筋性部心室中隔欠損症を伴う房室弁交叉（criss-cross heart）に大動脈スイッチ手術を行った1例

Arterial Switch Operation for the Criss-cross Heart Complicated by Muscular Ventricular Septal Defect：Report of a Case

はじめに　房室弁交叉（criss-cross heart：CCH）はさまざまな合併心奇形を伴うが，心室中隔欠損症は必発であり1），これに房室結合が一致するconcordant CCHでは完全大血管転位（TGA）または両大血管右室起始（DORV）を，房室結合が一致しないdiscordant CCHでは修正大血管転位（cTGA）を合併することが多い2～5）．また右室は低形成であることが多く，房室弁騎乗を高率に合併することから，2心室修復が困難なことが多い1）．

Criss-cross heart is an extreamly rare anomaly characterized by abnormal rotation of the heart on its long axis. Almost always there are associated cardiac anomalies such as pulmonary stenosis, ventricular septal defect （VSD） and ventriculoarterial connection discord, and most cases are candidates for Fontan procedure due to hypoplasia of right ventricle or straddling atrioventricular valve. We report a case of arterial switch operation for criss-cross heart with muscular ventricular septal defect. The patient was diagnosed with criss-cross heart, double outlet right ventricle, subpulmonary VSD, muscular VSD and patent ductus arteriosus （PDA）. PDA ligation and pulmonary artery banding （PAB） was performed in the neonatal period, and an arterial switch operation （ASO） was planed at 6 months of age. Preoperative angiography showed nearly normal right ventricular volume and echocardiography showed normal subvalvular structures of atrioventricular valves. ASO, intraventricular rerouting and muscular VSD closure by sandwitch technique were successfully performed.