d'Udekem d'Acoz Yves, Pasquet Agnes, Van Caenegem Olivier, Barrea Catherine, Sluysmans Thierry, Noirhomme Philippe, Rubay Jean
Cardiac Surgery Unit, Royal Children's Hospital, Melbourne, Australia.
J Heart Valve Dis. 2004 Mar;13(2):307-12.
Right ventricular dilatation observed after tetralogy of Fallot repair regresses after pulmonary valve implantation, unless the dilation is too severe. The presence of an akinetic patch in the right ventricular outflow tract (RVOT), a known factor promoting right ventricular dilatation, may prevent right ventricular recovery after valve implantation. The exclusion of a large akinetic RVOT area during reoperation of patients presenting with severe post-repair right ventricular dilatation was investigated.
Eight patients underwent a pulmonary infundibuloplasty between May 2000 and October 2002. Their mean preoperative cardothoracic index was 0.66 +/- 0.08, and preoperative NYHA class II (n = 4), III (n = 3) or IV (n = 1). Three patients were offered heart transplantation but refused. All had severe pulmonary regurgitation and underwent a RVOT valve implantation except one patient who had a previous homograft pulmonary valve insertion. Concomitant procedures were tricuspid ring implantation (n = 3), atrial septal defect closure (n = 2), mitral valve repair (n = 1) and modified right atrial Maze (n = 1).
Median follow up time was 13 months (range: 6 -29 months). One patient suffered a fatal ventricular fibrillation at home. All patients but one were in NYHA class I. After a mean of 5 +/- 3 months, their mean workload capacity rose from 115 +/- 19 W to 155 +/- 62 W, and mean VO2max rose from 16.5 +/- 2 to 18.3 +/- 2 ml/min/kg.
Pulmonary infundibuloplasty may be a useful adjunct in reoperation of tetralogy of Fallot patients presenting with severe right ventricular dilatation and large akinetic area of the RVOT.
法洛四联症修复术后观察到的右心室扩张，在植入肺动脉瓣后会消退，除非扩张过于严重。右心室流出道（RVOT）存在运动不能区，这是促进右心室扩张的一个已知因素，可能会妨碍瓣膜植入术后右心室的恢复。本研究探讨了在严重修复后右心室扩张患者再次手术时，排除大面积运动不能的RVOT区域的情况。
2000年5月至2002年10月期间，8例患者接受了肺动脉漏斗部成形术。他们术前平均心胸指数为0.66±0.08，术前纽约心脏协会（NYHA）心功能分级为II级（n = 4）、III级（n = 3）或IV级（n = 1）。3例患者被建议进行心脏移植但拒绝了。所有患者均有严重的肺动脉反流，除1例曾植入同种异体肺动脉瓣的患者外，均接受了RVOT瓣膜植入术。同期手术包括三尖瓣环植入术（n = 3）、房间隔缺损修补术（n = 2）、二尖瓣修复术（n = 1）和改良右心房迷宫手术（n = 1）。
中位随访时间为13个月（范围：6 - 29个月）。1例患者在家中发生致命性心室颤动。除1例患者外，所有患者NYHA心功能分级均为I级。平均5±3个月后，他们的平均工作负荷能力从115±19瓦升至155±62瓦，平均最大摄氧量从16.5±2毫升/分钟/千克升至18.3±2毫升/分钟/千克。
对于存在严重右心室扩张和大面积RVOT运动不能区的法洛四联症患者再次手术，肺动脉漏斗部成形术可能是一种有用的辅助治疗方法。
