Kaminishi Y, Atsumi N, Terada Y, Nakamura K, Gomi S, Mitsui T
Department of Cardiovascular Surgery, University of Tsukuba Hospital, Ibaraki, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1996 Dec;44(12):2164-71.
A 3-year-old boy with hereditary spherocytosis (HS) underwent anatomical correction for double-outlet right ventricle. In addition to acute injury to the red blood cells during cardiopulmonary bypass, chronic mechanical trauma related to the presence of internal conduit and transannular patch must be considered in this patient with HS. We therefore performed splencetomy prior to cardiac surgery to prevent perioperative serious hemolysis. Preoperative examination showed the anatomy was considered suitable for REV procedure. The position of the aorta was anterior, VSD was classified as subpulmonic and associated with subvalvular stenosis. Under cardiopulmonary bypass, infundibular septum was resected and intraventricular tunnel was constructed. The pulmonary artery was transected at the level of the valve commissures and translocated onto the right ventricle without transection of aorta. Right ventricular outflow tract was reconstructed with a patch bearing monocusp. No serious complication due to hemolysis occurred perioperatively. Postoperative angiocardiography revealed no stenosis in both left and right ventricular outflow tract. The patient was discharged on the 57th postoperative day and is doing well.
一名3岁遗传性球形红细胞增多症（HS）男孩接受了双出口右心室的解剖矫正术。对于该HS患者，除了体外循环期间红细胞的急性损伤外，还必须考虑与体内管道和跨环补片相关的慢性机械性创伤。因此，我们在心脏手术前进行了脾切除术，以预防围手术期严重溶血。术前检查显示解剖结构适合采用REV手术。主动脉位置靠前，室间隔缺损归类为肺动脉瓣下型并伴有瓣下狭窄。在体外循环下，切除漏斗部间隔并构建心室内隧道。肺动脉在瓣膜交界处水平横断并移位到右心室，主动脉未横断。用带单瓣的补片重建右心室流出道。围手术期未发生因溶血导致的严重并发症。术后心血管造影显示左、右心室流出道均无狭窄。患者术后第57天出院，恢复良好。
