2017 年第 12 期 第 12 卷

Berry综合征13例超声心动图诊断及临床分析

Echocardiographic diagnosis and clinical analysis of Berry syndrome in 13 cases

作者：杨静丁文虹莫莹范祥明程沛金梅刘迎龙

英文作者：Yang Jing Ding Wenhong Mo Ying Fan Xiangming Cheng Pei Jin Mei Liu Yinglong

单位：100029首都医科大学附属北京安贞医院小儿心脏中心北京市心肺血管疾病研究所

英文单位：Pediatric Cardiac Center Beijing Anzhen Hospital Capital Medical University Beijing Institute of Heart Lung and Blood Vessel Diseases Beijing 100029 China

关键词：Berry综合征；超声诊断；生存率

英文关键词：Berrysyndrome；Ultrasonicdiagnosis；Survivalrate

• 摘要：

• 目的    总结Berry综合征的超声心动图特征，以提高临床诊断准确率。方法    回顾性分析2007年1月至2017年8月于首都医科大学附属北京安贞医院小儿心脏中心经超声心动图诊断并经手术或CT、心导管检查证实的13例Berry综合征患儿心脏超声表现及血流动力学特点，总结Berry综合征的超声诊断特点。结果    Berry综合征超声心动图特征：①左心室长轴显示右肺动脉起自升主动脉后壁；②大动脉短轴显示主、肺动脉间隔回声缺失，彩色多普勒显示主、肺动脉间隔双向低速分流信号；③大动脉短轴及胸骨上窝切面显示动脉导管未闭及大动脉水平右向左或双向分流信号；④胸骨上窝矢状切面显示主动脉弓离断或缩窄；⑤四腔心切面示右心增大，右心室壁增厚；多普勒测及三尖瓣高速反流，提示肺动脉高压。13例患儿中7例行外科手术（围术期死亡2例），2例行内科治疗（死亡1例、存活1例），4例放弃治疗失访。结论    Berry综合征是罕见的严重心脏畸形，出生后应尽早手术，以提高生存率。掌握其解剖构成，正确运用超声切面早期诊断并评估手术条件是保证手术成功的要素。

• 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.