Tucker W Y, McKone R C, Weesner K M, Kon N D
Department of Cardiothoracic Surgery, Bowman Gray School of Medicine, Wake Forest University Medical Center, Winston-Salem, N.C. 27103.
J Thorac Cardiovasc Surg. 1990 May;99(5):885-8.
Our 100% mortality rate with first-stage palliation of hypoplastic left heart syndrome performed with cardiopulmonary bypass led us to a procedure not necessitating bypass. In nine neonates with this congenital heart defect, a woven Dacron graft was placed from the main pulmonary artery to the descending thoracic aorta. The patent ductus arteriosus was ligated and the main pulmonary artery banded distal to the graft and proximal to the bifurcation. Five patients were extubated within 4 days. Only low-dose inotropic support was required in eight of the nine. There were no bleeding problems. Four patients died in the hospital: one of Candida sepsis at 81 days, one of low cardiac output at 2 days, and two of restrictive atrial septal defect at 3 and 5 days. The five living patients were discharged 11 to 80 days postoperatively (mean 38 days). We now perform balloon septostomies preoperatively in all patients and believe that this will improve the survival rate. We believe this simpler approach to the treatment of hypoplastic left heart syndrome may allow survival for a cardiac transplant or a staged Fontan procedure at a later date for more definitive treatment.
我们在采用体外循环进行第一阶段姑息性治疗的左心发育不全综合征患者中，100%的死亡率促使我们采用一种无需体外循环的手术方法。对于9例患有这种先天性心脏缺陷的新生儿，将编织涤纶移植物从主肺动脉放置至胸降主动脉。结扎动脉导管，并在移植物远端和分叉近端对主肺动脉进行束带处理。5例患者在4天内拔管。9例中的8例仅需要低剂量的正性肌力药物支持。未出现出血问题。4例患者在医院死亡：1例在81天时死于念珠菌败血症，1例在2天时死于低心排血量，2例在3天和5天时死于限制性房间隔缺损。5例存活患者在术后11至80天（平均38天）出院。我们现在对所有患者术前均进行球囊房间隔造口术，并认为这将提高生存率。我们相信这种治疗左心发育不全综合征的更简单方法可能使患者存活至后期进行心脏移植或分期Fontan手术以获得更确切的治疗。
