Thomas Collins R, Weinberg Paul M, Gruber Peter J, St John Sutton Martin G
Division of Cardiology, Arkansas Children's Hospital, Little Rock, AR 72202, USA.
Pediatr Cardiol. 2012 Feb;33(2):252-7. doi: 10.1007/s00246-011-0125-3. Epub 2011 Sep 30.
This study aimed to identify the anatomic and pathologic structural cardiac abnormalities in conjoined twins and to focus on those that have prevented the successful separation of conjoined hearts. A retrospective review was undertaken to examine consecutive cases of thoracopagus conjoined twins with conjoined hearts evaluated at The Children's Hospital of Philadelphia from 1 January 1980 through 6 October 2008. The records included autopsy and surgical findings as well as clinical reports. The study group included nine sets of conjoined twins with a mean gestational age at birth of 33.8 ± 5.5 weeks. Three twin pairs were stillborn. Five twin pairs died afterward. One pair died of cardiopulmonary failure. The median age at death was 22 days (range, 0-345 days). Major congenital heart disease was present in 94.4% (17/18) of the hearts, and 72.2% (13/18) of the hearts had single-ventricle physiology. Total anomalous pulmonary venous return occurred in 39% (7/18) of the cases. The clinical outcome for thoracopagus twins with conjoined hearts remains poor because of inability to separate conjoined and single ventricles. Surgical nonintervention and palliative care should be strongly considered for these patients.
本研究旨在确定联体双胎心脏的解剖和病理结构异常，并关注那些阻碍联体心脏成功分离的异常情况。我们进行了一项回顾性研究，以检查1980年1月1日至2008年10月6日期间在费城儿童医院评估的胸腹联体双胎合并联体心脏的连续病例。记录包括尸检和手术结果以及临床报告。研究组包括9对联体双胎，出生时平均胎龄为33.8±5.5周。3对双胎为死产。5对双胎随后死亡。1对死于心肺衰竭。死亡时的中位年龄为22天（范围为0 - 345天）。94.4%（17/18）的心脏存在主要先天性心脏病，72.2%（13/18）的心脏具有单心室生理特征。39%（7/18）的病例发生了完全性肺静脉异位引流。由于无法分离联体心脏和单心室，胸腹联体双胎合并联体心脏的临床结局仍然很差。对于这些患者，应强烈考虑手术不干预和姑息治疗。
