption. The origin of the vessel was seen along with the origins of the internal and external carotid arteries as a trifurcation, at the level of the thyroid gland. It then descended down the neck to enter the right arm. The right recurrent laryngeal nerve was seen to course around the origin of the right subclavian artery, much higher than normal. The pathogenesis of CORSA can be explained by impairment of fourth aortic arch development, before the involution of the right ductus caroticus (segment of the dorsal aorta between the third and fourth arches). The right ductus caroticus, which normally involutes at the 14-mm crown-rump length, is retained and forms the initial segment of the right subclavian artery at its origin from the common carotid bifurcation. The seventh intersegmental artery and the dorsal aorta below the level of the third arch form the rest of the ...
Protocol: TRANSREG is a multicentric, uncontrolled, open-label study, comparing biological and clinical responses to the administration of low doses IL2 across 14 selected pathologies: rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus, psoriasis, Behcets disease, Wegeners granulomatosis, Takayasus disease, Crohns disease, ulcerative colitis, autoimmune hepatitis, sclerosing cholangitis, Gougerot-sjögren, Systemic Sclerosis and Idiopathic Thrombocytopenic Purpura. Methods: Each patient will receive 1MUI /day of IL2 from Day-1 to Day-5 (the induction period), and then every 2 weeks (except systemic lupus erythematosuss patients will received every week) from Day-15 to Day-180 (the maintenance period). Patients will thereafter be followed up for 12 months (Day-181-Day-540). For each pathology, 6 patients will be included at Pitié-Salpêtrière, Cochin, Saint Antoine, Paul Brousse and Henri Mondor hospitals in Paris and Créteil, France. An interim analysis will be ...
Introduction: Takayasus arteritis (TA) is a rare systemic, chronic inflam-matory, progressive, idiopathic disease of aorta and its main branches. Taka-yasus arteritis causes narrowing, occlusion and aneurysm of arteries. The eti-ology of TA is still unknown. It may be autoimmune or genetic in origin or infective diseases. It is commonly found in Asia and oriental countries. Worldwide annual incidence is 1.2 - 2.6 cases/million. Women are affected in 80% - 90% of cases with age of onset below 40 years. TA does not worsen or improve with pregnancy but has adverse effect on pregnancy in the form of abortion, Intrauterine fetal death, superimposed preeclampsia, Intrauterine growth restriction, abruption and congestive heart failure. Because the disease is common in women of childbearing age, management of pregnancy in these patients becomes important; however optimal management for pregnant pa-tients with this disease has not yet been established. Due to the manifold car-diovascular complications that can
DOI: http://dx.doi.org/10.5915/20-4-13298. A middle-aged male patient is described who suffered left hemiplegia because of occlusion of the brachiocephalic and right common carotid artery. He was found to have Takayasus Arteritis. An in-depth review of the history of arteritis, especially as related to nosology, is presented. Literature is reviewed with emphasis on the neurologic and cardiovascular manifestations. Etiology and pathology are briefly discussed.. ...
BACKGROUND: The present paper reports the preliminary experience with surgical treatment of 49 cases of cerebral ischaemia caused by cervical arterial lesions due to Takayasus arteritis (TA). METHODS: Six men and 43 women were treated between June 1
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Full Papers; Authors: C. Arslan>, C. Tel>, B. Arapi>, S. Esatoglu>, K. Besirli>, A. Bozkurt>, V. Hamuryudan>, H. Tuzun>, E. Seyahi>
CONCLUSIONS: TAK does co-occur with IBD, AS and less frequently with BS in about 1/5 of the patients, at least in a hospital setting. There is no clear temporal pattern. The high prevalence of inflammatory back pain in the dorsal spine in TAK needs further scrutiny. PMID: 31969224 [PubMed - as supplied by publisher]...
Hey all. Im not here often as my kiddos issue was minor (ASD) and diagnosed at 6 months. It was just a waiting game to see if it would close until her 4 year check, which just occurred on 9/6. At that time, she had a complete echocardiogram, which had not been done since she was diagnosed with the ASD at 6 months - reason being is her pediatric cardio wasnt going to intervene until she hit 40 lbs., so he didnt think it was necessary. She always had an EKG at her check-ups, and that was it. Anywho, she had the echo and needless to say, my very hyper daughter was not the best patient. They seemed to take forever. They were taking shots off and on for about 1.5 hours. Tech called in cardiologist who was trying to get a shot, but having trouble as well. Finally, cardiologist tells us that the ASD is closed. I say - wait, even *I* can still here her murmur clear as day. She says, We need to talk. We go in and she draws a picture and explains in too simple terms that Lauren has a right
Vascular ring anomalies occurs when a congenital heart abnormality causes the esophagus to become compressed. Learn why and how to treat it.
A 46-year-old man was given a diagnosis of hypertension about 20 years previously. At age 41, aortitis syndrome was diagnosed, with descending thoracic aortic aneurysm and the coarctation of abdominal aorta by CT scan. He then underwent surgery to replace the descending thoracic aortic aneurysm and right axillo-bifemoral bypass. Recently, a thoraco-abdominal aortic aneurysm was pointed out at the distal site of the graft and, he was referred to our institute. We occluded the distal end of the aneurysm using an endoluminal occlusion stent graft. Today, in most cases of aortopathy associated with aortitis syndrome, surgical replacement of the aneurysms and extra-anatomical bypass is performed. An endovascular stent graft treatment combined with extra-anatomical bypass could be useful for various aortic disorders ...
TY - JOUR. T1 - Renal manifestations in toddlers with Takayasus arteritis and malignant hypertension. AU - Hijazi, Rana. AU - Chandar, Jayanthi. AU - Nwobi, Obioma. AU - Muneeruddin, Samina. AU - Zilleruelo, Gastón. AU - Abitbol, Carolyn L.. PY - 2009/1/1. Y1 - 2009/1/1. N2 - Three children under the age of 3 years presented with malignant hypertension, proteinuria, and acute kidney injury. Takayasus arteritis was diagnosed on the basis of clinical symptoms of weight loss and low grade fever in conjunction with elevated sedimentation rate and radiographic evidence of aortic and renal artery stenosis. One patient had a renal biopsy which showed arteriolar sclerosis and focal glomerulosclerosis. All three patients required multiple antihypertensive agents, ultimately including angiotensin receptor blockers and/or angiotensin converting enzyme inhibitors. The vasculitis was treated with pulse corticosteroids followed by cyclophosphamide in one patient and mycophenolate mofetil as maintenance ...
Disease affecting the arterial supply to the kidneys, leading to ischaemia and resultant stimulation of the renin-*angiotensin-aldosterone axis. In the major vessels, the most common cause is atheromatous plaque disease. Other causes are fibromuscular dysplasia and Takayasus disease. ...
Takayasus arteritis is a chronic granulomatous vasculitis of unknown cause characterized by stenosis, occlusion, and aneurysm of large elastic arteries, mainly the aorta and its branches. It mainly affects young women, predominantly in Asian, Middle Eastern, and South American countries.. Clinical presentation-symptoms in the acute stage are nonspecific, such as general fatigue and fever, which can persist for months to years. Symptoms in the chronic stage depend on the anatomical location of the vascular lesions, with typical complaints relating to ischaemia of the brain, eyes, or arms. The commonest finding on physical examination is a weak or absent pulse in one or both brachial, radial, and/or ulnar arteries. Bruits can often be heard over affected arteries. Hypertension and aortic insufficiency are strongly associated with poor prognosis.. Diagnosis-comprehensive angiographic imaging is required for diagnosis, evaluation of the extent of disease, and to guide therapy. American College of ...
