DOI: 10.1097/md.0000000000041009 ISSN: 1536-5964

Berry syndrome, a rare congenital cardiac structural abnormality with 1-stage surgical repair: A case report

Yueqiu Su, Zhou Leng

Rationale:

Berry syndrome is a complex congenital heart anomaly characterized by a combination of aortopulmonary window, interrupted aortic arch or hypoplastic aortic arch or coarctation of the aorta, anomalous origin of the right pulmonary artery, patent ductus arteriosus, and intact ventricular septum. It is an extremely rare condition, with approximately 100 reported cases to date.

Patient concerns:

In this article, we report a case of a 6-year-old girl with Berry syndrome who presented with significant ischemic and hypoxic symptoms.

Diagnoses:

Based on the patient’s medical history and examinations, a definitive diagnosis of Berry syndrome was established.

Interventions:

She underwent a 1-stage surgical treatment and had a successful discharge. The surgical process was aimed to preserve the growth potential of the aorta and pulmonary arteries while achieving unobstructed left ventricular outflow and continuity of the aortic arch, ensuring no shunting between the main pulmonary arteries.

Outcomes:

Intraoperative transesophageal echocardiography confirmed continuous flow through the aortic arch and no shunting between the MPA and the aorta.

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