Cardiovascular abnormalities in thoracopagus twins: embryological interpretation and review

Abstract

The cardiovascular abnormalities of two sets of thoracopagus twins with conjoined heart and liver are described and compared with 27 well documented cases. An embryological interpretation of the cardiovascular abnormalities is suggested. The common heart in both sets showed a common atrium and two ventricles. In case 1 the great arteries were L. malposed in twin A. In case 2 the great arteries originated from their respective double outlet single ventricle. The systemic and pulmonary veins drained directly into the common atrium in case 1 and indirectly via systemic veins in case 2. The type of cardiovascular abnormalities are complex and discordent from one set to another and in the same set. However among 27 published cases of thoracopagus twins, cardiac union, including atrial union with separate ventricles, or atrial and ventricular union, was encountered in 16 cases. Approximately 90% of them are not suitable for surgical separation because of the high degree of cardiac union and the complexity of cardiovascular abnormalities. Surgical separation could be attempted in only two cases, but at the cost of the life of one of the twins.