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Obstruction in Hypertrophic Cardiomyopathy: Many Faces
Muhannad Abbasi1, Kevin C Ong2, D Brian Newman1
1Department of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota.
Insights
Hypertrophic cardiomyopathy (HCM) involves thickened heart muscle, often causing outflow obstruction. Echocardiography, particularly Doppler, is key for diagnosing, managing, and monitoring this inherited condition.
Area of Science:
- Cardiology
- Inherited cardiovascular diseases
Background:
- Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy.
- It is characterized by left ventricular hypertrophy not caused by other conditions.
- Enhanced actin-myosin interaction leads to excessive myocardial contraction and potential left ventricular outflow tract obstruction.
Purpose of the Study:
- To explore the role of echocardiography in evaluating HCM.
- To emphasize Doppler assessment for identifying and monitoring left ventricular outflow tract obstruction.
- To guide therapeutic decision-making through noninvasive hemodynamic assessment.
Main Methods:
- Utilizing transthoracic echocardiography for screening, diagnosis, and risk stratification.
- Employing Doppler echocardiography to assess dynamic obstruction.
- Incorporating provocative maneuvers for hemodynamic assessment.
- Reviewing echocardiographic findings in relation to anatomical abnormalities.
Main Results:
- Left ventricular outflow tract obstruction occurs in 75% of HCM patients, impacting prognosis and symptoms.
- Echocardiography provides crucial noninvasive hemodynamic assessment.
- Doppler assessment is vital for monitoring responses to medical and interventional therapies.
Conclusions:
- Echocardiography is indispensable in the comprehensive management of HCM.
- Doppler assessment of obstruction is critical for guiding treatment strategies.
- Recognition of obstructive HCM phenotypes informs therapeutic choices, including medical and septal reduction therapies.
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.
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