Abstract
Atresia of the aortic arch is a rare congenital heart defect with a high mortality when associated with other intracardiac defects. Cardiac magnetic resonance (CMR) provides the exact anatomy of the aortic arch and collateral circulation and is useful to diagnose-associated aortic arch anomalies. 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. On clinical exam, the femoral pulses were not palpable and there was a significant differential blood pressure between the upper and lower limbs. The echocardiography showed a severely stenotic bicuspid aortic valve but was limited for the exact description of the aortic arch. 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 patient benefited from a successful surgical commissurotomy of the aortic valve and reconstruction of the aortic arch with a homograft. The post-operative CMR confirmed the good surgical result. This case emphasizes the utility of CMR to provide good anatomical information to establish the exact diagnosis and the operative strategy.
Highlights
Aortic arch obstructive lesions can have a large spectrum of presentation, going from coarctation through atresia or interruption of the aortic arch
CASE REPORT We report the case of a 4-year-old boy referred to our institution for surgical treatment of aortic coarctation and bicuspid aortic valve
In a large series of patients with interruption, 42% of patients were of type A, with the arch interrupted at the aortic isthmus beyond the origin of the left subclavian artery, 53% of type B, with the interrupted segment between the origin of the left common carotid and left subclavian artery, and 4% of type C, when the interruption was between the innominate and the left common carotid artery (1)
Summary
Aortic arch obstructive lesions can have a large spectrum of presentation, going from coarctation through atresia or interruption of the aortic arch. Cardiac magnetic resonance (CMR) provides the exact anatomy of the aortic arch and collateral circulation and is useful to establish the surgical strategy. The echocardiography showed a bicuspid aortic valve (Figure 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 3). The patient benefited from successful surgical commissurotomy of the aortic valve and aortic arch reconstruction with interposition of a pulmonary homograft by extended end-to-end anastomosis under cardiopulmonary bypass, with an uneventful post-operative course. The post-operative CMR 1 month after surgery showed good surgical result with patency of the aortic isthmus (Figure 7), and there was no differential in blood pressure between the upper and lower limbs
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