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肥厚型心肌病儿童及青少年患者手术矫正后双心室梗阻和左心室流出道梗阻的中期结果

Mid-term outcomes of biventricular obstruction and left ventricular outflow tract obstruction after surgery correction in child and adolescent patients with hypertrophic cardiomyopathy.

作者信息

Zhai Shanshan, Xu Haitao, Fan Chaomei, Yang Yinjian, Hang Fei, Guo Xiying, Wang Hongyue, Duan Fujian, Yan Jun

机构信息

Key Laboratory of Clinical Trial Research in Cardiovascular Drugs, Ministry of Health, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Department of Pediatric Cardiovascular Surgery, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

出版信息

PLoS One. 2018 Feb 6;13(2):e0192218. doi: 10.1371/journal.pone.0192218. eCollection 2018.

Abstract

BACKGROUND

Data on the outcomes of hypertrophic cardiomyopathy (HCM) with biventricular obstruction are limited.

OBJECTIVE

Our aim is to compare mid-term outcomes of biventricular outflow tract obstruction (BVOTO) HCM, left ventricular outflow tract obstruction (LVOTO) HCM and nonobstructive hypertrophic cardiomyopathy (NO-HCM) in children and adolescents who were treated with standard medication or surgical resection.

METHODS

This retrospective study identified 21 BVOTO patients and recruited 27 LVOTO and 24 NO-HCM patients younger than 18 years presenting at our institution. The primary endpoint was all-cause death, and secondary endpoints were cardiovascular events.

RESULTS

More BVOTO patients (61.9%) than LVOTO (19.2%) and NO-HCM patients (25%) exhibited New York Heart Association (NYHA) III/IV status (p < 0.01). Fourteen BVOTO and 16 LVOTO patients obtained a significant reduction of outflow tract pressure gradients after surgery (vs. preoperative baseline, p < 0.001). One of the 14 BVOTO patients died, whereas no deaths occurred among LVOTO patients. Three of 14 BVOTO surgery patients had complete heart block (CHB) and 4 had new right bundle branch block (RBBB), while no CHB or RBBB occurred in the LVOTO surgery patients. The BVOTO patients had a longer duration of aortic cross-clamping and postoperative hospital days than the LVOTO patients (p < 0.05). During a median 42-month follow-up, no deaths occurred among the remaining patients. The primary and secondary endpoint-free survival rates of the BVOTO group were comparable to those of the LVOTO and NO-HCM groups.

CONCLUSIONS

In children and adolescents, BVOTO patients were associated with more severe symptoms than LVOTO and NO-HCM patients; however, good mid-term outcomes similar to those of the LVOTO and NO-HCM groups can be achieved with the application of contemporary cardiovascular treatment strategies. Notably, BVOTO surgery was associated with an increased risk of CHB and RBBB compared to LVOTO surgery.

摘要

背景

关于双心室梗阻性肥厚型心肌病(HCM)预后的数据有限。

目的

我们的目的是比较接受标准药物治疗或手术切除的儿童和青少年中,双心室流出道梗阻(BVOTO)型HCM、左心室流出道梗阻(LVOTO)型HCM和非梗阻性肥厚型心肌病(NO-HCM)的中期预后。

方法

这项回顾性研究确定了21例BVOTO患者,并招募了在我们机构就诊的27例LVOTO患者和24例18岁以下的NO-HCM患者。主要终点是全因死亡,次要终点是心血管事件。

结果

与LVOTO患者(19.2%)和NO-HCM患者(25%)相比,更多的BVOTO患者(61.9%)表现为纽约心脏协会(NYHA)III/IV级状态(p<0.01)。14例BVOTO患者和16例LVOTO患者术后流出道压力阶差显著降低(与术前基线相比,p<0.001)。14例BVOTO患者中有1例死亡,而LVOTO患者中无死亡病例。14例BVOTO手术患者中有3例发生完全性心脏传导阻滞(CHB),4例出现新的右束支传导阻滞(RBBB),而LVOTO手术患者中未发生CHB或RBBB。BVOTO患者的主动脉交叉阻断时间和术后住院天数比LVOTO患者长(p<0.05)。在中位42个月的随访期间,其余患者无死亡病例。BVOTO组的主要终点和次要终点无事件生存率与LVOTO组和NO-HCM组相当。

结论

在儿童和青少年中,BVOTO患者的症状比LVOTO和NO-HCM患者更严重;然而,应用当代心血管治疗策略可取得与LVOTO组和NO-HCM组相似的良好中期预后。值得注意的是,与LVOTO手术相比,BVOTO手术发生CHB和RBBB的风险增加。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1ea/5800690/98304604a6fa/pone.0192218.g001.jpg

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