Takayasus arteritis is a chronic granulomatous vasculitis of unknown cause characterized by stenosis, occlusion, and aneurysm of large elastic arteries, mainly the aorta and its branches. It mainly affects young women, predominantly in Asian, Middle Eastern, and South American countries.Clinical presentation-symptoms in the acute stage are nonspecific, such as general fatigue and fever, which can persist for months to years. Symptoms in the chronic stage depend on the anatomical location of the vascular lesions, with typical complaints relating to ischaemia of the brain, eyes, or arms. The commonest finding on physical examination is a weak or absent pulse in one or both brachial, radial, and/or ulnar arteries. Bruits can often be heard over affected arteries. Hypertension and aortic insufficiency are strongly associated with poor prognosis.Diagnosis-comprehensive angiographic imaging is required for diagnosis, evaluation of the extent of disease, and to guide therapy. American College of Rheumatology
Right aortic arch with aberrant left subclavian artery (RAA/aLSCA) is a rare aortic arch anomaly. The clinical association of aLSCA stenosis with RAA/aLSCA has not yet been fully elucidated. The aim of this study was to investigate the diagnosis, incidence, management and outcome of aLSCA stenosis in infants with prenatally diagnosed RAA/aLSCA. Ten fetuses who were diagnosed as having RAA/aLSCA in Kyushu University Hospital between January 2011 and December 2014 were enrolled. The maternal and child medical records were reviewed to investigate sex, gestational age at the fetal diagnosis, gestational age and body weight at birth, the findings of computed tomography (CT), Doppler ultrasonography of the vertebral artery and angiography, and the complications and outcomes of aLSCA stenosis ...
Case Reports in Obstetrics and Gynecology is a peer-reviewed, Open Access journal that publishes case reports in all areas of obstetrics and gynecology.
Congenital anomaly of the aortic arch is a rare and usually an incidental finding. Vascular rings constitute less than 1% of the congenital cardiovascular diseases and were first identified by Gross in 1945 [1]. Double aortic arch is the most frequent form of arch anomaly that requires surgery for tracheal or oesophageal compression [2]. Respiratory symptoms at birth or during infancy should raise the possibility of a vascular ring. The classic history in a patient with double aortic arch is noisy breathing in the first few weeks of life. Rarely, neonates may present with Acute life-threatening event (ALTE) characterized by episodes of acute apnea and cyanosis ...
Anatomic variants of the aortic arch and its branches are relatively common, with an estimated prevalence of 0.5% to 3%.1 The majority of these variations ar...
Click on the image to enlarge) From Jim Locks Green Book (Cath book) 2nd edition. 2000. page 58. Data reportedly pooled from se ...
INTRODUCTION: Floating thrombus in an aberrant right subclavian artery is a rare cause of peripheral arterial embolic events. CASE REPORT: We report a 45-year-old woman who presented with an ischemia of the right superior limb from embolic event. The diagnosis of mobile thrombus in an aberrant right subclavian artery was obtained with transoesophageal echocardiography and computed tomography. After three weeks of oral anticoagulant therapy, there was no significant resolution of the thrombus, and a surgical treatment was performed to prevent further embolization. The surgical procedure consisted of thrombectomy and reimplantation of the aberrant right subclavian artery to the right carotid artery. Postoperative recovery was uneventful. CONCLUSION: This case report illustrates that transoesophageal echography and computed tomography are useful to detect mobile thrombus of the thoracic aorta and is warranted in any embolic event in young patients.
A two-year-old Border Collie presented with a three-month history of regurgitation. Investigation with plain radiography, digital fluoroscopy, endoscopy and CT angiography (CTA) confirmed the presence of an aberrant right subclavian artery causing dorsal oesophageal compression. In this report, CTA was used to depict the anatomy of an aberrant right subclavian vessel and to highlight the importance of this imaging modality to identify the structures involved in a vascular ring. This report also identifies a patient with a congenital vascular ring anomaly presenting with adult-onset regurgitation, which has been successfully managed with medical treatment. ...
Takayasus arteritis is a chronic inflammatory condition that affects the aorta, the largest blood vessel in the body, and its branches. Inflammation causes segments of the vessels to become narrowed, blocked, or even stretched, possibly resulting in aneurysms. The disease is very rare but most commonly occurs in young Asian women. Symptoms may include arm pain with use, decreased or absent pulses, lightheadedness or dizziness, headaches, and visual disturbances. Many individuals with Takayasu arteritis, however, have no apparent symptoms despite disease activity. Additionally, current tests used to measure vessel inflammation are considered unreliable and inconsistent. It is therefore very difficult to determine the extent of disease activity in a person with Takayasus arteritis. This study will use novel scientific methods to establish new biomarkers that can be used to monitor disease activity in individuals with Takayasus arteritis. These biomarkers may be used to help direct clinical ...
a Four branches arises from the aortic arch. Aberrant right subclavian artery(ARSA) marked by astrex. b ARSA arises distal to left subclavian artery. c Obstruct
Idiopathic dilatation of the pulmonary trunk is a rare congenital anomaly comprising of pulmonary trunk enlargement with or without dilatation of the right and left pulmonary arteries. For this diagnosis, exclusion of pulmonary and cardiac disea...
TY - JOUR. T1 - Management of arch hypoplasia after successful coarctation repair. AU - DeLeon, Maryann M.. AU - DeLeon, Serafin Y.. AU - Quinones, Jose A.. AU - Roughneen, Patrick. AU - Magliato, Kathy E.. AU - Vitullo, Dolores A.. AU - Cetta, Frank. AU - Bell, Timothy J.. AU - Fisher, Elizabeth A.. PY - 1997. Y1 - 1997. N2 - Background. Pronounced arch obstruction can be seen after a well-repaired coarctation, and this probably results from the failure of a somewhat hypoplastic arch to grow or from clamp injury at the time of the initial repair, or from both causes. Because of mediastinal adhesions and minimal collateral circulation, use of extraanatomic bypass grafts appears to be the preferred approach. Methods. Six children or young adults presented with arch obstruction over a 3-year period. Their mean age was 13.5 ± 4 years, and the mean interval from the time of the initial repair was 10 ± 4 years. The mean age of the patients at the time of the initial repair was 3.2 ± 5 years. ...
Abstract: An unusual vascular ring anomaly consisting of a persistent right aortic arch and a left ligamentum arteriosum extending from the main pulmonary artery to an aberrant left subclavian artery and left aortic arch remnant complex was identified in a German shepherd dog and a great Dane. The left subclavian artery and left aortic arch remnant complex originated at the junction between the right distal aortic arch and the descending aorta and coursed dorsal to the oesophagus in a cranial direction. The attachment of the ligamentum arteriosum to the aberrant left subclavian artery was approximately 5 cm cranial to the point of origin of the aberrant left subclavian artery and left aortic arch remnant complex from the descending aorta in both dogs. This anomaly observed in both dogs is similar to an anomaly reported in humans, in which a persistent right aortic arch is found in conjunction with an aberrant left subclavian artery and a left aortic arch remnant (Kommerells diverticulum). ...
