Atresia of the Aortic Arch in 4-Year-Old Child: A Clinical Case Study

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.

Congenital anomalies of the aortic arch

“…This fibrous remnant is not visualized on imaging as there is no patent lumen. Nevertheless, the presence of diverticulum on both sides of the distant aortic segments may point towards presence of arch atresia (73).…”

A Case of Postnatal Aortic Arch Atresia

“…The persistence of fibrous junctions limits the length of the gap between the atretic segments. According to a case reported by Stimato et al, 15 as shown by gadolinium-enhanced magnetic resonance angiography, a patient with aortic atresia had diverticula on both sides of the interrupted zone. This indicates that there were fiber bundles in this area, which is consistent with the diagnostic criteria for AAA.…”

Ascending aortic obstruction with hypoplastic innominate artery

“…Although obstructive aortic disease is uncommon, the first-year mortality of aortic interruption or atresia is relatively high if untreated; [1] Hence, the precise identification and comprehensive assessment of the aortic disease are of importance for the timely intervention and the surgical planning, and in particular presentations of obstructive aortic disease vary by the types, locations and degree [2]. One of the phenotypes is ascending aortic hypoplasia, a lesion involving some or all segments of the ascending aorta, which is always combined with other cardiac anomaly, including ventricular septal defect, patent arterial duct or hypoplastic left ventricle.…”