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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Interrupted aortic arch is a duct-dependent heart disease, and this is why the use of prostaglandins E, which keep the ductus arteriosus open to feed the descending aorta, has transformed its formerly sombre prognosis. The authors report the cases of 9 neonates and infants presenting with interrupted aortic arch associated with ventricular septal defect who underwent total repair of the malformations under extracorporeal circulation. Five children were less than 10 years' old. The aortic lesions were of type A in 2 cases (interruption distal to the left subclavian artery) and of type B in 7 cases (interruption between the left carotid and subclavian arteries). In the second group the lesions were of type B1 in 5 cases (without retro-oesophageal right subclavian artery) and of type B2 in 2 cases (right retro-oesophageal artery distal to the interruption). There was one death (11 p. 100). So far, no child has been reoperated upon for residual aortic obstruction or reopened ventricular septal defect. This technique of total repair in one stage, with closure of the ventricular septal defect, seems to be preferable to the two-stage technique in which stage one includes, in addition to aortic arch repair, cerclage of the pulmonary artery, a procedure with numerous disadvantages and a higher overall mortality rate.
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PMID:[Complete repair of the interrupted aortic arch by an anterior approach. Apropos of 9 cases]. 313 22

Interrupted aortic arch (IAA) is an uncommon congenital anomaly representing approximately 1% of congenital heart disease. More than 97% of the cases also have associated cardiac anomalies complicating their treatment. Because the median age at death in untreated cases is 10 days, this condition usually occurs as a complicated neonatal surgical emergency. There are three types of IAA: Type A is interrupted distal to the left subclavian artery. Type B, which is the most common form, is interrupted between the left common carotid and the left subclavian arteries. Type C, which is the most uncommon type, is interrupted between the innominate and left common carotid arteries. Since the first patient with this congenital anomaly was treated at our institution in 1965, 39 patients have undergone surgical treatment. Two of these (4.6%) had no associated intracardiac lesions, and both survived bypass grafting without complications. There were 12 Type A (30%), 26 Type B (67%) and one Type C (3%). A variety of individualized techniques were used to repair the defects. The three basic techniques were (1) direct aortic to aortic anastomosis bridging the gap, (2) "turndown" or "turnup" of one of the arch vessels to the aorta across the gap, or (3) bypass of the interruption with graft material. Because aortic arch and associated cardiac anomalies represent a neonatal surgical emergency, our therapeutic plan consists of treatment for biventricular failure and PGE(1) infusion to maintain ductal patency, and surgical correction as soon as feasible.
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PMID:Interrupted aortic arch: brief review and summary of an eighteen-year experience. 1522 58

Interrupted aortic arch is the complete loss of luminal continuity between the ascending aorta and the descending aorta and accounts for 1% of all congenital heart disease. Of the affected patients, 75% die within the first month of life and about 90% within the first year if not surgically treated. We report a case of an echocardiographic diagnosis of a type C interrupted aortic arch in an adult who presented with exertional dyspnea and angina after a recent coronary event.
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PMID:Transthoracic echocardiographic diagnosis of a type C interrupted aortic arch in an adult. 1768 5

Interrupted aortic arch (IAA) is characterised by a lack of luminal continuity between the ascending and descending thoracic aorta. It represents a critical ductus dependent congenital heart disease, which without surgery is associated with high mortality in the neonatal period. Management remains challenging, although in recent years overall mortality appears to be improving. Long-term morbidity and need for repeated interventions are areas of concern. It is difficult to make generalised recommendations based on this literature review as complex associated anomalies often require individualised management strategy.
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PMID:Management strategies for interrupted aortic arch with associated anomalies. 1923 79

Interrupted aortic arch is a rare and usually lethal malformation, representing approximately 1% of congenital heart disease. This presents as a missing segment of the aortic arch and is divided into three types: A-called extreme form of coarctation, and is characterized by disruption of aorta's continuity distal to the left subclavian artery (30-40%), B-disruption between the left subclavian and the left carotid arteries (55-60%), and C-the most uncommon type, interruption proximal to the left common carotid artery. The suspicion of coarctation of the aorta can be made from a combination of physical findings including systolic ejection murmur, the murmurs of collateral blood vessels, diminished or absent femoral pulse, and difference in blood pressure between arms and legs. Interrupted aortic arch is an extremely rare anomaly in adult patients. To our knowledge, the world medical literature contains only about 13 reports of interrupted aortic arch diagnosed in adults.
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PMID:Interrupted aortic arch in an adult female. 2105 Feb 64

Interrupted aortic arch is a rare congenital heart disease, whereas right-sided interrupted aortic arch is an extremely rare disorder with few reported cases in the literature. We report our institutional experience with two such patients and review the recent literature.
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PMID:Single institution experience with a right-sided interrupted aortic arch. 2275 34

Interrupted aortic arch anomaly (IAA) characterized by complete luminal dissociation between the ascending and descending aorta and accounting for less than 1% of all cases of congenital heart disease. IAA is a rare condition in infants that occurs approximately three times per million births. It is usually diagnosed and repaired during the neonatal period and is extremely rare in adults. We present the case of an adult man who was diagnosed with IAA accompanied by intracranial aneurysms causing subarachnoid hemorrhage and demonstrate the imaging findings with 256-slice computed tomography angiography and digital subtraction angiography.
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PMID:Type A interrupted aortic arch accompanied by intracranial aneurysms causing subarachnoid hemorrhage in an adult man. 2409 29

Interrupted aortic arch is an extremely rare congenital malformation representing about 1% of congenital heart disease. Early symptoms usually occur early in the neonatal period and clinical deterioration is often rapid and long-term prognosis is limited. Nonetheless, this condition has been identified later in adult life in rare cases. We report a case in an adult male with absence of hypertension history and no further cardiac compromise, with a severe posterior chest pain alongside dyspnea and sweating. Computed tomography angiography revealed interrupted aortic arch type A, bivalve aorta, hemopericardium, aortic dissection Stanford A, and important collateral circulation.
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PMID:Interrupted aortic arch diagnosis by computed tomography angiography and 3-D reconstruction: A case report. 2955 41

Interrupted aortic arch (IAA) is a rare form of critical neonatal heart disease in which there is lack of continuity between the ascending aorta and the descending thoracic aorta. In the absence of prenatal diagnosis, patients with IAA present in shock when the patent ductus arteriosus closes. Diagnosis can generally be made by echocardiography, and initiation of prostaglandin E1 infusion allows for adequate lower body perfusion prior to surgical repair. Full neonatal repair can be achieved with good outcomes in most cases. However, there is often underdevelopment of the left ventricular outflow tract and risk for later surgical re-intervention. Many patients with IAA, particularly those with type B, have DiGeorge syndrome, which has important development implications.
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PMID:Preoperative Physiology, Imaging, and Management of Interrupted Aortic Arch. 2964 38

Interrupted aortic arch (IAA) is an extremely rare congenital cyanotic heart disease characterized by complete disruption between the ascending and descending aorta. A patent ductus arteriosus (PDA) or other collateral pathways provide blood flow to the distal descending aorta. Mortality is extremely high at early infancy, particularly after closure of the ductus arteriosus. Survival and presentation in adulthood are extremely rare. Here, we illustrate a rare case of type B IAA in an adult who presented with secondary polycythaemia. The blood supply to the descending aorta and beyond was almost solely via a PDA. This case demonstrates the value of multimodality imaging, including CT and MRI, for diagnosis and treatment planning in these patients.
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PMID:Type B Interrupted Aortic Arch with a Patent Ductus Arteriosus in an Adult Presenting with Secondary Polycythaemia. 3230 60


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