Looking for online definition of pulmonary trunk in the Medical Dictionary? pulmonary trunk explanation free. What is pulmonary trunk? Meaning of pulmonary trunk medical term. What does pulmonary trunk mean?
OBJECTIVE: To discuss the diagnosis and surgical treatment of congenital vascular ring and prognostic factors. METHODS: The clinic data of 42 cases of congenital vascular from January 2010 to December 2013 was analyzed retrospectively (accounting for 1.04% congenital heart operations over the same period ). There were 26 male and 16 female patients, aged at surgery 24 days to 6 years (average 10.7 months). The diagnosis including pulmonary artery sling in 26 cases, double aortic arch in 10 cases, right aortic arch with aberrant left subclavian artery in 3 cases, pulmonary artery sling and right aortic arch with vagus left subclavian artery in 2 cases, pulmonary artery sling and left aortic arch with vagus right subclavian artery in 1 case ...
Aberrant right subclavian artery (ARSA) or arteria lusoria is one of the most common congenital aortic arch anomaly with prevalence ranging from 1-2%. However, it is mostly asymptomatic and consequently often found sporadically during various diagnostic procedures with prevalence of 0.45% during coronary angiography.
Aberrant right subclavian artery (ARSA), also called "lusorian artery", is a well known anatomical variant of the origin of the right subclavian artery, one of the most common congenital vascular abnormalities of the aortic arch (the prevalence among the population is 1-2% in according to different authors) [1-4]. Usually ARSA arises after the left subclavian artery and crosses the mediastinum behind the oesophagus and the trachea. For its particular position ARSA can cause compression to the close anatomical structures. Dysphagia lusoria is the most frequent symptom (in 10 % of cases). For the same reason the prolonged compression caused by medical devices such as nasogastric or endotracheal tubes on the arterial wall of the ARSA can cause severe injury, especially when an ARSA aneurysm is present. In rare cases it can lead up to an oesophageal fistula with profuse bleeding characterised by high mortality rate (till now less than 20 cases were reported in literature). In our case fistulisation ...
Teaching Files with CT Medical Imaging and case studies on Anatomical Regions including Adrenal, Colon, Cardiac, Stomach, Pediatric, Spleen, Vascular, Kidney, Small Bowel, Liver, Chest | CTisus
This is 3d model of aortic arch with bicarotid trunk anomaly and aberrant right subclavian artery (arteria lusoria), which was made from CTA scan. The patient is caucasian woman with stenosis of the left vertebral artery (which is not included) and severe disphagia. Its part of a anatomical serie...
CT examination of the chest with IV contrast performed accoring to a PE/DVT protocol demonstrated an aberrant right subclavian artery. The mediastinum was othe
Your forearms and hands should be strengthened if you to handle the ball better. Tips For Improving Your NBA Live Mobile Skills Today NBA Live Mobile season tends to be an exciting time of year...
A-16-year-old male adolescent with a 4-year history of protean clinical manifestations such as fever, abdominal pain, back pain, erythema nodosum and uveitis developed complete occlusion of left renal artery. Although he had been suspicious of having
B. Right lateral view clearly demonstrates posterior indentation in the esophagram confirming the presence of a vascular ring. This child on further evaluation was found to have a right aortic arch with a left ductus arteriosus remnant. ...
Double aortic arch is a relatively rare congenital cardiovascular malformation. Double aortic arch is an anomaly of the aortic arch in which two aortic arches form a complete vascular ring that can compress the trachea and/or esophagus. Embryologically, the aortas right sided 4th arch failed to r...
The fact that a right aortic arch with a constricting vascular ring was found in a body that had been bequeathed to the Department of Anatomy enabled a detailed correlation to be made between the clinical history, previous radiological examinations, and the postmortem findings.
Patients with suspicious or known heart disease undergo cardiac CT. AI algorithm identifies the caliber of pulmonary arteries. After evaluation of indexed dimensions, the algorithm automatically inserts the dimensions of pulmonary arteries in the report, specifying if the vessels are enlarged or reduced ...
PROGNOSIS: Mild subaortic stenosis is of no consequence to an individual dog and it is usually recommended that the dos not be bred. Moderate or severe subaortic stenosis may cause symptoms and may increase the risk of sudden death. Depending on the exact degree of severity, treatment may be required, and other measures (such as limiting activity) may be recommended to minimize the amount of work done by the heart. There is no cure for subaortic stenosis. Since it is thought to be of genetic origin it is often recommended that dogs with subaortic stenosis not be bred to avoid passing the disease along to future generations.. TREATMENT/PROCEDURE: If the disease is mild, treatment is not required. However, subaortic stenosis can get worse as a growing dog reaches its adult age and body size. Therefore, dogs with moderate or severe subaortic stenosis, may require medication. The most common form of treatment is a medication given orally called βeta blockers, which reduce the intensity of the ...
HLA-B52 (B52) is an HLA-B serotype. The serotype identifies the more common HLA-B*52 gene products. B52 is a split antigen of the broad antigen B5, and is a sister type of B51. B*5201 likely formed as a result of a gene conversion event between another HLA-B allele and HLA-B*5101. There are a number of alleles within the B*52 allele group. There are 18 alleles, with 14 amino acid sequence variants in B52. Of these only 9 are frequent enough to have been reliably serotyped. B*5201 is the most common, but others have a large regional abundance. HLA-B52 appears to have the strongest linkage to ulcerative colitis in Japan. This form of disease is frequently found with Takayasus arteritis. Takayasus arteritis appears to have an independent link to B52 associated disease. The association with B*5201 increases risk of pulmonary infarction, ischemic heart disease, aortic regurgitation, systemic hypertension, renal artery stenosis, cerebrovascular disease, and visual disturbance. Marsh SG, Albert ED, ...
Results Conventional echocardiographic measurements (LV end diastolic diameter, LV end systolic diameter and LV EF) were similar between the groups. Longitudinal peak systolic strain (13.20±3.5% to 20.97±4.5%, p=0.0001) and strain rate (0.23±0.18 1/s to 4.92±0.55 1/s, p=0.0001) of the LV were significantly impaired in patients with TA, compared to controls, demonstrating subclinical ventricular systolic dysfunction. Supporting these results, we revealed impaired subclinical LV systolic function in patients with SLE, when compared to the control group (14±4.53% to 20.97±4.5%, p=0.0001 for strain; 0.23±0.12 1/s to 4.92±0.55 1/s, p=0.0001 for strain rate). The mean disease duration of the patient was 5.6 years in patients with TA. The LV systolic dysfunction was negatively correlated with disease duration, but this correlation couldnt reach the statistical significance(r=-0,085, p=0,648.). ...
Pulmonary trunk aka Truncus pulmonalis in the latin terminology and part of structures of the left atrium and ventricle. Learn more now!
