Successful atrial septal defect transcatheter closure in a patient with pentalogy of Cantrell and ectopia cordis.

Abstract

Pentalogy of Cantrell (PC) is an extremely rare multiple congenital anomaly syndrome, characterized by the presence of five major malformations: midline supraumbilical abdominal wall defect (which results in omphalocele), lower sternal defect, diaphragmatic pericardial defect, anterior diaphragmatic defect and various intracardiac malformations (mostly ventricular septal defect) [1]. Additionally, although not part of the classic PC, some severe cases have been associated with herniation of the heart through the diaphragmatic defect, resulting in ectopia cordis (EC) [2]. Such a diagnosis carries a disastrous prognosis without surgical correction or palliation [3]. Furthermore, miscellaneous intracardiac abnormalities even worsen the survival. Multiple chest interventions and complex anatomy impede their surgical treatment; therefore, the transcatheter approach may be the only possibility. According to our best knowledge, no percutaneous atrial septal defect (ASD) closure has been reported in a patient with PC.