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BMC Cardiovascular Disorders
Published in: BMC Cardiovascular Disorders 1/2021

Open Access 01-12-2021 | Patent Ductus Arteriosus | Case report

Berry syndrome: a case report and literature review

Authors: Wen-jing Bi, Yang-jie Xiao, Yue-jia Liu, Yang Hou, Wei-dong Ren

Published in: BMC Cardiovascular Disorders | Issue 1/2021

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Abstract

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.
Appendix
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Metadata
Title
Berry syndrome: a case report and literature review
Authors
Wen-jing Bi
Yang-jie Xiao
Yue-jia Liu
Yang Hou
Wei-dong Ren
Publication date
01-12-2021

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