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Echocardiographic and Histological Examination of Cardiac Morphology in the Mouse
Published on: October 26, 2017
Imaging cardiac morphology in hypertrophic cardiomyopathy: recent advances
1From the Research Department and the Cardiac Imaging Program, St. Francis Hospital-the Heart Center and Stony Brook University, New York, USA.
Insights
Cardiac MRI (CMR) reveals new findings in hypertrophic cardiomyopathy (HCM). Advanced CMR techniques help risk-stratify patients and identify novel phenotypes, improving diagnosis and prognosis.
Area of Science:
- Cardiology
- Medical Imaging
Background:
- Hypertrophic cardiomyopathy (HCM) is a primary genetic heart muscle disease.
- Accurate risk stratification and understanding of HCM phenotypes are crucial for patient management.
Purpose of the Study:
- To detail novel cardiac magnetic resonance (CMR) findings related to cardiac structure and myocardial composition in HCM.
- To explore the role of advanced CMR techniques in HCM evaluation.
Main Methods:
- Quantitative CMR assessment of myocardial fibrosis (replacement and interstitial).
- Analysis of cardiac structure, including left ventricular hypertrophy (LVH) distribution and severity.
- Identification of associated abnormalities like myocardial noncompaction and mitral valve apparatus issues.
Main Results:
- Quantitative CMR assessment of fibrosis aids in risk stratification for adverse outcomes in HCM patients.
- Global LVH is associated with poorer outcomes, while a spiral hypertrophy pattern suggests a better prognosis.
- New CMR phenotypes, including myocardial noncompaction and abnormalities in relatives of HCM probands, are identified.
Conclusions:
- CMR provides valuable structural insights, including hypertrophy and fibrosis patterns, augmenting clinical HCM evaluation.
- Emerging CMR methods offer potential for detecting myocardial fiber disarray and altered stiffness, enhancing understanding of HCM pathophysiology and outcomes.
Purpose Of Review:
To describe new cardiac MRI (CMR) findings on cardiac structure and myocardial composition in hypertrophic cardiomyopathy (HCM).
Recent Findings:
Quantitative CMR assessment of replacement fibrosis and interstitial fibrosis can risk stratify HCM patients for adverse outcomes. Patients with global LVH (increased LV mass index) have more adverse outcomes. The HCM phenotype with a spiral distribution of hypertrophy entails a good prognosis. Myocardial noncompaction can be associated with HCM, as are papillary muscle and mitral apparatus abnormalities. Genotype positive, phenotype negative relatives of HCM probands may be detected by myocardial motion abnormalities. Emerging CMR methods for myocardial fiber disarray and altered myocardial stiffness may shed more light on cardiac structure, function and outcomes in HCM in coming years.
Summary:
CMR structural features of HCM, including severity and distribution of hypertrophy and fibrosis, can augment clinical evaluation of HCM. New CMR phenotypes, associated papillary muscle, mitral leaflet and myocardial noncompaction abnormalities, role of left atrial enlargement, findings in genotype positive phenotype negative HCM, and emerging methods for the detection of myocardial fiber disarray and altered myocardial stiffness may shed light in coming years.
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