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老年非限制性室间隔缺损合并重度肺动脉高压患者诊断性治疗及修复策略的中期结果

Midterm results of diagnostic treatment and repair strategy in older patients presenting with nonrestrictive ventricular septal defect and severe pulmonary artery hypertension.

作者信息

Liu Aijun, Li Zhiqiang, Li Xiaofeng, Fan Xiangming, Su Junwu, Zhang Jing, He Yan, Liu Yinglong

机构信息

Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China.

Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029, China. Email:

出版信息

Chin Med J (Engl). 2014;127(5):839-44.

Abstract

BACKGROUND

Congenital heart disease with severe pulmonary arterial hypertension (SPAH), previously thought to have irreversible pulmonary vascular disease (PVD), has been recently successfully corrected using diagnostic treatment and repair strategy by our surgery team. This study aimed to evaluate the midterm results of a selected cohort of older patients with nonrestrictive ventricular septal defect (VSD) and SPAH using diagnostic treatment and repair strategy.

METHODS

The records of 56 patients older than six years with nonrestrictive VSD and SPAH undergoing diagnostic treatment and repair strategy from 2006 to 2012 were retrospectively reviewed. All patients received advanced pulmonary arterial hypertension (PAH) therapy and radical repairs were performed when transcutaneous oxygen saturation (SPO2) increased up to 93%.

RESULTS

There were no operative deaths. SPO2 and baseline six-minute walk test (SMWT) distance of all selected patients increased significantly and mean pulmonary artery pressure (MPAP) regressed significantly after diagnostic treatment and at late follow-up (P < 0.01). The incidence of late postoperative PAH was seen in six (10.7%) patients and by Logistic regression analysis, early postoperative PAH was an independent risk factor related to late postoperative PAH CONCLUSIONS: Diagnostic treatment and repair strategy was effective and safe for treatment of nonrestrictive VSD and SPAH and the midterm results were excellent. Diagnostic treatment strategy was effective in assessing the reversibility of SPAH in older patients associated with nonrestrictive VSD and the PVD in these selective patients is generally reversible.

摘要

背景

先天性心脏病合并严重肺动脉高压(SPAH),以往认为存在不可逆的肺血管疾病(PVD),最近我们的手术团队采用诊断性治疗和修复策略已成功矫正。本研究旨在评估采用诊断性治疗和修复策略的一组大龄非限制性室间隔缺损(VSD)合并SPAH患者的中期结果。

方法

回顾性分析2006年至2012年期间56例年龄大于6岁、患有非限制性VSD合并SPAH并接受诊断性治疗和修复策略的患者记录。所有患者均接受了晚期肺动脉高压(PAH)治疗,当经皮血氧饱和度(SPO2)升至93%时进行根治性修复。

结果

无手术死亡病例。所有入选患者的SPO2和基线六分钟步行试验(SMWT)距离显著增加,诊断性治疗后及晚期随访时平均肺动脉压(MPAP)显著下降(P<0.01)。6例(10.7%)患者出现术后晚期PAH,经逻辑回归分析,术后早期PAH是与术后晚期PAH相关的独立危险因素。结论:诊断性治疗和修复策略治疗非限制性VSD合并SPAH有效且安全,中期结果良好。诊断性治疗策略可有效评估大龄非限制性VSD合并SPAH患者SPAH的可逆性,这些选择性患者的PVD通常是可逆的。

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