Results Arteriographic lesions were identified in 145 patients with TAK and 62 patients with GCA. Cluster analysis demonstrated that arterial involvement was contiguous in the aorta and usually symmetric in paired branch vessels for TAK and GCA. There was significantly more left carotid (p=0.03) and mesenteric (p=0.02) artery disease in TAK and more left and right axillary (p,0.01) artery disease in GCA. Subclavian disease clustered asymmetrically in TAK and in patients ≤55 years at disease onset and clustered symmetrically in GCA and patients ,55 years at disease onset. Computer derived classification models distinguished TAK from GCA in two subgroups, defining 26% and 18% of the study sample; however, 56% of patients were classified into a subgroup that did not strongly differentiate between TAK and GCA.. ...
Double aortic arch is a common form of a group of defects that affect the development of the aorta in the womb. These defects cause an abnormal formation called a vascular ring (a circle of blood vessels).. Normally, the aorta develops from one of several curved pieces of tissue (arches). As babies develop in the womb, the arches split into several parts. The body breaks down some of the arches, while others form into arteries. A normally developed aorta is a single arch that leaves the heart and moves leftward.. In double aortic arch, some of the arches that should have disappeared are still present at birth in addition to the normal arch. Babies with a double aortic arch have an aorta that is made up of two vessels instead of one. The two parts to the aorta have smaller arteries branching off of them. As a result, the two branches go around and press down on the windpipe and the tube (esophagus) that carries food from the mouth to the stomach. A double aortic arch may occur in other congenital ...
... is a disorder that weakens a persons muscles over time. People who have the disease can gradually lose the ability to do everyday tasks.
An important management concern in regard to patients with subaortic stenosis has been progression of LVOTO and the development of AI. The medical follow up and the timing of surgery has been strongly influenced by the impression of almost inevitable progression in this regard.1 9 11 21 Our findings indicate that a large proportion of patients diagnosed with mild subaortic stenosis at our centres over a recent 14 year period had little progression of LVOTO. Other patients with more significant subaortic stenosis showed notable and rapid increases in LVOTO. Although we found a major incidence of AI at diagnosis that increased with time, this AI remained mild in all of our patients during medical follow up. The echograd at diagnosis was predictive of the progression of LVOTO and of the appearance of new AI. Similar findings have been reported by deVries and colleagues.12 However, other older reports based on cardiac catheterisation data have shown that LVOTO progresses notably, as was seen in some ...
Twenty-one patients with muscular subaortic stenosis were treated with oral propranolol for periods of 6 to 34 months for a total of 42.5 patient years. The average follow-up was 2 years. Four patients with latent obstruction became asymptomatic on propranolol therapy. Of the 17 patients with resting obstruction, 7 improved, 2 were unchanged, 5 deteriorated, and 2 died during the period of treatment. The 7 patients with resting obstruction who are still improved on propranolol have had relatively short periods of treatment (average 15 months), and none experienced the degree of improvement that occurred in the patients with latent obstruction. This study indicates that propranolol is most effective in patients with muscular subaortic stenosis who have latent obstruction. It is of limited value in patients with resting obstruction because the benefit of propranolol therapy in the majority of these patients is eventually overtaken by progression in the disease. ...
18F-flurodeoxyglucose (FDG) positron emission tomography (PET) provides information about vascular inflammation that is complimentary to, and unique from, clinical assessment in large-vessel vasculitis (LVV), a recent study found. Furthermore, FDG-PET scan activity during clinical remission was associated with future clinical relapse. Patients with Takayasus arteritis (TAK) and giant cell arteritis (GCA) were studied, along with a comparator group consisting of patients with hyperlipidemia, diseases that mimic LVV, and healthy controls.
Background: Some respiratory diseases may induce alveolar hypoxia thereby hypoxic pulmonary vasoconstriction (HPV). This study was the first to investigate the role of anion exchanger in sustained HPV.. Methods: Experiments were performed in the isolated perfused rabbit lung. In the HCO3- free groups, HEPES were added to the perfusate instead of bicarbonate. In the HEPES1 and HEPES2 groups, the lungs were ventilated with hypoxic gas with or without CO2 respectively.. Results: Ventilation the lungs with hypoxic gas resulted in biphasic HPV. No alteration in both phases of HPV was detected by DIDS (anion exchanger inhibitor,200 microM). However, DIDS (400 microM), extended ascending part of acute HPV until min 24. Both phases of HPV were decreased in the HEPES1 group. But in the HEPES 2 group, HPV tended to increase significantly during rising part of acute phase with no change during sustained phase.. Conclusions: This study is suggesting that anion exchanger may modulate HPV especially during ...
Pages that link to "Paper - On an instance of two subclavian arteries of the early arm bud of man and its fundamental significance ...
Coarctations are most common in the aortic arch. The arch may be small in babies with coarctations. Other heart defects may ... Some signs that can lead to a coarctation have been linked to pathologies such as Turner syndrome, bicuspid aortic valve, and ... This spectrum is dichotomized by the idea that aortic coarctation occurs in the aortic arch, at or near the ductus arteriosis, ... Aortic coarctation and aortic stenosis are both forms of aortic narrowing. In terms of word root meanings, the names are not ...
Milan B, Josip K (November 1967). "Ocular manifestations of the aortic arch syndrome (pulseless disease; Takayasu's disease) ( ... Takayasu's arteritis (also known as Takayasu's disease, "aortic arch syndrome," "nonspecific aortoarteritis," and "pulseless ... In the Western world, atherosclerosis is a more frequent cause of obstruction of the aortic arch vessels than Takayasu's ... Fainting may result from subclavian steal syndrome or carotid sinus hypersensitivity. There is also often anemia and marked ...
... arch a series of bony "loops" present in fish, which support the gills. Branchial artery, also known as aortic arches ... First and second branchial arch syndrome, also known as hemifacial microsomia. Ultimo-branchial bodies, also known as ...
Dissection of the aortic arch is rare due to medial scarring. As a result of this advanced disease process, standard methods of ... This disorder is also known eponymously as Heller-Döhle syndrome.[citation needed]. ... This leads to narrowing of the lumen of the vasa vasorum, causing ischemic injury of the medial aortic arch and then finally ... Inflammatory involvement of tertiary syphilis begins at the adventitia of the aortic arch which progressively causes ...
Thoracic aortic aneurysm. Hybrid arch debranching Thoracic endovascular aneurysm repair. Thoracic outlet syndrome. Surgical ... Vascular surgery also involves surgery of veins, for conditions such as May-Thurner syndrome and for varicose veins. In some ... August 1994). "Design of the abdominal aortic Aneurysm Detection and Management Study. ADAM VA Cooperative Study Group". J Vasc ... Conclusion: Intraoperative heparin, given before aortic cross clamping, is an important prophylaxic against perioperative MI in ...
In the normal embryological development of the aortic arches, the right dorsal aorta regresses caudal to the origin of the 7th ... Aberrant subclavian artery, or aberrant subclavian artery syndrome, is a rare anatomical variant of the origin of the right or ... This abnormality is the most common congenital vascular anomaly of the aortic arch, occurring in approximately 1% of ... Chaoui, R; Rake, A; Heling, KS (2008). "Aortic arch with four vessels: aberrant right subclavian artery". Ultrasound in ...
