Berry syndrome: a case report and literature review

Background Berry syndrome, a rare combination of cardiac anomalies, consists of aortopulmonary window (APW); aortic origin of the right pulmonary artery; interrupted aortic arch (IAA) or hypoplastic aortic arch or coarctation of the aorta; and an intact ventricular septum. There is lack of review articles that elucidate the clinical features, diagnosis, treatment, and outcomes of Berry syndrome. This publication systematically reviews the 89 cases published since 1982 on Berry syndrome. Case presentation A 38-year-old woman presented with a loud murmur and cyanosis. Transthoracic echocardiography demonstrated a severely dilated aorta and main pulmonary artery with a large intervening defect. Distal to the APW, the ascending aorta gave rise to the right pulmonary artery. Additionally, a type A IAA, an intact ventricular septum, and a large patent ductus arteriosus were revealed. Computed tomography angiography with 3-dimensional reconstruction confirmed above findings. This is the first report of a patient of this age with Berry syndrome who did not undergo surgery. Conclusions Berry syndrome is a rare but well-identified and surgically correctable anomaly. Patients with Berry syndrome should be followed up for longer periods to better characterize long-term outcomes.

A rare complication of myocardial ischaemia following single-stage repair in a case of Berry syndrome

Berry syndrome is a rare congenital cardiac lesion consisting of a distal aortopulmonary window, the aortic origin of the right pulmonary artery (PA), intact ventricular septum and an interrupted or hypoplastic aortic arch. Different repair techniques have been described in the literature. We report a case of Berry syndrome, in whom myocardial ischaemia developed following direct implantation of the right PA to the main PA, which was resolved using an interposition tube graft.