Aortic Arch Syndromes
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Rare Diseases
Heart Defects, Congenital
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)Types of Aortic Arch Syndromes:
1. Turner Syndrome: A genetic disorder that affects females and is caused by a missing X chromosome. This condition can result in short stature, infertility, and heart defects, including aortic arch syndrome.
2. Down Syndrome: A genetic disorder that occurs when there is an extra copy of chromosome 21. This condition can cause a range of symptoms, including heart defects such as aortic arch syndrome.
3. Williams Syndrome: A rare genetic disorder caused by a deletion of genetic material from chromosome 7. This condition is characterized by cardiovascular problems, including aortic arch syndrome.
4. Marfan Syndrome: An inherited disorder that affects the body's connective tissue, including the heart and blood vessels. This condition can cause aortic arch syndrome and other cardiovascular problems.
5. Ehlers-Danlos Syndrome: A group of inherited disorders that affect the body's connective tissue, including the heart and blood vessels. This condition can cause aortic arch syndrome and other cardiovascular problems.
Symptoms of Aortic Arch Syndromes:
1. Chest pain or pressure
2. Shortness of breath
3. Dizziness or fainting
4. Pulse deficiency in the arms or legs
5. Blue discoloration of the skin (cyanosis)
6. Heart murmurs
7. Abnormal heart rhythms
Diagnosis of Aortic Arch Syndromes:
1. Physical examination and medical history
2. Electrocardiogram (ECG)
3. Echocardiography
4. Cardiac catheterization
5. Magnetic resonance imaging (MRI) or computed tomography (CT) scans
Treatment of Aortic Arch Syndromes:
1. Medications to control symptoms such as high blood pressure, heart failure, or abnormal heart rhythms
2. Surgery to repair or replace the aortic arch, including open-heart surgery or minimally invasive procedures
3. Monitoring and follow-up care to manage the condition and prevent complications.
Prognosis for Aortic Arch Syndromes:
The prognosis for aortic arch syndromes varies depending on the underlying cause of the condition, the severity of the symptoms, and the effectiveness of treatment. In general, early diagnosis and appropriate treatment can improve the outlook for individuals with these conditions. However, without proper care, the condition can be life-threatening.
Symptoms of HLHS may include:
1. Blue tint to the skin, lips, and nails (cyanosis)
2. Rapid breathing
3. Fatigue
4. Poor feeding or inability to gain weight
5. Weak or absent pulse in the left arm or leg
Diagnosis of HLHS is typically made prenatally by ultrasound examination, and may also be confirmed after birth by echocardiogram or other diagnostic tests.
Treatment for HLHS usually involves a series of surgeries and catheterizations to repair or replace the affected heart structures. These procedures may include:
1. Shunt procedure: A small tube is placed between the right and left sides of the heart to allow oxygenated blood to flow to the underdeveloped left side.
2. Bidirectional Glenn procedure: A surgical procedure that connects the pulmonary artery to the aortic valve, allowing blood to be pumped to both the lungs and the body.
3. Fontan procedure: A surgical procedure that redirects blood flow from the upper body to the lungs, bypassing the underdeveloped left ventricle.
4. Heart transplantation: In some cases, a heart transplant may be necessary if other procedures are not successful or if there is significant damage to the heart.
Early detection and treatment of HLHS are crucial to prevent complications and improve outcomes. Children with HLHS require close monitoring and frequent medical evaluations throughout their lives to manage any potential issues that may arise. With appropriate treatment, many individuals with HLHS can lead active and productive lives well into adulthood.
Rare diseases can be caused by genetic mutations, infections, allergies, or other factors, and they can affect any part of the body. Some examples of rare diseases include cystic fibrosis, Huntington's disease, sickle cell anemia, and Tay-Sachs disease.
Because rare diseases are so uncommon, they often receive less attention and funding for research and treatment than more common conditions. However, there are organizations and resources available to support individuals with rare diseases and their families. These include patient advocacy groups, research foundations, and specialized healthcare providers.
Some of the key features of rare diseases include:
1. Low prevalence: Rare diseases affect a small percentage of the population, typically less than 1%.
2. Limited understanding: Many rare diseases are not well understood, and their causes and mechanisms are not yet fully understood.