"Tbx1 haploinsufficieny in the DiGeorge syndrome region causes aortic arch defects in mice". Nature. 410 (6824): 97-101. doi: ... Most cases of 22q11.2 deletion syndrome are caused by the deletion of a small piece of chromosome 22. This region of the ... Jerome LA, Papaioannou VE (March 2001). "DiGeorge syndrome phenotype in mice mutant for the T-box gene, Tbx1". Nature Genetics ... Baldini A (October 2003). "DiGeorge's syndrome: a gene at last". Lancet. 362 (9393): 1342-3. doi:10.1016/S0140-6736(03)14671-5 ...
Tricuspid atresia Interrupted aortic arch Pulmonary atresia (PA) Pulmonary stenosis (critical) Eisenmenger syndrome (reversal ... Total anomalous pulmonary venous connection Hypoplastic left heart syndrome (HLHS) Transposition of the great arteries (d-TGA) ...
The crura connect to form an arch, behind which is the aortic hiatus, through which pass the aorta, the azygos vein, and the ... Compression of celiac artery and celiac ganglia by the median arcuate ligament can lead to the median arcuate ligament syndrome ... "Median arcuate ligament syndrome". Curr Treat Options Cardiovasc Med. 10 (2): 112-6. doi:10.1007/s11936-008-0012-2. PMID ...
The proximal pulmonary artery is then connected to the hypoplastic aortic arch, while the coarcted segment of the aorta is ... "Physiologic Repair of Aortic Atresia-Hypoplastic Left Heart Syndrome". New England Journal of Medicine. 308 (1): 23-26. doi: ... This procedure is most often performed to treat hypoplastic left heart syndrome, certain types of mitral atresia, or other ... "Experience with operations for hypoplastic left heart syndrome". The Journal of thoracic and cardiovascular surgery. 82 (4): ...
... critical aortic stenosis, and interrupted aortic arch). Alprostadil is sold in the United States as urethral suppositories and ... hypoplastic left heart syndrome, pulmonary atresia/stenosis, tricuspid atresia/stenosis, transposition of the great arteries) ...
... the 4th aortic arch and dorsal aorta form the aortic arch on the left, but since the right dorsal aorta regresses distal to the ... Aberrant subclavian artery Subclavian steal syndrome Thoracic outlet syndrome This article incorporates text in the public ... On the left side of the body, the subclavian comes directly off the aortic arch, while on the right side it arises from the ... They receive blood from the aortic arch. The left subclavian artery supplies blood to the left arm and the right subclavian ...
Interruption of aortic arch (747.2) Other congenital anomalies of aorta (747.3) Congenital anomalies of pulmonary artery (747.4 ... Down syndrome (758.1) Patau's syndrome (758.2) Edward's syndrome (758.3) Autosomal deletion syndromes (758.31) Cri du chat ( ... Gonadal dysgenesis Turner's syndrome XO syndrome (758.7) Klinefelter's syndrome (758.8) Other conditions due to sex chromosome ... Prader-Willi syndrome (759.82) Marfan syndrome (759.83) Fragile X syndrome (759.89) Other specified congenital anomalies (759.9 ...
... such as aortic outflow tract obstruction, mitral stenosis, aortic arch syndrome) etc. A bounding pulse signifies high pulse ... Pulsus bisferiens: an unusual physical finding typically seen in patients with aortic valve diseases. If the aortic valve does ... unless there is coexisting aortic regurgitation). The delay can also be observed in supravalvar aortic stenosis. Several pulse ... It is seen in aortic valve stenosis. Pulsus paradoxus: a condition in which some heartbeats cannot be detected at the radial ...
Aortic arch syndrome (Takayasu) (M31.5) Giant cell arteritis with polymyalgia rheumatica (M31.6) Other giant cell arteritis ( ... Sjögren's syndrome (Sicca syndrome) (M35.1) Other overlap syndromes Mixed connective tissue disease (M35.2) Behçet's disease ( ... Rotator cuff syndrome (M75.2) Bicipital tendinitis (M75.3) Calcific tendinitis of shoulder (M75.4) Impingement syndrome of ... Churg-Strauss syndrome) (M30.2) Juvenile polyarteritis (M30.3) Mucocutaneous lymph node syndrome (Kawasaki) (M30.8) Other ...
"MRI and FDG-PET in the Assessment of Inflammatory Aortic Arch Syndrome in Complicated Courses of Giant Cell Arteritis." Annals ... Inflammatory aortic aneurysm (IAA), also known as Inflammatory abdominal aortic aneurysm (IAAA), is a type of abdominal aortic ... and measure inflammatory aortic arch syndrome. The results from the study were that MRI and FDG-PET were unreliable techniques ... "Aortic Aneurysm: Causes, Symptoms, Treatments, and More." WebMD. WebMD, n.d. Web. 22 July 2015. "Abdominal Aortic Aneurysm: ...
... syndrome Antley-Bixler syndrome Anton-Babinski syndrome Aortic arch syndrome Aortocaval compression syndrome Apert syndrome ... syndrome Wende-Bauckus syndrome Werner syndrome Wernicke-Korsakoff syndrome West syndrome Westerhof syndrome Wet lung syndrome ... syndrome Activation syndrome Acute aortic syndrome Acute brain syndrome Acute chest syndrome Acute coronary syndrome Acute HME ... syndrome Fetal warfarin syndrome FG syndrome Fibrinolysis syndrome Fibromyalgia syndrome Fibromyalgia First arch syndrome Fish ...
... is often associated with DiGeorge syndrome. It can be diagnosed with an echocardiogram. Patients will ... Interrupted aortic arch is a very rare heart defect (affecting 3 per million live births) in which the aorta is not completely ... Messner, Greg; Reul, George J.; Flamm, Scott D.; Gregoric, Igor D.; Opfermann, Ulrich Tim (2002). "Interrupted aortic arch in ... C.S. Mott Children's Hospital, Congenital Heart Center: Interrupted Aortic Arch at umich.edu Heart center encyclopedia at ...
... typically of the aortic arch and is classically found in Turner syndrome. A "complete" coarction is called an interrupted ... Aortic valve replacement - Replacement of the aortic valve due to aortic regurgitation, aortic stenosis, or other reasons. A ... a syndrome). The top two syndromic causes of congenital heart defects are Noonan syndrome and Down syndrome. Down syndrome is ... Aortic valve repair - Repair, instead of replacement, of the aortic valve. Aortic valvuloplasty - Repair of the valve by using ...
Placing the balloon too distal from the aortic arch may induce occlusion of the renal artery and subsequent kidney failure. ... and compartment syndrome. The leg is at highest risk of becoming ischemic if the femoral artery it is supplied by becomes ... The following conditions will always exclude patients for treatment: Severe aortic valve insufficiency Aortic dissection Severe ... The intra-aortic balloon pump (IABP) is a mechanical device that increases myocardial oxygen perfusion while at the same time ...
The proximal pulmonary artery is connected to the hypoplastic aortic arch, while the narrowed segment of the aorta is repaired ... Sharma J, Friedman D, Schiller M, Flynn P, Alonso ML (December 1997). "Aortic stenosis in hypoplastic right heart syndrome, ... Hypoplastic right heart syndrome is less common than hypoplastic left heart syndrome which occurs in 4 out of every 10,000 ... Hypoplastic right heart syndrome is a congenital heart defect in which the right atrium and right ventricle are underdeveloped ...
