Ectopia cordis and other midline defects

Background. Thoracic ectopia cordis and other midline defects are rare cong enital anomalies that often occur with other intracardiac defects. Despite significant improvements in neonatal and infant cardiac surgeries, operatio ns for thoracoabdominal ectopia cordis carry an extremely high mortality wi th only a few reported survivors of thoracic ectopia cordis. Methods. The clinical charts of 4 patients with ectopia cordis over a 6-yea r period were reviewed. Three of the patients showed varying degrees of Can trell's Pentalogy; thoracic ectopia cordis was found in 1. We have reviewed our surgical strategies and reported the patients' clinical outcomes. Results. All 4 patients are alive at follow-up. Two infants with double-out let right ventricle have been fully corrected, and extracorporeal membrane oxygenation was necessary in 1 infant for cardiac failure following the car diac repair. A newborn with thoracoabdominal ectopia cordis underwent prima ry repair of his diaphragmatic defect, and a silo was used to progressively reduce the omphalocele. He is currently awaiting elective repair of tetral ogy of Fallot. Lastly, the patient with thoracic ectopia cordis underwent s uccessful soft tissue coverage, and she is being followed in the clinic wit h restrictive muscular ventricular septal defects and a left ventricular di verticulum. Conclusions. Our experience along with other reports in the literature demo nstrates that patients with thoracic and thoracoabdominal ectopia cordis ca n undergo and survive full cardiac, neurologic, and abdominal repair during infancy. Furthermore we advocate different approaches determined by the se verity of the presentation and the presence of other complicating factors. (Ann Thorac Surg 2000;70:111-4) (C) 2000 by The Society of Thoracic Surgeon s.