Unusual Cardiac Malformations in Conjoined Twins: Thoracopagus Twins with Conjoined Pentalogy of Cantrell and an Omphalopagus Twin with Atretic Ventricles

Byline: R. Spencer (), W.H. Robichaux (), D.W. Superneau (), V.W. Lucas () 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. Author Affiliation: () Associate Professor of Surgery (retired), Louisiana State University School of Medicine, and Clinical Associate Professor of Surgery (retired), Tulane University School of Medicine, New Orleans, LA 70115, USA, US () Department of Pathology and Laboratory Medicine, Tulane University Health Sciences Center, New Orleans, LA 70115, USA, US () Section of Medical Genetics, Ochsner Clinic, New Orleans, LA 70115 USA, US () Pediatric Cardiology, Ochsner Pediatric Heart Institute, New Orleans, LA 70115, USA, US