Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease
Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease
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DOI：
10.1016/j.ijcard.2019.02.069
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发表时间：
2019-06-15
影响因子：
3.5
通讯作者：
Amedro, Pascal
中科院分区：
文献类型：
--
作者：
Abassi, Hamouda;Gavotto, Arthur;Amedro, Pascal
背景资料：肺功能受损是成人先天性心脏病（CHD）死亡率的独立预测因子，但在儿童CHD人群中的研究很少。目的：比较CHD患儿与健康对照组的肺功能，并评估其与临床结局、运动能力和生活质量的关系。方法：对834名儿童（555名CHD和279名对照受试者）进行了完整的肺量测定和心肺运动试验（CPET）的横断面多中心研究。使用第5百分位数（Z评分= -1.64）定义正常下限。使用多变量分析研究了临床和CPET变量与肺功能测定的相关性。儿童及其父母填写Kidscreen健康相关生活质量问卷。CHD患儿用力肺活量（FVC）和第一秒用力呼气量（FEV 1）Z评分值低于对照组（分别为-0.4 +/- 1.5 vs. 0.4 +/- 1.3，P < 0.001和-0.5 +/- 1.4 vs. 0.4 +/- 1.2，P < 0.001），无任何阻塞性气道疾病。冠心病组限制型的发生率高于对照组（20%比4%，P < 0.0001）。FVC Z评分主要在复杂CHD中受损，如异位（-1.1 +/-0.6）、单心室（-1.0 +/-0.2）和心室流出道复杂异常（-0.9 +/-0.1）。在多变量分析中，FVC与年龄、体重指数、峰值摄氧量、遗传异常、心脏手术和心导管手术次数相关。FVC和FEV 1与自我和代理相关的生活质量scores.Conclusion：这些结果表明，肺功能应监测在生命早期，从童年，在CHD人群。(C)2019 Elsevier B. V.版权所有。
Background: Impaired pulmonary function is an independent predictor of mortality in adult congenital heart disease (CHD), but has been scarcely studied in the paediatric CHD population.Aims: To compare the pulmonary function of children with CHD to healthy controls, and evaluate its association with clinical outcomes, exercise capacity, and quality of life.Methods: Cross-sectional multicentre study among 834 children (555 CHD and 279 control subjects) who underwent a complete spirometry and a cardiopulmonary exercise test (CPET). The 5th centile (Z-score = -1.64) was used to define the lower limit of normal. The association of clinical and CPET variables with spirometry was studied using a multivariate analysis. Children and their parents filled in the Kidscreen health-related quality of life questionnaire.Results: Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) Z-scores values were lower in children with CHD than controls (-0.4 +/- 1.5 vs. 0.4 +/- 1.3, P < 0.001 and -0.5 +/- 1.4 vs. 0.4 +/- 1.2, P < 0.001, respectively), without any obstructive airway disorder. Restrictive pattern was more frequent in CHD patients than in controls (20% vs. 4%, P < 0.0001). FVC Z-scores were predominantly impaired in complex CHD, such as heterotaxy (-1.1 +/- 0.6), single ventricle (-1.0 +/- 0.2), and complex anomalies of the ventricular outflow tracts (-0.9 +/- 0.1). In multivariate analysis, FVC was associated with age, body mass index, peak oxygen uptake, genetic anomalies, the number of cardiac surgery and cardiac catheter procedures. FVC and FEV1 correlated with self and proxy-related quality of life scores.Conclusion: These results suggest that pulmonary function should be monitored early in life, from childhood, in the CHD population. (C) 2019 Elsevier B.V. All rights reserved.