3. Lack of effective treatments: There may be limited or no effective treatments for rare diseases, leading to a significant impact on quality of life.
4. High cost: Treatment for rare diseases can be expensive, and the financial burden can be significant for families and individuals affected.
5. Limited access to care: Due to the rarity of the disease, individuals may have limited access to specialized healthcare providers and resources.
Rare diseases are a significant public health concern, as they affect millions of people worldwide and can have a profound impact on their quality of life. There is a need for increased research, advocacy, and support for individuals with rare diseases and their families.
Types of congenital heart defects include:
1. Ventricular septal defect (VSD): A hole in the wall between the two lower chambers of the heart, allowing abnormal blood flow.
2. Atrial septal defect (ASD): A hole in the wall between the two upper chambers of the heart, also allowing abnormal blood flow.
3. Tetralogy of Fallot: A combination of four heart defects, including VSD, pulmonary stenosis (narrowing of the pulmonary valve), and abnormal development of the infundibulum (a part of the heart that connects the ventricles to the pulmonary artery).
4. Transposition of the great vessels: A condition in which the aorta and/or pulmonary artery are placed in the wrong position, disrupting blood flow.
5. Hypoplastic left heart syndrome (HLHS): A severe defect in which the left side of the heart is underdeveloped, resulting in insufficient blood flow to the body.
6. Pulmonary atresia: A condition in which the pulmonary valve does not form properly, blocking blood flow to the lungs.
7. Truncus arteriosus: A rare defect in which a single artery instead of two (aorta and pulmonary artery) arises from the heart.
8. Double-outlet right ventricle: A condition in which both the aorta and the pulmonary artery arise from the right ventricle instead of the left ventricle.
Causes of congenital heart defects are not fully understood, but genetics, environmental factors, and viral infections during pregnancy may play a role. Diagnosis is typically made through fetal echocardiography or cardiac ultrasound during pregnancy or after birth. Treatment depends on the type and severity of the defect and may include medication, surgery, or heart transplantation. With advances in medical technology and treatment, many children with congenital heart disease can lead active, healthy lives into adulthood.
Interrupted aortic arch
Ortner's syndrome
Aortic arch anomaly - peculiar facies - intellectual disability
Takayasu's arteritis
DiGeorge syndrome
PHACE syndrome
Branchial
Right-sided aortic arch
Adams-Oliver syndrome
Yasui procedure
Double aortic arch
Intra-aortic balloon pump
Hypoplastic right heart syndrome
Aberrant subclavian artery
Circle of Willis
Inflammatory aortic aneurysm
Median arcuate ligament syndrome
Cardiac neural crest
TBX1
Fetal aortic stenosis
List of syndromes
Cyanotic heart defect
Fryns syndrome
LeCompte maneuver
Vocal cord paresis
Tetralogy of Fallot
Coarctation of the aorta
List of ICD-9 codes 740-759: congenital anomalies
Pulse
Prostaglandin E1
List of cardiology mnemonics
Spinal cord
List of circulatory system conditions
Pulmonary atresia with ventricular septal defect
MicroRNA
Bradykinin
Congenital heart defect
List of skin conditions
Cystathionine beta synthase
Brachiocephalic artery
Syncope (medicine)
Lujan-Fryns syndrome
Thymus
Hypoplastic left heart syndrome
Interventional radiology
Subclavian artery
Karl Wilhelm Zimmermann
Trachea
GBX2
Dick Williams
Lars Georg Svensson
Spinal anaesthesia
Systemic vasculitis
Aortic arch syndrome: MedlinePlus Medical Encyclopedia
1.4 Congenital Anomalies - Definitions | CDC
Congenital Heart Defects - Types | NHLBI, NIH
Pediatric Metabolic Acidosis Differential Diagnoses
Hypoplastic left heart syndrome - About the Disease - Genetic and Rare Diseases Information Center
Turner syndrome - About the Disease - Genetic and Rare Diseases Information Center
Austrian Syndrome Associated with Pandemic (H1N1) 2009 in Child - Volume 16, Number 9-September 2010 - Emerging Infectious...
Biomarkers Search
Keipert syndrome - Ontology Browser - Rat Genome Database
Heersink School of Medicine News | UAB
NICHD Turner Syndrome Research Information | NICHD - Eunice Kennedy Shriver National Institute of Child Health and Human...
