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.

中文翻译：

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2020年梗阻性肥厚型心肌病的多学科评估和治疗：面向HCM心脏小组。

肥厚型心肌病（HCM）患者的表现型以心室肥大为主要表现，左心室（LV）流出道梗阻（LVOTO）为主要病理生理指标。患有严重LVOTO的患者通常表现为劳累性呼吸困难，劳累性晕厥和心力衰竭症状，而通过药理或侵入性干预措施成功缓解LVOTO可使临床状况得到显着改善。阻塞性HCM的正确管理仍然具有挑战性，并带来许多临床难题。自半个多世纪以来外科手术切除术的发展以来，HCM中LVOTO的管理进展与基因检测，心脏成像，心律失常预防，心脏手术和介入性心脏病学的技术进步并驾齐驱。这些变化已纳入大西洋两岸的专门科学指南中。但是，2011年美国指南或2014年欧洲指南仍主要基于专家共识，因为缺乏有关HCM常用治疗选择的证据为A级的建议。因此，梗阻性HCM患者的治疗在很大程度上仍然是主观的，并且取决于临床判断，当地专家和患者的喜好。遵循其他适合于侵入性干预措施的心脏病的发展趋势，当今对HCM梗阻的充分评估和管理要求多学科团队能够优化转诊，选择最佳可行方案，最大程度降低并发症并确保最新技术水平结果。HCM心脏团队的概念已经成熟。这篇综述旨在为成年或儿童期HCM中LVOTO的治疗提供可用的药理和侵入性治疗方法的更新，重点介绍了多学科整合和未来发展的领域。