1 Heart Failure Unit, Istituto di Ricerca e Cura a Carattere Scientifico Centro Cardiologico Monzino, Università degli Studi di Milano, Milan, Italy; and.
2 Department of Pediatric Cardiology and Congenital Heart Disease, Deutsches Herzzentrum München, Technische Universität München, Munich, Germany.
Ann Am Thorac Soc. 2017 Jul;14(Supplement_1):S93-S101. doi: 10.1513/AnnalsATS.201611-876FR.
Recently, the number of patients with congenital heart diseases reaching adulthood has been progressively increasing in developed countries, and new issues are emerging: the evaluation of their capacity to cope with physical activity and whether this knowledge can be used to optimize medical management. A symptom-limited cardiopulmonary exercise test has proven to be an essential tool, because it can objectively evaluate the functional cardiovascular capacity of these patients, identify the pathological mechanisms of the defect (circulatory failure, shunts, and/or pulmonary hypertension), and help prescribe an individualized rehabilitation program when needed. The common findings on cardiopulmonary exercise testing in patients with congenital heart diseases are a reduced peak [Formula: see text]o, an early anaerobic threshold, a blunted heart rate response, a reduced increase of Vt, and an increased [Formula: see text]e/[Formula: see text]co. All these measures suggest common pathophysiological abnormalities: (1) a compromised exercise capacity from anomalies affecting the heart, vessels, lungs, or muscles; (2) chronotropic incompetence secondary to cardiac autonomic dysfunction or β-blockers and antiarrhythmic therapy; and (3) ventilatory inefficiency caused by left-heart failure with pulmonary congestion, pulmonary hypertension, pulmonary obstructive vascular disease, or cachexia. Most of these variables also have prognostic significance. For these patients, cardiopulmonary exercise testing allows evaluation and decisions affecting lifestyle and therapeutic interventions.
最近，在发达国家，成年先天性心脏病患者的数量逐渐增加，新问题也随之出现：评估他们应对身体活动的能力，以及是否可以利用这些知识来优化医疗管理。症状限制心肺运动试验已被证明是一种必不可少的工具，因为它可以客观地评估这些患者的心血管功能，确定缺陷的病理机制（循环衰竭、分流和/或肺动脉高压），并在需要时帮助制定个体化的康复计划。先天性心脏病患者心肺运动试验的常见发现包括峰值 [Formula: see text]o 降低、无氧阈值提前、心率反应迟钝、Vt 增加减少和 [Formula: see text]e/[Formula: see text]co 增加。所有这些措施都表明存在常见的病理生理异常：（1）影响心脏、血管、肺部或肌肉的异常导致运动能力受损；（2）心脏自主神经功能障碍或β受体阻滞剂和抗心律失常治疗引起的变时性无能；（3）左心衰竭伴肺充血、肺动脉高压、肺阻塞性血管疾病或恶病质导致通气效率降低。这些变量中的大多数也具有预后意义。对于这些患者，心肺运动试验可以评估并做出影响生活方式和治疗干预的决策。
