Echocardiographic diagnosis and clinical analysis of Berry syndrome in 13 cases

Yang Jing; Ding Wenhong; Mo Ying; Fan Xiangming; Cheng Pei; Jin Mei; Liu Yinglong

doi:10.3760/cma.j.issn.1673-4777.2017.12.008

Abstract：Objective To investigate echocardiographic and clinical features of Berry syndrome.Methods Thirteen cases of Berry syndrome diagnosed by echocardiography and confirmed by operative results or CT or cardiac catheterization from January 2007 to August 2017 in Pediatric Cardiac Center,Beijing Anzhen Hospital,Capital Medical University were retrospectively reviewed.Echocardiographic findings and hemodynamic features of Berry syndrome were analyzed.Results Echocardiographic characteristics of Berry syndrome were summarized as follows:①Left ventricular long-axis view showed right pulmonary artery arising from posterior wall of ascending aorta.②Main artery short-axis view showed a communication between pulmonary artery and aorta;color Doppler showed bidirectional low-speed shunt at aorto-pulmonary septum.③Main artery short-axis view and suprasternal fossae view showed patent ductus arteriosus and right-to-left bidirectional shunt.④Suprasternal fossae sagittal view showed interruption or stenosis of aortic arch.⑤Four-chamber view showed right heart enlargement and right ventricular wall thickening;Doppler showed high-speed reverse flow of tricuspid valve which indicated pulmonary hypertension.Seven cases had surgical operation and 2 of them died during perioperative period;2 cases had conservative medical treatment and 1 of them survived;4 cases gave up treatment and they were lost to follow-up.Conclusions Berry syndrome is a rare severe cardiac malformation that needs early surgical treatment after birth to avoid severe comorbidity and improve survival rate.Early diagnosis and assessment through combined echocardiographic views contribute to the success of operation.