大動脈縮窄とEbstein奇形を合併した両大血管右室起始症に対する二期的修復術の1例

Successful Staged Surgical Management for Double Outlet Right Ventricle with Ebstein’s Anomaly and Aortic Coarctation；Report of a Case

はじめに　両大血管右室起始症に大動脈縮窄症とEbstein奇形が合併することはきわめてまれである．われわれは新生児期より心不全症状を認めた症例に対し，二期的修復術を施行して良好な結果を得たので報告する．

A combination of double outlet right ventricle （DORV） with Ebstein’s anomaly and aortic coarctation （CoA） is vary rare. We report a successful staged surgical repair for DORV with Ebstein’s anomaly associated with CoA. The patient had subaortic perimembronous ventricular septal defect （10×7.5 mm） and small atrial septal defect （3 mm）. Two great arteries were side by side position. The posterior leaflet of the tricuspid valve was 10 mm displaced to the right ventricle and tricuspid valve regurgitation was severe. The patients was unsuitable for primary biventricular repair because the size of the mitral valve was considered to be small. First, bilateral pulmonary artery banding was performed after balloon atrio-septostomy in the neonatal period. Three months after the 1st operation, cardiac catheteraization revealed that the left and right heart structure was suitable for biventricular repair. At 4-month-old-age, aortic arch reconstruction, intra-cardiac rerouting and tricuspid valve repair were performed. Post operative tricuspid valve regurgitation was mild.

The patient was discharged on the 12th postoperative day with good hemodynamic condition.