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MR Imaging in Hypertrophic Cardiomyopathy: From Magnet to Bedside
1From the Department of Radiology, Gasthuisberg University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium (J.B.); and Referral Center for Myocardial Diseases, Azienda Universitaria Careggi, Florence, Italy (I.O.).
Insights
Hypertrophic cardiomyopathy (HCM) is a complex genetic heart disease. Magnetic resonance (MR) imaging aids in diagnosis, risk stratification, and differentiating HCM from other conditions.
Area of Science:
- Cardiology
- Medical Imaging
- Genetics
Background:
- Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder.
- HCM presents with genetic, phenotypic, and clinical heterogeneity.
- Early diagnosis and risk stratification are crucial for managing HCM patients.
Purpose of the Study:
- To review the role of magnetic resonance (MR) imaging in diagnosing and managing hypertrophic cardiomyopathy.
- To highlight MR imaging's utility in risk profiling and differentiating HCM from phenocopies.
Main Methods:
- Review of current literature on MR imaging in HCM.
- Discussion of MR imaging's multiparametric capabilities (spatial, contrast, temporal resolution).
Main Results:
- MR imaging provides detailed characterization of HCM phenotype and functional consequences.
- MR imaging assesses dynamic obstruction, perfusion abnormalities, and myocardial fibrosis.
- MR imaging is crucial for differentiating HCM from other cardiac conditions like amyloidosis.
Conclusions:
- MR imaging is an essential tool complementing echocardiography in HCM management.
- It aids in treatment planning, monitoring disease progression, and arrhythmic risk stratification.
- MR imaging enhances clinical decision-making for hypertrophic cardiomyopathy patients.
Abstract:
Hypertrophic cardiomyopathy ( HCM hypertrophic cardiomyopathy ), the most common genetically transmitted cardiac disorder, has been the focus of extensive research over the past 50 years. HCM hypertrophic cardiomyopathy is a multifaceted disease with highly heterogeneous genetic background, phenotypic expression, clinical presentation, and long-term outcome. Though most patients have an indolent course with a life expectancy comparable to that of the general population, early diagnosis and accurate risk profiling are essential to identify the sizeable subset at increased risk of sudden cardiac death or disease progression and heart failure-related complications, requiring aggressive management options. Imaging has a central role in the diagnosis and prognostic assessment of HCM hypertrophic cardiomyopathy patients, as well as screening of potentially affected family members. In this context, magnetic resonance (MR) imaging has recently emerged as an ideal complement to transthoracic echocardiography. Its multiparametric approach, fusing spatial, contrast, and temporal resolution, provides the clinician with detailed characterization of the HCM hypertrophic cardiomyopathy phenotype and assessment of its functional consequences including causes and site of dynamic obstruction, presence and extent of myocardial perfusion abnormalities, and fibrosis. Moreover, MR is key in differentiating HCM hypertrophic cardiomyopathy from "phenocopies"-that is, hearts with similar morphology but profoundly different etiology, such as amyloid or Anderson-Fabry disease. Long term, the incremental information provided by MR is relevant to planning of septal reduction therapies, identification of the early stages of end-stage progression, and stratification of arrhythmic risk. The aim of this review is to depict the increasingly important role of MR imaging in relation to the complexity of HCM hypertrophic cardiomyopathy , highlighting its role in clinical decision making.
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