Fallot四徴症に対し根治術を施行したDiGeorge症候群の1例

Cardiac operation on a patient of Tetralogy of Fallot associated with DiGeorge syndrome:a case report

　DiGeorge症候群は第3,4鰓弓の発生異常により胸腺と副甲状腺の無ないし低形成を認め，高率に心大血管異常を合併するまれな疾患であるが，その手術例の報告は少ない。今回我々はpartial DiGeorge症候群を合併したFallot四徴症の根治手術例を経験したので，若干の文献的考察を加え報告する。

A case of 7-year-old boy with Teralogy of Fallot associated with DiGeorge syndrome was reported. He had been diagnosed as cardiofacial syndrome with hypoparathyroidism because of hypocalcemia, convul-sion, cardiac defect and the characteristic face. But immunologic study showed hypofunction of cellular immunity and cardiac catheterization and angiogram revealed the Tetralogy of Fallot. So we diagnosed as DiGeorge syndrome and performed operation. The thymus was not found and we close the ventricular defect with patch and reconstructed right ventricular outflow tract with nottransannular patch. We often measured serum ionized calcium level and correceted with calcium gluconate intravenously. Peritonealdia-lysis was done due to oligouria and much inotropic support was necessary.