Zhang Yonghui, Hua Zhongdong, Yang Keming, Zhang Hao, Yan Jun, Wang Xu, Chu Junmin, Ma Kai, Li Shoujun
State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
Eur J Cardiothorac Surg. 2014 Aug;46(2):297-303; discussion 303. doi: 10.1093/ejcts/ezt622. Epub 2014 Jan 12.
To determine the effect and safeness of the right ventricle to pulmonary artery connection with occlusion of major aortopulmonary collaterals and pulmonary artery angioplasty to rehabilitate the hypoplastic pulmonary arteries in patients with pulmonary atresia and ventricular septal defect beyond the infant period.
From December 2009 to August 2012, 37 consecutive patients (mean age 1.9 ± 1.7 years, range 0.6-7.2 years) diagnosed with pulmonary atresia, ventricular septal defect and pulmonary artery hypoplasia (Nakata index 90.9 ± 42.6 mm(2)/m(2); McGoon ratio 1.0 ± 0.2) were included in this retrospective study. All patients underwent the procedure of right ventricle to pulmonary artery connection, during which most of them received transcatheter occlusion of major aortopulmonary collaterals and/or pulmonary angioplasty. Mean follow-up was 1.6 ± 0.8 years (range 0.6-3.3 years). Continuous variables are expressed as means ± standard deviation.
There were no early deaths, but one patient died of myocarditis 1 year after the rehabilitation. Significant pulmonary artery growth was obtained (Nakata index 215.1 ± 95.1 mm(2)/m(2), P < 0.001; McGoon ratio 1.6 ± 0.5, P < 0.001) in all of the 37 patients, and among them, 17 patients (45.9%) whose pulmonary growth was considered adequate obtained a complete repair without perforation of the ventricular septal defect. The preoperative McGoon ratio might be a good predictor for adequate pulmonary growth. There was one early death after anatomical repair. At the last visit, all survivors who underwent anatomical repair were in New York Heart Association class I-II with satisfactory haemodynamics.
Connection of the right ventricle to the pulmonary artery is safe and effective to promote the growth of the native pulmonary arteries in patients with pulmonary atresia, ventricular septal defect and hypoplastic native pulmonary arteries. Ultimately, this strategy allows complete repair in the majority of patients beyond infancy.
确定右心室与肺动脉连接术联合封堵主要体肺侧支血管及肺动脉血管成形术对婴儿期后肺动脉闭锁合并室间隔缺损患者发育不良的肺动脉进行修复的效果及安全性。
本回顾性研究纳入了2009年12月至2012年8月期间连续收治的37例患者（平均年龄1.9±1.7岁，范围0.6 - 7.2岁），这些患者均诊断为肺动脉闭锁、室间隔缺损及肺动脉发育不良（中田指数90.9±42.6mm²/m²；麦戈恩比率1.0±0.2）。所有患者均接受了右心室与肺动脉连接术，在此过程中，大多数患者接受了经导管封堵主要体肺侧支血管和/或肺动脉血管成形术。平均随访时间为1.6±0.8年（范围0.6 - 3.3年）。连续变量以均值±标准差表示。
无早期死亡病例，但有1例患者在修复术后1年死于心肌炎。所有37例患者的肺动脉均有显著生长（中田指数215.1±95.1mm²/m²，P<0.001；麦戈恩比率1.6±0.5，P<0.001），其中17例（45.9%）肺动脉生长被认为充足的患者获得了完全修复，且室间隔未穿孔。术前麦戈恩比率可能是肺动脉充分生长的良好预测指标。解剖修复后有1例早期死亡。在最后一次随访时，所有接受解剖修复的幸存者纽约心脏协会心功能分级均为I-II级，血流动力学状况良好。
右心室与肺动脉连接术对于促进肺动脉闭锁、室间隔缺损及发育不良的肺动脉患者自身肺动脉的生长是安全有效的。最终，该策略可使大多数婴儿期后的患者获得完全修复。
