Ectopia cordis and other midline defects

Thoracic ectopia cordis and other midline defects are rare congenital anomalies that often occur with other intracardiac defects. Despite significant improvements in neonatal and infant cardiac surgeries, operations for thoracoabdominal ectopia cordis carry an extremely high mortality with only a few reported survivors of thoracic ectopia cordis.

The clinical charts of 4 patients with ectopia cordis over a 6-year period were reviewed. Three of the patients showed varying degrees of Cantrell's Pentalogy; thoracic ectopia cordis was found in 1. We have reviewed our surgical strategies and reported the patients' clinical outcomes.

All 4 patients are alive at follow-up. Two infants with double-outlet right ventricle have been fully corrected, and extracorporeal membrane oxygenation was necessary in 1 infant for cardiac failure following the cardiac repair. A newborn with thoracoabdominal ectopia cordis underwent primary repair of his diaphragmatic defect, and a silo was used to progressively reduce the omphalocele. He is currently awaiting elective repair of tetralogy of Fallot. Lastly, the patient with thoracic ectopia cordis underwent successful soft tissue coverage, and she is being followed in the clinic with restrictive muscular ventricular septal defects and a left ventricular diverticulum.

Our experience along with other reports in the literature demonstrates that patients with thoracic and thoracoabdominal ectopia cordis can undergo and survive full cardiac, neurologic, and abdominal repair during infancy. Furthermore we advocate different approaches determined by the severity of the presentation and the presence of other complicating factors.