Department of Clinical and Molecular Medicine, University "La Sapienza," Rome, Italy.
Ann Am Thorac Soc. 2017 Jul;14(Supplement_1):S102-S109. doi: 10.1513/AnnalsATS.201611-884FR.
Understanding the functional limitation in hypertrophic cardiomyopathy, the most common inherited heart disease, is challenging. In addition to the occurrence of disease-related complications, several factors are potential determinants of exercise limitation, including left ventricular hypertrophy, myocardial fiber disarray, left ventricular outflow tract obstruction, microvascular ischemia, and interstitial fibrosis. Furthermore, drugs commonly used in the daily management of these patients may interfere with exercise capacity, especially those with a negative chronotropic effect. Cardiopulmonary exercise testing can safely and objectively evaluate the functional capacity of these patients and help the physician in understanding the mechanisms that underlie this limitation. Features that reduce exercise capacity may predict progression to heart failure in these patients and even the risk of sudden cardiac death.
了解肥厚型心肌病(最常见的遗传性心脏病)的功能障碍具有挑战性。除了发生与疾病相关的并发症外,一些因素也是运动受限的潜在决定因素,包括左心室肥厚、心肌纤维排列紊乱、左心室流出道梗阻、微血管缺血和间质纤维化。此外,这些患者日常管理中常用的药物可能会影响运动能力,特别是那些具有负变时作用的药物。心肺运动试验可以安全且客观地评估这些患者的功能能力,并帮助医生了解导致这种限制的机制。降低运动能力的特征可以预测这些患者进展为心力衰竭,甚至发生心源性猝死的风险。