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先天性心脏病的心肺运动试验:从儿科到成人期的一个永无止境的故事

Cardiopulmonary Exercise Testing in Congenital Heart Disease: A Never-Ending Story from Paediatrics to Adult Life.

作者信息

Guglielmi Giulia, Moscatelli Sara, Rocchetti Giorgia, Agostoni Piergiuseppe, Chessa Massimo, Mapelli Massimo

机构信息

Pediatric and Adult Congenital Heart Centre, IRCCS-Policlinico San Donato, Piazza Edmondo Malan, 2, 20097 Milan, Italy.

Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London WC1N 3JH, UK.

出版信息

Children (Basel). 2025 Sep 3;12(9):1175. doi: 10.3390/children12091175.

Abstract

Cardiopulmonary exercise testing (CPET) is increasingly recognized as a key tool for evaluating patients with congenital heart disease (CHD). While traditional assessments focus on resting parameters, CPET provides dynamic, integrated insight into cardiovascular, respiratory, and muscular function during exertion. This review explores the clinical value of CPET across the spectrum of CHD, with dedicated focus on its applications in both adult and paediatric populations. We analyse the prognostic significance of key CPET parameters-particularly peak oxygen consumption (peak VO), ventilatory efficiency (VE/VCO slope), and heart rate dynamics-within distinct anatomical and physiological categories of CHD. CPET reliably detects exercise intolerance, guides intervention timing, informs exercise prescription, and stratifies risk. Peak VO and heart rate reserve are consistently associated with adverse outcomes across most CHD types. However, the prognostic utility of other variables, such as the VE/VCO slope, varies with pathophysiology-being less reliable in cyanotic lesions like Eisenmenger syndrome. In paediatric patients, CPET must be adapted to growth-related physiological variability and is increasingly used to assess quality of life, functional limitation, and response to therapy. CPET is a powerful, non-invasive tool that should be integrated into routine management of CHD patients across all ages. It enhances risk assessment, supports tailored care, and promotes safe physical activity, ultimately contributing to improved long-term outcomes and quality of life.

摘要

心肺运动试验(CPET)日益被视为评估先天性心脏病(CHD)患者的关键工具。传统评估侧重于静息参数,而CPET能在运动过程中对心血管、呼吸和肌肉功能提供动态、综合的洞察。本综述探讨了CPET在各类CHD中的临床价值,特别关注其在成人和儿科人群中的应用。我们分析了关键CPET参数(尤其是峰值耗氧量(peak VO)、通气效率(VE/VCO斜率)和心率动态)在不同解剖和生理类别的CHD中的预后意义。CPET能可靠地检测运动不耐受,指导干预时机,为运动处方提供依据并分层风险。在大多数CHD类型中,峰值VO和心率储备始终与不良结局相关。然而,其他变量(如VE/VCO斜率)的预后效用因病理生理而异,在艾森曼格综合征等紫绀性病变中不太可靠。在儿科患者中,CPET必须适应与生长相关的生理变异性,并且越来越多地用于评估生活质量、功能受限和对治疗的反应。CPET是一种强大的非侵入性工具,应纳入所有年龄段CHD患者的常规管理中。它增强了风险评估,支持个性化护理并促进安全的体育活动,最终有助于改善长期结局和生活质量。

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