Song T B, Lee J Y, Kim Y H, Oh B S, Kim E K
Department of Obstetrics and Gynecology, School of Medicine, Chonnam National University, Kwangju, Korea.
J Obstet Gynaecol Res. 2000 Jun;26(3):223-6. doi: 10.1111/j.1447-0756.2000.tb01315.x.
Pulmonary atresia with intact ventricular septum is an uncommon congenital cardiac anomaly which very often present varying degrees of downward displacement and dysplasia of the tricuspid valve. A 23-year-old woman was referred at 23 weeks' gestation because of fetal cardiomegaly detected by routine ultrasonography. Ebstein's anomaly with pulmonary atresia was diagnosed by fetal echocardiography. On pulsed Doppler examination, a severe tricuspid regurgitation was detected. Pulmonary atresia was suspected, as forward flow was not detected into pulmonary artery. The fetus was terminated at 25 weeks. At the postmorterm examination Ebstein's anomaly with pulmonary atresia and intact ventricular septum were confirmed. The finding of right atrial enlargement on an early sonogram should prompt the search for tricuspid valve abnormalities with tricuspid regurgitation and the presence or development of pulmonary atresia or stenosis. With the information provided, early detection of tricuspid valve disease should be possible so that the option of therapeutic abortion can be offered.
室间隔完整的肺动脉闭锁是一种罕见的先天性心脏畸形，常伴有不同程度的三尖瓣向下移位和发育异常。一名23岁女性在妊娠23周时因常规超声检查发现胎儿心脏增大而前来就诊。胎儿超声心动图诊断为埃布斯坦畸形合并肺动脉闭锁。脉冲多普勒检查发现严重的三尖瓣反流。由于未检测到肺动脉的前向血流，怀疑有肺动脉闭锁。该胎儿在25周时终止妊娠。尸检证实为埃布斯坦畸形合并肺动脉闭锁且室间隔完整。早期超声检查发现右心房增大应促使医生寻找伴有三尖瓣反流的三尖瓣异常以及肺动脉闭锁或狭窄的存在或发展情况。根据所提供的信息，应能够早期发现三尖瓣疾病，从而可以提供治疗性流产的选择。
