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肥厚型心肌病中的梗阻:多面问题。

Obstruction in Hypertrophic Cardiomyopathy: Many Faces.

机构信息

Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.

Division of Cardiology, St. Paul's Hospital, Vancouver, British Columbia, Canada.

出版信息

J Am Soc Echocardiogr. 2024 Jun;37(6):613-625. doi: 10.1016/j.echo.2024.02.010. Epub 2024 Feb 28.

DOI:10.1016/j.echo.2024.02.010
PMID:38428652
Abstract

Hypertrophic cardiomyopathy (HCM), the most common inherited cardiomyopathy, exhibits left ventricular hypertrophy not secondary to other causes, with varied phenotypic expression. Enhanced actin-myosin interaction underlies excessive myocardial contraction, frequently resulting in dynamic obstruction within the left ventricle. Left ventricular outflow tract obstruction, occurring at rest or with provocation in 75% of HCM patients, portends adverse prognosis, contributes to symptoms, and is frequently a therapeutic target. Transthoracic echocardiography plays a crucial role in the screening, initial diagnosis, management, and risk stratification of HCM. Herein, we explore echocardiographic evaluation of HCM, emphasizing Doppler assessment for obstruction. Echocardiography informs management strategies through noninvasive hemodynamic assessment, which is frequently obtained with various provocative maneuvers. Recognition of obstructive HCM phenotypes and associated anatomical abnormalities guides therapeutic decision-making. Doppler echocardiography allows monitoring of therapeutic responses, whether it be medical therapies (including cardiac myosin inhibitor therapy) or septal reduction therapies, including surgical myectomy and alcohol septal ablation. This article discusses the hemodynamics of obstruction and practical application of Doppler assessment in HCM. In addition, it provides a visual atlas of obstruction in HCM, including high-quality figures and complementary videos that illustrate the many facets of dynamic obstruction.

摘要

肥厚型心肌病(HCM)是最常见的遗传性心肌病,表现为非继发于其他原因的左心室肥厚,具有不同的表型表现。增强的肌球蛋白-肌动蛋白相互作用是心肌过度收缩的基础,常导致左心室的动态梗阻。75%的 HCM 患者在静息或激发状态下发生左心室流出道梗阻,预示着不良预后,导致症状,并常成为治疗靶点。经胸超声心动图在 HCM 的筛查、初步诊断、管理和风险分层中发挥着关键作用。本文探讨了 HCM 的超声心动图评估,重点强调了梗阻的多普勒评估。超声心动图通过非侵入性血流动力学评估为管理策略提供信息,这通常通过各种激发操作获得。识别梗阻性 HCM 表型和相关解剖异常指导治疗决策。多普勒超声心动图可监测治疗反应,无论是药物治疗(包括心肌肌球蛋白抑制剂治疗)还是间隔减少治疗,包括手术心肌切除术和酒精间隔消融术。本文讨论了梗阻的血流动力学以及多普勒评估在 HCM 中的实际应用。此外,它还提供了 HCM 梗阻的视觉图谱,包括高质量的图像和补充视频,这些图像和视频说明了动态梗阻的许多方面。

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