Cases of Ultrasound-Diagnosed Right Aortic Arch with Right Arterial Duct and the Treatment
MeSH Browser
Michael Ma - Stanford Medicine Children's Health
Numerical Study for Blood Flows in Thoracic Aorta | SpringerLink
Case Based Pediatrics Chapter
Aortic Disease - 2017 Outcomes Report for Cardiovascular Services | NewYork-Presbyterian
Browse Topic: Heart and Vascular | Comprehensive Cancer Center | UC Davis Health
MeSH Browser
DeCS
Pesquisa | Portal Regional da BVS
Frontiers | Identification of Immune-Related Gene Signature in Stanford Type A Aortic Dissection
مقالات بر حسب مجله - IranMedex
Prefix: mid
Cardiology | International and Private Care - GOSH
Archives of Medicine and Surgery
NCIt Code NCIt PT NICHD NRNT PT NICHD NRNT SY NCIt Definition NICHD Definition NCIt Code of NICHD Parent NICHD Parent PT NCIt...
Aorta11
- In HLHS, the heart's left side (including the aorta, aortic valve, left ventricle and mitral valve) is underdeveloped. (nih.gov)
- May include atresia of the aortic or mitral orifice and hypoplasia of the ascending aorta. (nih.gov)
- Research also discovered a new abnormality of the aorta, common in up to half of women with Turner syndrome. (nih.gov)
- The abnormality, called elongated transverse arch of the aorta, appears to put women at risk for aortic complications. (nih.gov)
- As the aorta goes down the right side of the main bronchus, it is referred as right aortic arch (RAA), and the occurrence rate is about 1‰ [ 1 ]. (hindawi.com)
- Involvement of the ascending aorta may cause aortic regurgitation. (who.int)
- Weill Cornell vascular surgeons are using fenestrated and branched endograft technology to offer lessinvasive treatments for patients with thoracoabdominal aortic aneurysms that involve the visceral vessels of the aorta. (nyp.org)
- Stanford type A aortic dissection (ATAAD) is a common life-threatening event in the aorta. (frontiersin.org)
- However, there are situations a transfemoral approach can not be done such as extensive atherosclerotic disease in the aortic arch, iliofemoral occlusive disease, dissection of the thoracic aorta, etc. (sch.ac.kr)
- CT angiography of the lower extremity was done showing Leriche syndrome, an aortoiliac occlusive disease involving the blockage of the abdominal aorta as it transitions into the common iliac arteries ( Fig. 2 ). (sch.ac.kr)
- Imaging studies revealed circumferential wall thickening of arch of aorta, bilateral carotids, left sub clavian, left axillary. (who.int)
Dissection3
- Higher admission rates and in-hospital mortality for acute type A aortic dissection during Influenza season: a single center experience. (uchicago.edu)
- Managing patients with acute type A aortic dissection and mesenteric malperfusion syndrome: A 20-year experience. (uchicago.edu)
- Endovascular Fenestration/Stenting First Followed by Delayed Open Aortic Repair for Acute Type A Aortic Dissection With Malperfusion Syndrome. (uchicago.edu)
Hypoplastic6
- Hypoplastic left heart syndrome (HLHS) is a heart condition present from birth (congenital heart defect). (nih.gov)
- When Do Symptoms of Hypoplastic left heart syndrome Begin? (nih.gov)
- Moreover, congenital diseases such as hypoplastic left heart syndrome constitute an important issue for our society. (springer.com)
- When their son was born with hypoplastic left heart syndrome, the Gomez-Flores family held onto hope and put their trust in UC Davis Children's Hospital. (ucdavis.edu)
- To assess the feasibility of interatrial stenting for left atrial decompression in infants with hypoplastic left heart syndrome treated by a "hybrid-approach", with bilateral surgical banding of the pulmonary arteries and percutaneous stenting of the arterial duct. (cambridge.org)
- In the context of the hybrid approach, definitive decompression of the left atrium can be achieved by stenting the atrial septum in infants with hypoplastic left heart syndrome. (cambridge.org)
Aneurysms5
- In aging societies, cardiovascular conditions such as aortic aneurysms and aortic dissections persist as life-threatening diseases. (springer.com)
- We assumed a blood vessel as a rigid body and applied finite-difference method on a centerline-fitted curvilinear coordinate system, where the centerlines and cross-sections were extracted from patient-specific CT scans of patients with aortic aneurysms. (springer.com)
