Successful staged-Fontan operation in a patient with ectopia cordis

Ectopia cordis is a very rare and often fatal disease. We report a successful surgery case of thoracoabdominal ectopia cordis with univentricular heart. This patient underwent a three-stage Fontan procedure, a right-modified Blalock-Taussig shunt at the age of 1 month, bidirectional Glenn shunt and pulmonary arterioplasty at 2 years 8 months, and finally a total cavopulmonary connection at 4 years. This patient was discharged from the hospital in good condition and has been doing well since. Thus, ectopia cordis is not a contraindication for a Fontan operation.