This fibrous arch forms the anterior aspect of the aortic hiatus, through which the aorta, thoracic duct, and azygos vein pass ... It has also been called Harjola-Marable syndrome and Marable syndrome. Superior mesenteric artery syndrome Nutcracker syndrome ... also known as celiac artery compression syndrome, celiac axis syndrome, celiac trunk compression syndrome or Dunbar syndrome) ... Median arcuate ligament syndrome (celiac artery compression syndrome)". Radiographics. 13 (6): 1400-2. doi:10.1148/ ...
"Tbx1 haploinsufficieny in the DiGeorge syndrome region causes aortic arch defects in mice". Nature. 410 (6824): 97-101. doi: ... Shprintzen syndrome, DiGeorge sequence/syndrome, Sedlackova syndrome, and conotruncal anomaly face syndrome. All are now ... DiGeorge syndrome, also known as 22q11.2 deletion syndrome, is a syndrome caused by the deletion of a small segment of ... McDonald-McGinn, Donna (2011). "Chromosome 22q11.2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome)". Medicine ( ...
... including septal defects and aortic arch anomalies, renal cysts, urinary tract malformations, and distal limb hypoplasia. Most ... noted the phenotypic overlap between Fryns syndrome and the Pallister-Killian syndrome (601803), which is a dysmorphic syndrome ... a new lethal syndrome". J Genet Hum. 28 (1): 57-60. PMID 7400786. Fitch N (February 1988). "Fryns syndrome". J. Med. Genet. 25 ... Fryns syndrome is an autosomal recessive multiple congenital anomaly syndrome that is usually lethal in the neonatal period. ...
Angina Acute coronary syndrome Anomic aphasia Aortic dissection Aortic regurgitation Aortic stenosis Apoplexy Apraxia ... Tricuspid atresia Interrupted aortic arch Coarctation of aorta Pulmonary atresia (PA) Pulmonary stenosis (critical) Atrial ... Lenegre-Lev syndrome) Long QT syndrome Lown-Ganong-Levine syndrome Multifocal atrial tachycardia Wolff-Parkinson-White syndrome ... Acyanotic heart defect Atrial septal defect Cor triatriatum Dextro-Transposition of the great arteries Double aortic arch ...
Arch. Dermatol. Res. 302 (1): 47-55. PMC 2799629. . PMID 19701759. doi:10.1007/s00403-009-0989-8. !CS1 manut: Nomes múltiplos: ... 2009). «Lack of Association of Polymorphisms in Elastin With Pseudoexfoliation Syndrome and Glaucoma». Journal of glaucoma. 19 ... and characterization of seven novel mutations of elastin gene in a cohort of patients affected by supravalvular aortic stenosis ... 2004). «A novel elastin gene mutation resulting in an autosomal dominant form of cutis laxa». Arch Dermatol. 140 (9): 1135-9. ...
M31.4 - Aortic arch syndrome [Takayasu]. SNOMEDCT:. 359789008 - Takayasus disease. Look For. Subscription Required ... aortic coarctation or middle aortic syndrome; unlikely to have constitutional symptoms. *Marfan syndrome - look for ... Takayasu arteritis, also referred to as pulseless disease and aortic arch syndrome, is a rare chronic inflammatory vasculitis ... Ehlers-Danlos syndrome - look for fragile skin, easy bruising, joint hyperextendability, frequent dislocations ...
Aortic arch syndrome, group of disorders that cause blockage of the vessels that branch off from the aorta in the area in which ... the aorta arches over the heart. The aorta is the principal vessel through which the heart pumps oxygen-rich blood into the ... Aortic arch syndrome, group of disorders that cause blockage of the vessels that branch off from the aorta in the area in which ... A rare form of the aortic arch syndrome that primarily affects Oriental women is called Takayasus disease, or nonspecific ...
Aortic arch syndrome refers to a group of signs and symptoms associated with structural problems in the arteries that ... The aortic arch is the top part of the main artery carrying blood away from the heart. ... The aortic arch is the top part of the main artery carrying blood away from the heart. Aortic arch syndrome refers to a group ... Aortic arch syndrome problems can be due to trauma, blood clots, or malformations that develop before birth. These defects ...
Aortic arch syndrome definition at Dictionary.com, a free online dictionary with pronunciation, synonyms and translation. Look ... Obstruction of the branches of the aortic arch caused by thrombosis.. .css-grsm00{display:none;position:absolute;bottom:0px; ... aortic arch syndrome. .css-7w6khc{padding-top:20px;}. .css-1oucjfz{list-style-type:none;line-height:22px;}. *aorist ... aortic arch syndrome. .css-xojh1k{position:relative;height:auto;overflow:hidden;padding-bottom:0px;margin-bottom:0px;}. .css- ...
Aortic arch syndrome. Definition. Aortic arch syndrome refers to a group of signs and symptoms associated with structural ... Aortic arch syndrome problems can be due to trauma, blood clots, or malformations that develop before birth. These defects ... An inflammatory disease called Takayasu syndrome may result in narrowing (stenosis) of the vessels of the aortic arch. This ... Surgery is most often needed to treat the underlying cause of aortic arch syndrome. ...
... and the lateral view suggested the presence of an isodensity double contour at the aortic arch (figure 1B). A CT angiography ... revealed a narrow-necked double aortic arch (DAA) with partial encirclement of the trachea (figure 2 … ...
To the best of our knowledge, a prenatal diagnosis of Holt-Oram syndrome in association with a type B interrupted aortic arch ... Prenatal sonographic diagnosis of familial Holt-Oram syndrome associated with type B interrupted aortic arch ... The foetus had serious bilateral upper limb malformations, a ventricular septal defect and a type B interrupted aortic arch, ... The clinical features of our case completely fulfilled the strict diagnostic criteria for the syndrome. The cardiac ...
... refers to a group of signs and symptoms associated with structural problems in the arteries that branch ... The aortic arch is the top part of the main artery carrying blood away from the heart. ... Aortic arch syndrome Aortic arch syndrome. Diseases and Conditions Subclavian artery occlusive syndrome; Carotid artery ... Results for aortic, aortic arch syndrome, arch, artery, basilar. * You Can Cut Your Odds for an Aortic Aneurysm. April 22, 2019 ...
Interrupted aortic arch is a rare finding in Kabuki syndrome patients5. Interrupted aortic arch (IAA) is a congenital anomaly ... revealed a left aortic arch with normal branching, a type B interruption of the aortic arch (between the left common carotid ... Number 18-11: Late Diagnosis of Interrupted Aortic Arch in a Child with Kabuki Syndrome. Sarmento JA1, 2, Banka P1, Beroukhim ... Number 18-11: Late Diagnosis of Interrupted Aortic Arch in a Child with Kabuki Syndrome ...
We report a case of common variable immunodeficiency syndrome with right aortic arch. An association of right-sided arch and ... Common variable immunodeficiency syndrome syndrome is a clinical entity that should be kept in mind in patients with recurrent ... analysis revealed common variable immunodeficiency syndrome and radiological evaluation confirmed right aortic arch and ... common variable immunodificiency syndrome has not been previously reported. A 41-year-old female patient presented with a ...
