I nterrupted aortic arch (IAA) is a rare congenital abnormality (incidence rate, 3 per million live births per annum). Loss of luminal continuity between the ascending and descending portions of the aorta is the main pathologic condition.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.2 On the basis of the site of the lesion, 3 types of disease have been reported. 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). 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.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. 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.
Case ReportIn August 2014, a 76-year-old woman was referred to our department for dyspnea on exertion (New York Heart Association functional class II), which had begun one month before referral. No other relevant information was found in her medical history. Upon physical examination, her peripheral pulses were palpable symmetrically, over the carotid arteries and in the upper limbs. We heard a machine-like murmur over the right scapula and a soft systolic murmur over the left sternal border space. Lower-limb pulses were not palpable. Chest radiographs revealed no pathologic abnormalities. An electrocardiogram showed sinus rhythm and right bundle branch block.Transthoracic echocardiography revealed a normal LV ejection fraction (0.55), severe right ventricular dysfunction, BAV, mild aortic valve regurgitation, and severe tricuspid valve regurgitation (pulmonary artery pressure, 40 mmHg). Coronary angiography was performed via a right radial artery approach. The left anterior descending artery and the right coronary artery had significant stenoses. Aortography showed enlargement of the ascending aorta, BAV, normal aortic arch dimensions, and occlusion of the aorta immediately distal to the origin of the left subclavian artery (Fig. 1). Computed tomographic (CT) angiographic findings were compatible