- Through the collaboration of cardiothoracic surgeons, cardiologists, and cardiovascular radiologists, we are seeking to transform care for ascending aortic aneurysms that will enable preservation of a patient's native valve. (nyp.org)
- At NewYork-Presbyterian, our endovascular specialists, who are experts in catheter and wire-based techniques, obtain the best possible outcomes for patients with aortic aneurysms, complicated Type B dissections, and aortic coarctation. (nyp.org)
- JAK out of the box: myeloproliferative neoplasms--associated JAK2 V617F mutations contribute to aortic aneurysms. (uchicago.edu)
Native aortic valve1
- In pneumococcal endocarditis, the native aortic valve is the most frequent location of vegetation. (cdc.gov)
Aneurysm8
- 15. Ortner's syndrome associated with aortic arch aneurysm. (nih.gov)
- Aneurysm of the aortic arch and recurrent left vocal cord paralysis]. (nih.gov)
- Tim Stottlemyer is one of 13 patients who have received life-saving surgical procedures thanks to vascular surgery's new abdominal aortic aneurysm surveillance program. (ucdavis.edu)
- UC Davis Health and AI software company Illuminate have developed a centralized abdominal aortic aneurysm surveillance program using artificial intelligence software. (ucdavis.edu)
- Aortic Aneurysm" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings) . (uchicago.edu)
- This graph shows the total number of publications written about "Aortic Aneurysm" by people in this website by year, and whether "Aortic Aneurysm" was a major or minor topic of these publications. (uchicago.edu)
- Below are the most recent publications written about "Aortic Aneurysm" by people in Profiles. (uchicago.edu)
- Prophylactic sac outflow vessel embolization is associated with improved sac regression in patients undergoing endovascular aortic aneurysm repair. (uchicago.edu)
Regurgitation1
- positive result, aortic regurgitation and Takayasu arteritis (TA) is a chronic i n f e c t i v e e n d o c a r d i t i s ( I E ) w e r e vasculitis of unknown aetiology involving discovered. (who.int)
Endovascular aortic2
- Many endovascular aortic repairs are performed completely percutaneously with no incisions. (nyp.org)
- Right-sided Upper Extremity Access for Patients Undergoing Parallel Graft Placement during Endovascular Aortic Repair is Not Associated with Increased Neurologic Events When Compared with Left Upper Extremity Access. (uchicago.edu)
Thoracic1
- UC Davis Health is one of 30 health systems in the U.S. to take part in new clinical study of a minimally treatment for thoracic aortic arch disease. (ucdavis.edu)
Called Takayasu syndrome1
- An inflammatory disease called Takayasu syndrome may result in narrowing (stenosis) of the vessels of the aortic arch. (medlineplus.gov)
Disease9
- Heterotaxy syndromes have an incidence of 1 in 10,000 newborn births but account for about 4% of all congenital heart disease (CHD). (medscape.com)
- NewYork-Presbyterian's open heart and endovascular surgery programs for aortic disease continue to grow as international and nationally recognized centers of excellence. (nyp.org)
- These programs bring together the expertise of comprehensive care teams with advanced treatment algorithms that result in excellent outcomes for aortic disease. (nyp.org)
- Additional studies utilize a multidisciplinary approach to analyze and optimize aortic disease surgery in collaboration with cardiac surgeons, cardiologists, radiologists, bioengineers, and basic scientists. (nyp.org)
- C99267 Congenital Malformation Syndrome C99147 Neonatal Research Network Terminology C101040 Total Colonic Aganglionosis Total Colonic Aganglionosis The severe form of Hirschsprung disease, this is characterized by a complete lack of nerve cells in the large intestine, and often a partial lack in the small intestine. (nih.gov)
- CAS for each carotid stenosis was attempted by the usual femoral access, but there was no pulse on either side of the femoral artery, and computed tomography angiogram of the lower extremity revealed Leriche syndrome, which is an aortoiliac occlusive disease. (sch.ac.kr)
- We report a case of symptomatic carotid artery stenosis combined with Leriche syndrome which is an aortoiliac occlusive disease, treated by CAS by transradial approach with good result. (sch.ac.kr)
- It is also called as Pulseless Disease or Aortic Arch Syndrome and usually seen before 40 years with female and male ratio being 10:1. (who.int)
