Anesthetic management of abdominal ectopia cordis with complex congenital heart disease: A case report

Ectopia cordis is a rare congenital malformation in which the heart is partially or completely displaced outside the thoracic cavity. Anesthetic management is particularly challenging because of abnormal cardiothoracic anatomy, associated congenital heart disease, and the risk of abrupt hemodynamic deterioration during cardiac manipulation and thoracoabdominal closure. We report the perioperative anesthetic management of a 1-day-old male neonate with abdominal ectopia cordis and complex congenital heart disease. Echocardiography demonstrated a functional single ventricle, a common atrioventricular valve, and a large atrial septal defect. Computed tomography confirmed exteriorization of the heart through a major anterior thoracoabdominal wall defect with absent parietal pericardium. General anesthesia was induced with propofol, fentanyl, and rocuronium and maintained with sevoflurane. During patch placement, bleeding was accompanied by a decrease in end-tidal carbon dioxide and arterial hypotension, requiring packed red blood cell transfusion and norepinephrine infusion. During attempted cardiac repositioning, cardiac arrest occurred, followed by return of spontaneous circulation (ROSC) after approximately 20 s of cardiopulmonary resuscitation. The surgical procedure was completed, but the neonate died 6 h postoperatively. This case highlights the extreme hemodynamic vulnerability associated with ectopia cordis and complex congenital heart disease. Cardiac repositioning and thoracoabdominal closure may critically compromise venous return and cardiac output, emphasizing the need for meticulous hemodynamic monitoring, anticipation of cardiovascular instability, and close anesthetic-surgical coordination.