肺动脉闭锁伴室间隔完整的手术治疗

摘要

肺动脉闭锁伴室间隔完整(pulmonary atresia with intact ventricular septum,PA/IVS)为复杂发绀型先天性心脏病之一,病死率高,需及早干预.随着对该病认识的不断深入及外科手术的发展,该病手术治疗方案也在逐渐发生着变化,某些方面发生了根本变化.对该病血流动力学认识程度,是手术方案选择及改善预后的重要影响因素.此外术前全面准确评估右室发育程度,对手术方案选择及预后改善也至关重要.目前,普遍认为在形态学上对右室发育程度进行分类,可获得最好效果的治疗策略.手术治疗方案由早期的外科体肺分流术,发展至现在的介入手术及杂交手术,极大改善预后、减小创伤.其中经皮肺动脉瓣射频打孔、球囊扩张及PDA支架植入可作为伴右室发育不良PA/IVS首选治疗方案.尤其值得注意的是,对于右室轻度甚至中度发育不良的PA/IVS患儿,可单纯应用介入手术达到治愈效果.杂交手术较传统外科手术明显减少创伤,对于介入手术发展水平相对落后地区,也是一种选择.该文回顾并总结了PA/IVS认识历程及手术治疗方法相关进展,旨在为该病临床诊断及治疗提供参考.%Pulmonary atresia with intact ventricular septum(PA/IVS) is one of the complicated cyanotic congenital heart diseases with high mortality,which needs early intervention after birth.With further understanding of the disease and the development of surgery,the operative plan of the disease is gradually changing and some fundamental changes have taken place.The hemodynamic awareness of the disease is an important factor in the selection of surgery and the improvement of prognosis.In addition,it is also important for the selection of surgery and the improvement of prognosis to evaluate the development degree of the right ventricle comprehensively and accurately.At present,it is generally believed that the most effective treatment strategy can be obtained by classifying the development degree of the right ventricle in morphology.The surgical treatment has developed from early surgical pulmonary bypass to the interventional and hybrid surgery,which greatly improves prognosis and reduces surgical trauma.Among them,the pereutaneous pulmonary valve radiofrequency perforation plus balloon dilation and PDA stent implantation can be used as the preferred treatment for PA/IVS with dysplasia of the right ventricle.Particularly,PA/IVS can be cured with the interventional surgery alone for children with mild or even moderate dysplasia of the right ventricle.Hybrid surgery that significantly reduces trauma compared with traditional surgery is also an important choice for the areas where interventional surgery is relatively outdated.This article reviews and summarizes the progress of recognition and surgical treatment of PA/IVS,in order to provide reference for the clinical diagnosis and treatment.