OBJECTIVE:Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long-term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure. DESIGN, SETTINGS, AND PATIENTS:We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute contraindications. INTERVENTION AND OUTCOME MEASURES:Demographic and procedural data were correlated with outcome measures. Outcomes analyzed included procedural success, reintervention rates, final circulation type, and functional class. Multivariate analysis and receiver operator curve were used to identify for parameters in predicting biventricular circulation. RESULTS:The procedural success rate was 92% (33 out of 36) in this group with moderate right ventricular hypoplasia (tricuspid valve z score -4.2 ± 3.0, 69.4% of patients with z score <-2.5). Early reintervention rate was 39%, mostly being insertion of modified Blalock-Taussig shunt. Overall reintervention-free survival was 53%, 30%, and 19% at 1, 6, and 12 months postintervention. Despite no significant catch-up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circulation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good predictor of biventricular outcome. (specificity of 100%, positive predictive value 100%) CONCLUSION: Encouraging long-term results with biventricular circulation and functional status were demonstrated with transcatheter pulmonary valve perforation in patients even with moderate hypoplastic right ventricle, which is comparable to that with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio was identified as a potentially useful tool in predicting biventricular circulation.

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目的:经导管瓣膜穿孔治疗肺动脉闭锁完整的室间隔是轻度右室发育不全患者的标准治疗方法。但是,其在中度右心室发育不全中的作用尚未明确。我们试图报告接受该手术的中度发育不良右心室患者的长期预后。
设计,地点和患者:我们对1996年1月至2015年1月间在本机构行经导管肺动脉瓣穿孔的患者进行了回顾性分析。只要没有绝对的禁忌症,就可以进行手术,而与右心室的大小无关。
干预措施和结果指标:人口统计学和程序数据与结果指标相关。分析的结果包括手术成功率,再干预率,最终循环类型和功能类别。多变量分析和接收机操作员曲线用于确定预测双心室循环的参数。
结果:该组中度右心室发育不全(三尖瓣z评分-4.2±3.0,z评分<-2.5的患者中有69.4%)的手术成功率为92%(36分之33)。早期再干预率为39%,主要是插入改良的Blalock-Taussig分流器。干预后1、6和12个月的无干预总生存率为53%,30%和19%。尽管右心室没有明显的追赶性增长,但大多数幸存者(84%)的双心室循环功能状态良好。三尖瓣对二尖瓣的比率> 0.79是双室结局的良好预测指标。 (特异性为100%,阳性预测值为100%)结论:即使是中度右室发育不良的患者,经导管肺动脉穿孔也证明了双室循环和功能状态的长期结果令人鼓舞。肥大。基线三尖瓣对二尖瓣比被确定为预测双心室循环的潜在有用工具。

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