![]() It is often associated with other cardiac defects, particularly persistent patency of the arterial duct, ventricular septal defect, and sub-aortic or aortic valvar stenosis. There was no patent ductus arteriosus (PDA), no associated endofibroelastosis and the descending aorta was fed exclusively by massive systemic collateral circulation (Figures (Figures4 4– 6).Ītresia of the aortic arch is a rare congenital anomaly. There was a bend in the arch and diverticulum on either side of the zone of discontinuity, making the diagnosis of atresia rather than interruption of the aortic arch. CMR with angiography showed absence of continuity between the ascending aorta distal to the left subclavian artery (Type 1) and the descending aorta, extending over 5 mm. The echocardiography showed a bicuspid aortic valve (Figure (Figure2) 2) with severe stenosis (peak systolic gradient of 60 mmHg and mean of 30 mmHg), left ventricular hypertrophy ( Z-score +3.3), preserved systolic and diastolic function (mitral E/A ratio 1.6 mitral A deceleration time 0.10 s, E/E′ 22 secondary to increased left heart filling pressure), and tortuous aortic arch with narrowing and acceleration of flow at the aortic isthmus (Figure (Figure3). The chest radiography (Figure (Figure1) 1) showed mild cardiomegaly (ICT 0.64) and an absent aortic knob, compatible with the diagnosis of coarctation. The electrocardiogram showed sinus rhythm with signs of left auricular hypertrophy, but no ventricular hypertrophy. Normal values at 4 years SBP 102–115 mmHg DBP 62–71 mmHg. The differential of blood pressure is maximum 15 mmHg for systolic blood pressure (SBP) and 10 mmHg for diastolic blood pressure (DBP). This case emphasizes the utility of CMR to provide good anatomical information to establish the exact diagnosis and the operative strategy. The post-operative CMR confirmed the good surgical result. The patient benefited from a successful surgical commissurotomy of the aortic valve and reconstruction of the aortic arch with a homograft. CMR showed absence of lumen continuity between the ascending and descending aorta distal to the left subclavian artery, extending over 5 mm, with the presence of a bend in the arch and diverticulum on either side of the zone of discontinuity, suggesting the diagnosis atresia of the aortic arch rather than coarctation or interruption. The echocardiography showed a severely stenotic bicuspid aortic valve but was limited for the exact description of the aortic arch. On clinical exam, the femoral pulses were not palpable and there was a significant differential blood pressure between the upper and lower limbs. This report describes the case of a 4-year-old child with atresia of the aortic arch, referred to our institution with the diagnosis of aortic coarctation and bicuspid aortic valve. Cardiac magnetic resonance (CMR) provides the exact anatomy of the aortic arch and collateral circulation and is useful to diagnose-associated aortic arch anomalies. Atresia of the aortic arch is a rare congenital heart defect with a high mortality when associated with other intracardiac defects.
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