![]() 6 In adults, the clinical presentation of IAA varies from absence of symptoms to headache, malaise, hypertension, claudication, differential blood pressure between the arms and legs, limb swelling, and congestive heart failure. The initial defect, one supposes, is coarctation of the aorta, which progresses to complete obstruction of the lumen (especially when the interruption is at the aortic isthmus). 4, 5 The presence of collateral circulation is the only means of survival ( Fig. If the condition is left untreated, 90% of the affected infants die in the first year of life. Severe congestive heart failure is the major presentation of IAA in infants. 2, 3 In this report, we describe the case of a 76-year-old woman with asymptomatic IAA, severe tricuspid regurgitation (TR), and BAV, a perhaps unique combination of pathologic conditions that no one, to our knowledge, has reported before. 3 Because of the high mortality rate-75% by 10 days and 90% at 12 months of life (without surgical correction in infancy)-IAA is very rare among adults. In type B, the lesion is distal to the origin of the left common carotid artery and in type C, the interruption is between the common carotid arteries. In type A, arch interruption occurs distal to the origin of the left subclavian artery (this is also known as interruption at the aortic isthmus). 2 On the basis of the site of the lesion, 3 types of disease have been reported. 1 Some cardiac malformations-including patent ductus arteriosus, ventricular septal defect, bicuspid aortic valve (BAV), left ventricular (LV) outflow tract obstruction, and aortopulmonary window-have customarily been associated with IAA. Loss of luminal continuity between the ascending and descending portions of the aorta is the main pathologic condition. Interrupted aortic arch (IAA) is a rare congenital abnormality (incidence rate, 3 per million live births per annum). ![]()
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