... Subclavian artery occlusive syndrome; Carotid artery occlusion syndrome; Subclavian steal syndrome; ... Vertebral-basilar artery occlusive syndrome; Takayasu disease; Pulseless disease. Aortic arch syndrome refers to a group of ... Aortic arch syndrome problems can be due to trauma, blood clots, or malformations that develop before birth. These defects ... An inflammatory disease called Takayasu syndrome may result in narrowing (stenosis) of the vessels of the aortic arch. This ...
Aortic Arch Syndromes. Vasculitis. Vascular Diseases. Cardiovascular Diseases. Aortic Diseases. Skin Diseases, Vascular. Skin ...
Aortic Arch Syndromes. Vasculitis. Vascular Diseases. Cardiovascular Diseases. Aortic Diseases. Skin Diseases, Vascular. Skin ...
Aortic Arch Syndromes. Arthritis, Juvenile. Purpura, Schoenlein-Henoch. Churg-Strauss Syndrome. Polyarteritis Nodosa. Anemia, ... Purpura, Schoenlein-Henoch Graft Versus Host Disease Anemia, Hemolytic, Autoimmune Rheumatoid Arthritis Churg-Strauss Syndrome ... Churg-Strauss syndrome. Schonlein-Henoch purpura. Takayasu arteritis. Wegeners granulomatosis. arthritis & connective tissue ... Vasculitis Definitive diagnosis of 1 of the following forms: Churg-Strauss syndrome Giant cell arteritis Henoch-Schonlein ...
Aortic Arch Syndromes. Churg-Strauss Syndrome. Polyarteritis Nodosa. Vascular Diseases. Cardiovascular Diseases. Autoimmune ...
Aortic Arch Syndromes. Microscopic Polyangiitis. Purpura, Schoenlein-Henoch. Polyarteritis Nodosa. Giant Cell Arteritis. ... Behcet Syndrome. Purpura. Vasculitis. Arteritis. Systemic Vasculitis. Granulomatosis with Polyangiitis. Takayasu Arteritis. ...
Aortic Arch Syndromes. Myositis. Mixed Connective Tissue Disease. Joint Diseases. Musculoskeletal Diseases. Autoimmune Diseases ... European Study Group on Classification Criteria for Sjögrens Syndrome. Classification criteria for Sjögrens syndrome: a ... Sjogrens Syndrome. Spondylarthritis. Arthritis, Juvenile. Arteritis. Dermatomyositis. Collagen Diseases. Systemic Vasculitis. ... EULAR Sjogrens syndrome disease activity index: development of a consensus systemic disease activity index for primary ...
Aortic Arch Syndromes. Churg-Strauss Syndrome. Polyarteritis Nodosa. Vascular Diseases. Cardiovascular Diseases. Lung Diseases ... II.Pulmonary-renal syndrome. Defined as haemoptysis / pulmonary haemorrhage with acute renal impairment. III.Acute renal ... Patients with Churg Strauss syndrome. 1st half of these patients will be assigned to the development cohort and the second half ... Patients with a new presentation of Churg Strauss syndrome. 1st half of these patients will be assigned to the development ...
Aortic Arch Syndromes. Purpura, Schoenlein-Henoch. Churg-Strauss Syndrome. Polyarteritis Nodosa. Purpura. Blood Coagulation ... Behcet Syndrome. Granulomatosis with Polyangiitis. Microscopic Polyangiitis. Vasculitis, Central Nervous System. Vasculitis. ... Churg-Strauss Syndrome (CSS) Granulomatosis With Polyangiitis (GPA) Wegeners Granulomatosis IgA Vasculitis Henoch-Schoenlein ...
Behcet Syndrome. Hepatitis, Autoimmune. Takayasu Arteritis. Aortic Arch Syndromes. Arthritis. Joint Diseases. Musculoskeletal ...
Hypoplastic left heart syndrome. Interrupted aortic arch. Pulmonary atresia (with intact septum) ... These seven defects are hypoplastic left heart syndrome, pulmonary atresia, tetralogy of Fallot, total anomalous pulmonary ...
Hypoplastic Left Heart Syndrome*. *Interrupted Aortic Arch*. *Pulmonary Atresia*. *Single Ventricle*. *Tetralogy of Fallot* ...
Hypoplastic left heart syndrome. *Interrupted aortic arch. *Pulmonary atresia. *Single ventricle. *Tetralogy of Fallot ...
... of a short section of that portion of the aorta that arches over the heart. The aorta is the principal artery conducting blood ... aortic arch syndrome. …must be closed surgically, and coarctation (narrowing) of the aorta, which causes an increased work load ... In infants the artery that supplies the left arm is tied, divided, and used as a flap to repair the narrowed aortic area. In ... In coarctation of the aorta there is a narrowing of the aortic wall, usually at that portion of the aorta just beyond the site ...
  • Occasionally, symptoms relate to intermittent or persistent brain ischaemia, due to a subclavian steal syndrome (SSS), narrowing of other aortic arch vessels or intracerebral vascular disease. (patient.info)
  • The 22q11.2 deletion is the underlying cause of the medical problems associated with DiGeorge syndrome, velocardiofacial syndrome and conotruncal anomaly face syndrome, as well as some of the problems associated with Opitz G/BBB and Cayler cardiofacial syndromes. (chop.edu)
  • When a child has tetrology of fallot does that mean that the digeorge syndrome is present? (medhelp.org)
  • The 22q11 deletion (or DiGeorge) syndrome (22q11DS), the result of a 1.5- to 3-megabase hemizygous deletion on human chromosome 22, results in dramatically increased susceptibility for "diseases of cortical connectivity" thought to arise during development, including schizophrenia and autism. (pnas.org)
  • Researchers from the Walter and Eliza Hall Institute made the discovery while investigating foetal development in an animal model of DiGeorge syndrome. (healthcanal.com)
  • DiGeorge syndrome affects approximately one in 4000 babies. (healthcanal.com)
  • The research team found a protein called MOZ, the 'switch' which is involved in chromatin modification, was a key to explaining the range of defects seen in an animal model of DiGeorge syndrome. (healthcanal.com)
  • We showed that MOZ is crucial for normal activity of Tbx1 , and the level of MOZ activity may contribute to determining how severe the defects are in children with DiGeorge syndrome," Dr Voss said. (healthcanal.com)
  • Dr Voss said the study also showed that the severity of birth defects in DiGeorge syndrome could be compounded by the mother's diet, particularly if the MOZ switch is not working properly. (healthcanal.com)
  • The research team showed that reduced MOZ activity could conspire with excess retinoic acid (a type of vitamin A) to markedly increase the frequency and severity of DiGeorge syndrome. (healthcanal.com)
  • In fact, in mice that had one normal copy of MOZ and one mutated copy, the offspring look completely normal, but if the mother's diet was high in vitamin A, the offspring developed a DiGeorge-like syndrome. (healthcanal.com)
  • This suggests that MOZ, when coupled with a diet high in vitamin A (retinoic acid), may play a role in the development of DiGeorge syndrome in some cases. (healthcanal.com)
  • Introduction The 22q11.2 deletion syndrome (22q11.2DS), also known as velo-cardio-facial syndrome (MIM #192430) and DiGeorge syndrome (MIM #188400), is a fairly common human congenital anomaly disorder occurring in 1:1000 fetuses (1) and 1:4000 live births (2). (deepdyve.com)