- Although respiratory symptoms are the most prevalent disease manifestation of infection by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2), nearly 20% of hospitalized patients are at risk for thromboembolic events. (bvsalud.org)
Endocarditis1
- One case of Austrian syndrome has been reported in the pediatric age group, in a 7-year-old girl in whom aortic valve endocarditis developed after pneumococcal meningitis infection ( 2 ). (cdc.gov)
Malformations6
- Aortic arch syndrome problems can be due to trauma, blood clots, or malformations that develop before birth. (medlineplus.gov)
- Section research has shown that malformations of cardiac veins are more common than originally thought, occurring in more than 20% of women with Turner syndrome. (nih.gov)
- RAA-ALSA and left arterial duct are the most common vascular ring malformations, whereas right aortic arch accompanied by right arterial duct will not form effective vascular rings or cause compression to the trachea and esophagus due to the reason that both aortic arch and arterial duct are on the right side of the trachea. (hindawi.com)
- As for fetuses diagnosed as right aortic arch and right arterial duct according to prenatal ultrasound, a comprehensive evaluation of the risk of pregnancy is made to decide whether to terminate pregnancy or not based on the analysis of other possible malformations and chromosomal karyotype. (hindawi.com)
- Goldenhar Syndrome is a rare, generally sporadic condition, whose physical manifestations include asymmetric incomplete facial development, epibulbar dermoids, auricular malformations e auricular appendices, vertebral anomalies, central nervous system disturbances, ocular irregularities and visceral abnormalities. (bvsalud.org)
- It is considered a variant of craniofacial (hemifacial) microsomia (CFM), a group of congenital malformations that occur during the embryonic development of the first and second branchial arches, which give rise to the ear, face and eyelids 3-5 . (bvsalud.org)
Valve4
- In 1957, an American internist reported the preference of Streptococcus pneumoniae for the aortic valve and its frequent association with meningitis and pneumonia ( 1 ), an association now known as Austrian syndrome. (cdc.gov)
- The use of a new imaging method - four-dimensional flow MRI and computational fluid dynamics - is being explored to visualize and analyze flow dynamics that will help guide surgery to alleviate stress on the repaired aortic valve. (nyp.org)
- Two major research projects are underway at Weill Cornell investigating the flow dynamics in the aortic root after valve-sparing operations using 4-D MRI, computational dynamics, and sophisticated in vitro simulators. (nyp.org)
- Short description: Aortic valve disorder. (icd9data.com)
Dissections1
- Distinguishing acute from chronic aortic dissections using CT imaging features. (uchicago.edu)
Pulmonary2
- While there is a doubt about the position of the aortic arch and arterial duct we especially observe the connection status of the aortic arch and pulmonary arterial duct, and the relative position of the aortic arch, arterial duct and trachea. (hindawi.com)
- Williams Syndrome: Supravalvar Aortic, Aortic Arch, Coronary and Pulmonary Arteries: Is Comprehensive Repair Advisable and Achievable? (stanfordchildrens.org)
Cardiac4
- Standard ultrasound sections of fetal heart: including upper abdominal transverse section, four-chamber cardiac section, left and right ventricular outflow tract section, the aortic arch section, arterial duct section, three vessel view(3VV), grayscale and color Doppler flow imaging of three vessel and trachea view (3VT) with the fetus echocardiography. (hindawi.com)
- At the same time, we are expanding clinical and translational research efforts in all areas of adult cardiac and vascular surgery, with a major focus on aortic surgery. (nyp.org)
- Omphalocele is frequently (50% of cases or more) associated with additional birth defects (particularly cardiac, urogenital, brain, spina bifida), with certain complex anomaly patterns (pentalogy of Cantrell, OEIS), or with genetic syndromes (e.g. trisomies 13 and 18, Beckwith-Wiedemann syndrome, Donnai-Barrow syndrome). (cdc.gov)
- Surgical removal of the stents was uneventful during reconstruction of the aortic arch and creation of a bidirectional cavopulmonary connection in 4 patients, and during cardiac transplantation in one. (cambridge.org)
