[Thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis. A case report of successful surgical repair].

A case of thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis is reported. A cyanotic boy with omphalocele was brought to our hospital soon after his birth and was diagnosed as thoracoabdominal ectopia cordis with single ventricle and pulmonary stenosis. A conservative treatment of omphalocele was performed successfully and a plastic chest shield had been made for him which protected the anterior surface of his chest. He continued to develop in a fairly satisfactory condition and operation was delayed until he was 8 years old when he had severe cyanosis and frequent episodes of headache. The operative procedures were; 1) ascending aorta to main pulmonary artery shunt with EPTFE tube; 2) to bring the heart into the thoracic and abdominal cavity by developing widely the pericardial and diaphragmatic defect behind the heart's preoperative position; 3) to reconstruct the new pericardial sac lying from thoracic to abdominal cavity by covering the heart using EPTFE sheet. This trial of making a new pericardial sac for thoracoabdominal ectopia cordis is not reported so far in the literature. It is expected that the heart has being raised to the thoracic cavity with patient's physical growth after this operation.