三尖瓣闭锁的产前诊断：宫内过程和结局。

Prenatal diagnosis of tricuspid atresia: intrauterine course and outcome.

机构信息

Department of Obstetrics and Prenatal Medicine University of Bonn, Bonn, Germany.

出版信息

Ultrasound Obstet Gynecol. 2010 Feb;35(2):183-90. doi: 10.1002/uog.7499.

DOI:10.1002/uog.7499

PMID:20101636

Abstract

OBJECTIVE

To evaluate the intrauterine course and outcome of tricuspid atresia detected in the fetus.

METHODS

This was a retrospective review of all confirmed cases of tricuspid atresia detected prenatally between 1998 and 2006 in three tertiary referral centers in Germany.

RESULTS

Fifty-four cases of tricuspid atresia were detected prenatally during the study period and confirmed postnatally: 28 (51.9%) cases had a concordant ventriculoarterial connection of which 14 also had pulmonary outflow obstruction; 25 (46.3%) cases had a discordant ventriculoarterial connection of which 14 also had aortic outflow obstruction, six had pulmonary outflow tract obstruction and two had other associated intracardiac anomalies; and one (1.9%) had a common arterial trunk. The peak velocity index for veins in the ductus venosus was significantly elevated in 19 of the 37 (51.4%) cases assessed; however, this finding did not correlate with adverse intrauterine outcome. There were associated extracardiac anomalies in 12 cases: five with chromosomal anomalies, two with VACTERL association, one with unilateral renal agenesis, one with hypospadia, one with hydrothorax, one with megacystis and one with agenesis of the ductus venosus. Seventeen of the 54 (31.5%) cases underwent termination of pregnancy, two (3.7%) died in utero, two (3.7%) died in infancy and 33 (61.1%) children survived with a median follow-up of 26 (range, 12-120) months. Prenatal echocardiography correctly anticipated the postnatal course and the need for neonatal intervention in 29/35 (82.9%) continued pregnancies; in the remaining six (17.1%) cases the right outflow tract obstruction had been underestimated.

CONCLUSIONS

Tricuspid atresia and the frequently associated intracardiac anomalies can be diagnosed in the fetus with considerable accuracy. A thorough search for extracardiac malformations should be performed in order to rule out chromosomal anomalies and multiple malformation syndromes. Elevated pulsatility in the ductus venosus does not indicate cardiac failure. The short-term overall survival in continued pregnancies in our study exceeded 89%, with the greatest rate of loss being in the first year of postnatal life.

摘要

目的

评估在胎儿中检测到的三尖瓣闭锁的宫内过程和结局。

方法

这是对 1998 年至 2006 年期间在德国三个三级转诊中心通过产前检查确诊的 54 例三尖瓣闭锁的回顾性分析。

结果

在研究期间，54 例三尖瓣闭锁在产前被检测到，并在产后得到证实：28 例（51.9%）具有一致的心室-动脉连接，其中 14 例也存在流出道梗阻；25 例（46.3%）具有不一致的心室-动脉连接，其中 14 例也存在主动脉流出道梗阻，6 例存在流出道梗阻，2 例存在其他相关的先天性心脏病；1 例（1.9%）具有共同动脉干。在 37 例评估的病例中，19 例（51.4%）静脉导管的峰值速度指数显著升高；然而，这一发现与不良的宫内结局无关。12 例存在心脏外畸形：5 例伴有染色体异常，2 例伴有 VACTERL 关联，1 例伴有单侧肾发育不全，1 例伴有尿道下裂，1 例伴有胸腔积液，1 例伴有巨膀胱，1 例伴有静脉导管发育不全。54 例中的 17 例（31.5%）接受了终止妊娠，2 例（3.7%）在宫内死亡，2 例（3.7%）在婴儿期死亡，33 例（61.1%）儿童存活，中位随访时间为 26（范围，12-120）个月。在 35 例（82.9%）继续妊娠中，产前超声心动图正确预测了产后病程和新生儿干预的需要；在其余 6 例（17.1%）中，右流出道梗阻被低估。