Vessels1
- A retrospective analysis of fetal echocardiography characteristics of 10 fetuses who were diagnosed as right aortic arch with right arterial duct from December 2016 to March 2021 is made, and focus is put on the relationship between the aortic arch and arterial duct, and the position of aortic arch, arterial duct arch and trachea on the three vessels and trachea view (3VT). (hindawi.com)
Incompetence1
- hypokinetic, a dilated aortic root and V.G. was a 32 year old man was admitted moderate aortic incompetence. (who.int)
Incidence1
- The incidence of Goldenhar syndrome ranges from 1:3500 to 1:5600 live births, with a gender ratio of 3:2 (male:female), presenting more frequently in children with congenital deafness, at a rate of approximately 1:1000 live births 8-9 . (bvsalud.org)
Centers1
- This international network of highvolume aortic centers utilizes blinded external analysis of data by a dedicated group of experts for outcome analysis. (nyp.org)
Artery3
- The aortic arch is the top part of the main artery carrying blood away from the heart. (medlineplus.gov)
- As a result, all 10 cases with right aortic arch and right arterial duct do not show aberrant left subclavian artery, and aortic arch with arterial duct are still connected as "V-shaped", and do not get vascular rings. (hindawi.com)
- Right aortic arch is generally divided into 2 types: right aortic arch with aberrant left subclavian artery (RAA-ALSA) and right aortic arch with mirror image branching (RAA-MB). (hindawi.com)
Transposition1
- As illustrated in Table 1 , five of the ten instances include foetuses with a straight right aortic arch and a right arterial duct, and one involves a transposition of the major arteries. (hindawi.com)
Neonatal2
- Arch Dis Child Fetal Neonatal Ed . 2014 Feb 19. (medscape.com)
- C99232 Neonatal Respiratory System Disorder C99147 Neonatal Research Network Terminology C101039 Escobar Syndrome Escobar Syndrome A rare congenital disorder, this is the non-lethal variant of multiple pterygium syndrome, characterized by orthopedic and craniofacial abnormalities, pterygium and akinethesia. (nih.gov)
Marfan1
- The common inherited connective tissue disorders discussed herein include osteogenesis imperfecta, Marfan syndrome, and Ehlers-Danlos syndromes. (hawaii.edu)
Anomalies2
- For a more detailed listing of minor anomalies, please refer to Appendix B . Often, surveillance systems will collect information on specific syndromes that are multiple anomalies pathogenetically related due to a single cause - for example, genetic or environmental causes that are known to cause birth defects. (cdc.gov)
- Clinically, the size of the omphalocele correlates with the risk of associated anomalies and syndromes (the larger the omphalocele the higher the risk). (cdc.gov)
Defects4
- Similar to collecting information on internal defects, collecting information on syndromes often requires gathering data from multiple sources such as laboratory, imaging or genetic testing. (cdc.gov)
- Therefore, collecting surveillance data on internal defects and syndromes is typically not recommended when first starting a surveillance programme. (cdc.gov)
- 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. (nih.gov)
- OBJECTIVE: To evaluate the hypothesis that childhood survival for individuals with Down syndrome (DS) and congenital heart defects (CHDs) has improved in recent years, approaching survival of those with DS without CHDs. (cdc.gov)
Including Turner syndrome1
- HLHS has also been reported with certain genetic disorders including Turner syndrome, Jacobsen syndrome, trisomy 13, and trisomy 18. (nih.gov)
Influenza1
- The use of drugs such as thalidomide, retinoic acid, tamoxifen and cocaine during pregnancy have been suggested as risk factors for the development of this syndrome, while gestational diabetes, rubella and influenza have also been proposed as potential etiological factors 12-13 . (bvsalud.org)
Arterial4
- This paper aims to discuss the value of ultrasound to diagnose right aortic arch with right arterial duct. (hindawi.com)
- In conclusion, 3VT can simply and clearly detect the right aortic arch, and the key to diagnosing the right aortic arch and right arterial duct is thorough inspection of the aortic arch, arterial duct, and trachea in their respective positions. (hindawi.com)
