摘要
目的总结室间隔完整型肺动脉闭锁的外科治疗。方法回顾性分析1995年10月至2004年10月我院收治的3例室间隔完整型肺动脉闭锁患儿的临床资料,复习相关文献,探讨其临床分型、手术时机及手术方法。结果3例中男1例,女2例;年龄36小时～3个月,其中新生儿2例,婴儿1例,体重3.0～4.3kg,平均3.4kg。2例新生儿为急诊入院,行心脏超声心动图检查;1例婴儿术前行心导管及心脏造影检查。3例肺动脉闭锁部位均发生在肺动脉瓣水平,右心室及肺动脉发育较好(Z值2.2～3.0,肺动脉指数>150mm2/m2)。3例均在全身麻醉中度低温体外循环下行一期根治手术。术后早期死亡1例,死因为严重低心排血量综合征。长期随访2例,平均随访8.6年,心功能I级。结论室间隔完整型肺动脉闭锁明确诊断后应尽快手术治疗,根据患儿右心室、三尖瓣及肺动脉发育情况选择合适的手术时机和术式。Z值可正确反映右心室和三尖瓣发育情况,可作为选择术式的重要依据。
Objective To study thc surgical treatment of pulmonary atresia with intact ventricular septum（PA-IVS）. Methods The clinical data of three patients with PA-IVS underwent surgical treatment in our hospital from 1995 to 2004 were analyzed retrospectively. We explored the indicate.on, optimal time and technique of operate.on. Results There were one male and two female including two neonates and one infant. The patients＇ age ranged from 36 hours to three months old with body weight from 3.0 kg to 4.3 kg. All three patients complained with PDA and ASD/POF. Through a standard median stenotomy incision, three patients underwent the operation under moderate hypothemia and cardiopulmonary bypass. All of them received one-stage radical operation with mean Z value 2.7 and PIA〉150 mm^2/m^2. One neonate died two days after operation by cause of severe low cardiac output syndrome. The other two patients were followed-up with 8.6 years. The postoperative heart function （NYHA） was class I in 2 patients. Conclusion An early operation on patients with PA-IVS can be accomplished with satisfactory outcomes. It is feasible to evaluate indications of operation and choose radical or palliative procedures by consideration of Z value and PAl.
出处
《罕少疾病杂志》
2005年第6期5-7,10,共4页
Journal of Rare and Uncommon Diseases
基金
吴阶平医学基金会临床科研资助项目(编号2005-86-F)
关键词
室间隔完整型肺动脉瓣闭锁
外科治疗
先天性心脏病
婴幼儿
pulmonary atresia with intact ventricular septum
surgical treatment
congenital heart disease
