先天性三尖瓣口无功能的超声心动图诊断

Diagnosis of congenitally unguarded tricuspid valve orifice by echocardiography

目的分析先天性三尖瓣口无功能（congenitally unguarded tricuspid valve orifice,CUTVO）的超声心动图特征，评价超声心动图对CUTVO的诊断价值。方法回顾性分析9例经心外科手术或尸解诊断为CUTVO患儿的超声心动图资料。结果CUTVO声像图特征主要表现为三尖瓣叶完全或部分缺如，三尖瓣腱索及乳头肌缺如或发育不良，三尖瓣环及房室连接关系正常；三尖瓣口严重反流，右心房、右心室明显增大。本组单纯性CUTVO2例，合并其他心血管系统畸形7例，其中肺动脉闭锁伴室间隔完整5例，房间隔缺损及动脉导管未闭1例，肌部室间隔缺损1例。9例患儿中，超声诊断符合7例，误诊2例（22．2％），其中1例误诊为三尖瓣下移畸形，1例误诊为Uhl’S病。结论超声心动图技术可以准确诊断CUTV0，但需与三尖瓣下移畸形及Uhl’S病鉴别。

Objective To review the imaging characteristics and evaluate the diagnostic value of echocardiography for congenitally unguarded tricuspid valve orifice （CUTVO）. Methods Doppler echocardiography was performed and these images were compared with operative and necropsy findings in 9 children with CUTVO. Results CUTVO was characterized by the absence of the tricuspid valvar leaflets with normal atrioventricular junction and tricuspid annulus, severe tricuspid regurgitation, and dilated right atrium and right ventricles. The associated malformations included pulmonary atresia with intact ventricular septum （5 cases）, patent ductus arteriosus and atrial septal defect （1 case）, and muscular ventricular septal defect （1 case）. Seven of 9 cases were diagnosed correctly, and other 2 cases were miss-diagnosed, in which 1 case misdiagnosed as Ebstein anomaly, and 1 case misdiagnosed as Uhl＇s disease. Conclusions Congenitally unguarded tricuspid valve orifice can be diagnosed accurately by Doppler echocardiography. This malformation should be distinguished from Ebstein anomaly and Uhl＇s disease.