Obstruction in Hypertrophic Cardiomyopathy: Many Faces

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 obstructi...

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Veröffentlicht in:Journal of the American Society of Echocardiography 2024-06, Vol.37 (6), p.613-625
Hauptverfasser: Abbasi, Muhannad, Ong, Kevin C., Newman, D. Brian, Dearani, Joseph A., Schaff, Hartzell V., Geske, Jeffrey B.
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Sprache:eng
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Zusammenfassung: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. •Doppler allows for evaluation of LVOT, midventricular, and multilevel obstruction.•An organized approach is needed for dynamic LVOT obstruction provocation.•Doppler evaluation is integral for HCM myosin inhibitor management.•TTE guides septal reduction therapy (SRT) candidacy, intraprocedural assessment, and post-SRT follow-up.
ISSN:0894-7317
1097-6795
DOI:10.1016/j.echo.2024.02.010