- This paper aims to explore the characteristics of prenatal echocardiography of right aortic arch and right arterial duct and the treatment. (hindawi.com)
- Ten babies identified with right aortic arch with right arterial duct using prenatal echocardiography in our institution between December 2016 and March 2021 were selected as study participants. (hindawi.com)
Complications1
- PMID- 5428655 TI - Serious ophthalmological complications in the Ehlers-Danlos syndrome. (nih.gov)
Patients3
- Of these patients, 80% have a right-sided aortic arch. (medscape.com)
- Patients with aortitis syndrome often exhibit retinopathy. (bvsalud.org)
- Management of the aortic arch in patients with Loeys-Dietz syndrome. (uchicago.edu)
Typically not1
- Turner syndrome is typically not inherited, but it can be inherited in rare cases. (nih.gov)
Heart2
- Some people with Turner syndrome have skeletal abnormalities, kidney problems, and/or a congenital heart defect. (nih.gov)
- The location of the aortic arch in fetal heart and its abnormal diagnosis are determined by two experienced physicians. (hindawi.com)
Chronic1
- This syndrome mainly occurs in middle-age men who have predisposing factors, such as chronic alcoholism, altered immune state, dural fistula, and ear or sinus infections. (cdc.gov)
Cardiovascular3
- Turner syndrome affects women throughout their lives, with effects on their growth and development as well as fertility and cardiovascular health. (nih.gov)
- NICHD is at the forefront of research on Turner syndrome and its effects on women's reproductive, cardiovascular, and psychosocial health. (nih.gov)
- A main focus is the cardiovascular effects of Turner syndrome in women. (nih.gov)
Clinical2
- The Institute also is sponsoring a longitudinal study on the genetics of Turner syndrome and was instrumental in developing a clinical practice guideline that outlines lifelong care for women and girls with Turner syndrome. (nih.gov)
- The purpose of this case report was to describe clinical presentation of Goldenhar syndrome in a five-year old male with no significant family history, where almost all of the classic signs of the condition were present. (bvsalud.org)
Bilateral1
- 1 instance has a ventricular septal defect in addition to an aortic saddle, 1 case is complicated by PRUV (Persistent Right Umbilical Vein), 1 case has lateral ventricular widening without evidence of gallbladder, and 1 case has bilateral hydronephrosis. (hindawi.com)
Physicians2
- Narrowing of aortic arch, infant's otherwise good health prompt physicians to move Baby JJ's Glenn procedure up one month. (uab.edu)
- Drawing on an extensive aortic surgery database, our physicians are addressing aortic pathologies and procedures using complex statistical analyses with a goal toward durable outcomes. (nyp.org)
Chromosomal2
- Turner syndrome is a chromosomal disorder that affects development in females. (nih.gov)
- However, while some chromosomal studies have not revealed any specific genetic abnormalities, it has been suggested that the 22q 11 locus contains important genes that may influence regulatory signaling events in the development of the pharyngeal arch, which could potentially contribute to craniofacial dysmorphism 11 . (bvsalud.org)
Multidisciplinary1
- NICHD led the Turner Syndrome Consensus Study Group in hosting an international, multidisciplinary meeting on Turner syndrome in 2006. (nih.gov)
Surgery1
- Surgery is often needed to treat the underlying cause of aortic arch syndrome. (medlineplus.gov)
Occurs1
- Subsequently, secondary flow occurs on the cross-section and forms a set of twin vortices called Dean's vortices, thereby playing an important role in blood flow through the aortic arch where a strong curvature exists. (springer.com)
Manifestation1
- To the best of our knowledge, this is the first report mentioning the prenatal manifestation of cardiospondylocarpofacial syndrome. (bvsalud.org)
Left2
- As for a healthy person, the aortic arch is on the left of the trachea. (hindawi.com)
- The 2 primary subtypes of situs ambiguous include (1) right isomerism, or asplenia syndrome, and (2) left isomerism, or polysplenia syndrome. (medscape.com)
Newborn1
- Institute research is aimed at understanding the impact of Turner syndrome during various stages of a woman's life, including as a newborn, child, adolescent, and a woman of childbearing age. (nih.gov)