Pelliccia Francesco, Alfieri Ottavio, Calabrò Paolo, Cecchi Franco, Ferrazzi Paolo, Gragnano Felice, Kaski Juan Pablo, Limongelli Giuseppe, Maron Martin, Rapezzi Claudio, Seggewiss Hubert, Yacoub Magdi H, Olivotto Iacopo
Department of Cardiovascular Sciences, University Sapienza of Rome, Rome, Italy.
Department of Cardiovascular and Thoracic Surgery, San Raffaele University Hospital, Milan, Italy.
Int J Cardiol. 2020 Apr 1;304:86-92. doi: 10.1016/j.ijcard.2020.01.021. Epub 2020 Jan 11.
Patients with hypertrophic cardiomyopathy (HCM) exhibit a variable phenotype with ventricular hypertrophy as the cardinal manifestation and left ventricular (LV) outflow tract obstruction (LVOTO) as a key pathophysiologic determinant. Patients with severe LVOTO usually present with exertional dyspnea, exertional syncope, and heart failure symptoms, while successful relief of LVOTO by pharmacological or invasive interventions leads to a dramatic improvement in clinical status. Proper management of obstructive HCM remains challenging and poses numerous clinical dilemmas. Since the development of surgical myectomy over half a century ago, progress in the management of LVOTO in HCM has paralleled technological advances in genetic testing, cardiac imaging, arrhythmic prophylaxis, cardiac surgery and interventional cardiology. These changes have been incorporated in dedicated scientific guidelines on both sides of the Atlantic. However, either the 2011 American guidelines or the 2014 European guidelines remain largely based on expert consensus for lack of recommendations with level of evidence A regarding any of the treatment options commonly employed in HCM. Consequently, management of obstructive HCM patients remains largely subjective and dependent on clinical judgment, local expertise, and patient preference. Following the trend that has emerged for other cardiac diseases amenable to invasive interventions, adequate evaluation and management of obstruction in HCM today requires a multidisciplinary team capable of optimizing referral, choosing the best available options, minimizing complications and ensuring state-of-the-art results. The concept of an HCM Heart Team is coming of age. This review aims to provide an update of available pharmacologic and invasive options for the management of LVOTO in HCM, either in adulthood or in childhood, highlighting areas for multidisciplinary integration and future development.
肥厚型心肌病（HCM）患者表现出可变的表型，以心室肥厚为主要表现，左心室（LV）流出道梗阻（LVOTO）为关键的病理生理决定因素。严重LVOTO的患者通常表现为劳力性呼吸困难、劳力性晕厥和心力衰竭症状，而通过药物或侵入性干预成功缓解LVOTO可使临床状况显著改善。梗阻性HCM的恰当管理仍然具有挑战性，并带来诸多临床难题。自半个多世纪前开展外科心肌切除术以来，HCM中LVOTO的管理进展与基因检测、心脏成像、心律失常预防、心脏外科和介入心脏病学等技术进步同步。这些变化已被纳入大西洋两岸专门的科学指南。然而，2011年美国指南和2014年欧洲指南在很大程度上仍基于专家共识，因为对于HCM中常用的任何治疗选择，均缺乏证据等级为A的推荐。因此，梗阻性HCM患者的管理在很大程度上仍然主观，依赖于临床判断、当地专业知识和患者偏好。遵循其他适合侵入性干预的心脏病所出现的趋势，如今对HCM梗阻进行充分评估和管理需要一个多学科团队，该团队能够优化转诊、选择最佳可用方案、将并发症降至最低并确保达到最新的治疗效果。HCM心脏团队的概念正在走向成熟。本综述旨在提供关于HCM中LVOTO管理的现有药物和侵入性选择的最新信息，涵盖成人和儿童患者，突出多学科整合和未来发展的领域。
