联体双胎中的罕见心脏畸形：胸腹联体双胎合并Cantrell五联症，以及脐联体双胎合并心室闭锁

Unusual cardiac malformations in conjoined twins: thoracopagus twins with conjoined pentalogy of Cantrell and an omphalopagus twin with atretic ventricles.

作者信息

Spencer R, Robichaux W H, Superneau D W, Lucas V W

机构信息

Louisiana State University School of Medicine, New Orleans, LA 70115, USA.

出版信息

Pediatr Cardiol. 2002 Nov-Dec;23(6):631-8. doi: 10.1007/s00246-002-0162-z.

DOI:10.1007/s00246-002-0162-z

PMID:12530497

Abstract

Two unrelated cases of conjoined twins were found to have cardiac malformations that apparently have not been reported previously. In one case, thoracopagus twins had an extensive thoracoabdominal wall defect that resulted in ectopia cordis of a conjoined heart along with evisceration of the shared liver and intestine along with one spleen. These malformations, accompanied by defects in the sternum, diaphragm, and supraumbilical abdominal wall, constitute a conjoined pentalogy of Cantrell. In the second case, the heart of one of omphalopagus twins consisted of a solid ventricular mass with only a minute aortic cavity but no atrioventricular communication-an ineffective heart that could develop only in a conjoined or chorioangiopagus twin. In both cases, a common atrium lay in the primitive (embryologic) position caudal to the ventricles.

摘要

发现两例不相关的联体双胎病例存在心脏畸形，这些畸形显然此前未曾有过报道。在其中一例中，胸部联体双胎有广泛的胸腹壁缺损，导致联体心脏的心外膜异位，同时共享的肝脏、肠道以及一个脾脏脏器外翻。这些畸形伴有胸骨、膈肌和脐上腹壁的缺损，构成了坎特雷尔联体五联症。在第二例中，脐部联体双胎之一的心脏由一个实性心室团块组成，仅有一个微小的主动脉腔，但没有房室通道——这是一个无效心脏，仅能在联体或绒毛膜血管联体双胎中发育。在这两例中，共同心房位于心室尾侧的原始（胚胎学）位置。