  • It is found, in at least one out of four cases, to be associated with a genetic mutation, namely, a deletion of the 22q11 locus, also responsible for the DiGeorge syndrome. (news-medical.net)
  • Tbx1 haploinsufficiency in the DiGeorge syndrome region causes aortic arch defects in mice. (springer.com)
  • Interrupted aortic arch is often associated with DiGeorge syndrome. (wikipedia.org)
  • DiGeorge syndrome, also known as 22q11.2 deletion syndrome, is a syndrome caused by the deletion of a small segment of chromosome 22. (wikipedia.org)
  • DiGeorge syndrome is typically due to the deletion of 30 to 40 genes in the middle of chromosome 22 at a location known as 22q11.2. (wikipedia.org)
  • DiGeorge syndrome occurs in about 1 in 4000 people. (wikipedia.org)
  • The syndrome was first described in 1968 by Angelo DiGeorge. (wikipedia.org)
  • Salient features can be summarized using the mnemonic CATCH-22 to describe 22q11.2DS, with the 22 signifying the chromosomal abnormality is found on the 22nd chromosome, as below: Cardiac abnormality (commonly interrupted aortic arch, truncus arteriosus and tetralogy of Fallot) Abnormal facies Thymic aplasia Cleft palate Hypocalcemia/hypoparathyroidism Some experts support changing the name of both DiGeorge and velocardiofacial syndromes to CATCH-22. (wikipedia.org)
  • Small chromosomal abnormalities also frequently lead to congenital heart disease, and examples include microdeletion of the long arm of chromosome 22 (22q11, DiGeorge syndrome), the long arm of chromosome 1 (1q21), the short arm of chromosome 8 (8p23) and many other, less recurrent regions of the genome, as shown by high resolution genome-wide screening (Array comparative genomic hybridization). (wikipedia.org)
  • Oedema is a common clinical symptom in people with nephrotic syndrome and human albumin has been widely used in the treatment of oedema by increasing vascular volume and this inducing diuresis. (bioportfolio.com)
  • Targetable cellular signaling events mediate vascular pathology in vascular Ehlers-Danlos syndrome. (nih.gov)
  • Ortner syndrome refers to vocal cord paralysis resulting from compression of the left recurrent laryngeal nerve by abnormal mediastinal vascular structures. (nih.gov)
  • Takayasu's arteritis (also known as Takayasu's disease, "aortic arch syndrome," "nonspecific aortoarteritis," and "pulseless disease") is a form of large vessel granulomatous vasculitis with massive intimal fibrosis and vascular narrowing, most commonly affecting often young or middle-age women of Asian descent, though anyone can be affected. (wikipedia.org)
  • This abnormality is the most common congenital vascular anomaly of the aortic arch, occurring in approximately 1% of individuals. (wikipedia.org)
  • Proximal aortic dissections tend to be more associated with weakening of the vascular wall due to cystic medial degeneration. (wikipedia.org)
  • Foix-Alajouanine syndrome Chronic cerebrospinal venous insufficiency Peripheral vascular disease Caragine LP, Halbach VV, Ng PP, Dowd CF (June 2002). (wikipedia.org)
  • Neurological involvements range from aseptic meningitis to vascular thrombosis such as dural sinus thrombosis and organic brain syndrome manifesting with confusion, seizures, and memory loss. (wikipedia.org)
  • Although genetic investigation of TBX5 mutations was not available in our locality at the time of diagnosis, the geneticists made a clinical diagnosis of familial Holt-Oram syndrome. (hkmj.org)
  • To the best of our knowledge, a prenatal diagnosis of Holt-Oram syndrome in association with a type B interrupted aortic arch has not been reported in the English literature before. (hkmj.org)
  • The diagnosis of common variable immunodeficiency (CVID) syndrome was established with immunocytochemical tests. (biomedcentral.com)
  • Check if a diagnosis of Down syndrome, Trisomy 21 is confirmed or pending. (cdc.gov)
  • This study aims at prospectively enrolling a cohort of 400 incident cases of myelodysplastic syndromes (MDS) at diagnosis, to evaluate the impact of recurrent mutations on overall survival. (bioportfolio.com)
  • 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management of patients with thoracic aortic disease. (springer.com)
  • Measuring the serum free testosterone level rather than the total testosterone level has no advantage in the diagnosis of Kallmann syndrome or idiopathic hypogonadotropic hypogonadism except in very obese individuals. (medscape.com)
  • The diagnosis of CHARGE syndrome is based on a combination of major and minor characteristics. (nih.gov)
  • Median arcuate ligament syndrome is a diagnosis of exclusion. (wikipedia.org)
  • Ortner's syndrome: a radiological diagnosis" (PDF). (wikipedia.org)
  • Diagnosis of Crouzon syndrome usually can occur at birth by assessing the signs and symptoms of the baby. (wikipedia.org)
  • Further analysis, including radiographs, magnetic resonance imaging (MRI) scans, genetic testing, X-rays and CT scans can be used to confirm the diagnosis of Crouzon syndrome. (wikipedia.org)
  • In diagnosis, other causes of abrupt paralysis should be excluded such as cord compression, transverse myelitis (inflammation of the spinal cord) and Guillain-Barré syndrome. (wikipedia.org)
  • In addition to these blood vessels, the aortic arch crosses the left main bronchus. (wikipedia.org)
  • Peripheral chemoreceptors (of the carotid and aortic bodies) are so named because they are sensory extensions of the peripheral nervous system into blood vessels where they detect changes in chemical concentrations. (wikipedia.org)
  • It lies beneath the sternum, rests on the pericardium, and is separated from the aortic arch and great vessels by a layer of fascia. (wikipedia.org)
  • Most frequently this has manifested as a heart team to manage complex valvular heart disease as it pertains to TAVR, but the concept has also been applied to other cardiovascular conditions such as acute aortic syndromes or arrhythmias. (ahajournals.org)
  • The most serious defects include tetralogy of Fallot (TOF), persistent truncus arteriosus (PTA) and interrupted aortic arch (IAA) type B (6-9). (deepdyve.com)
  • Chronic pseudoaneurysm of the aortic arch: a case report. (biomedsearch.com)
  • Orbital disease is the most common manifestation, and may result in proptosis, restrictive ophthalmopathy, chronic orbital pain, and in chronic cases, orbital retraction syndrome and intractable socket pain. (wikipedia.org)
  • Causes of acute adrenal insufficiency are mainly sudden withdrawal of long-term corticosteroid therapy, Waterhouse-Friderichsen syndrome, and stress in people with underlying chronic adrenal insufficiency. (wikipedia.org)
  • In humans, evidence of a frameshift mutation in the bHLH domain of HAND1 has been correlated with hypoplastic left heart syndrome (a serious form of congenital heart disease where the left side of the heart is severely underdeveloped), aiding in the implication that HAND1 expression is a factor to patients suffering from the disease. (wikipedia.org)