室间隔完整型肺动脉闭锁及危重肺动脉瓣狭窄新生儿手术疗效评价及随访分析

Outcome and follow-up analysis of transcatheter or surgical therapy for neonates with pulmonary atresia with intact ventricular septum and critical pulmonary stenosis

目的分析室间隔完整型肺动脉闭锁(PA/IVS)及危重肺动脉瓣狭窄(CPS)新生儿的临床资料,评价手术安全性及有效性,探讨治疗策略的选择。方法2006年11月至2018年8月,共计105例新生儿患儿(PA/IVS 51例、CPS 54例)在广东省心血管病研究所接受手术治疗(介入手术组43例、外科手术组62例),术前均存在轻-中度右心室发育不良,介入手术方式包括经皮肺动脉瓣射频打孔术与经皮球囊肺动脉瓣成形术(PBPV),外科手术方式包括右心室流出道重建术、肺动脉瓣交界切开术、闭式Brock术,部分病例加行体-肺分流术。出院后定期随访,随访时长(40.1±36.9)个月。通过分析住院资料与随访结果,评价介入手术与外科手术的安全性与疗效。结果介入手术组手术时长、住院时长、并发症发生率均低于外科手术组[(107.8±61.6) min比(183.3±36.8) min,(18.6±7.9) d比(31.1±13.4) d,30.2%比80.6%],差异均有统计学意义(均P=0.000)。介入手术组死亡2例(4.7%),存活病例均获得双心室矫治;外科手术组死亡5例(8.1%),存活病例中,39例(69.6%)为双心室循环,3例(5.4%)行上腔静脉-肺动脉连接术,为部分双心室循环,另有14例(25.0%)待评估是否需下一期手术。结论右心室轻-中度发育不良的PA/IVS、CPS患儿在新生儿期经手术治疗后大部分可获得双心室循环。对于CPS新生儿,PBPV是安全有效的术式,而PA/IVS射频打孔术并发右心室流出道穿孔仍是介入治疗中亟待解决的技术难点。

Objective To evaluate the safety and effectiveness of transcatheter or surgical procedure for neonates diagnosed with pulmonary atresia with intact ventricular septum (PA/IVS) or critical pulmonary stenosis (CPS) by retrospectively analyzina the clinical data, thus to discuss the clinical strategy decision. MethodsFrom November 2006 to August 2018, 105 neonates (PA/IVS 51 cases, CPS 54 cases) went through transcatheter or surgical procedure in Guangdong Provincial Cardiovascular Institute (transcatheter procedure 43 cases, surgery 62 cases). All cases manifested mild to moderate right ventricular dysplasia.Transcatheter procedure included pulmonary radio frequency perforation and percutaneous balloon pulmonary valvuloplasty (PBPV). Surgical procedure included reconstruction of right ventricular outflow tract (transannular patch), pulmonary valvotomy and closed transventricular pulmonary valvuloplasty, while Aortic-Pulmonary shunt was performed in certain cases.Average follow-up period was (40.1±36.9) months.Hospitalization and follow-up data were analyzed to evaluate the safety and efficacy of transcatheter and surgical procedure. ResultsOperative time, hospital stay, complication rate were lower in transcatheter procedure group than those of surgery group[(107.8±61.6) min vs.(183.3±36.8) min,(18.6±7.9) d vs.(31.1±13.4) d, 30.2% vs.80.6%], and the differences were significant(all P=0.000). In transcatheter procedure group, 2 cases (4.7%) died and biventricular circulation is obtained in all survival cases.In surgery group, 5 cases (8.1%) died.Of the survival cases from surgery group, 39 cases (69.6%) obtained biventricular circulation, 3 cases (5.4%) obtained one and a half ventricular circulation while other 14 cases (25.0%) were candidates for biventricular repair. ConclusionsBiventricular circulation can be achieved in most PA/IVS or CPS neonates with mild-moderate right ventricular dysplasia.PBPV turns out to be a safe and effective therapy for neonates with CPS while perforation of right ventricular outflow tract remains a nonnegligible complication in transcatheter therapy for PA/IVS.