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Published on: November 2, 2020
Hypertrophic cardiomyopathy - phenotypic variations beyond wall thickness
Nikhil Goyal1, Graham Keir1, Yonah B Esterson1
1Department of Radiology, Northwell Health System, The Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 300 Community Drive, Manhasset, NY 11030, USA.
Insights
Hypertrophic cardiomyopathy (HCM) involves left ventricular hypertrophy (LVH) without other causes. Recognizing associated anatomic variations aids early diagnosis and treatment, even without significant LVH.
Area of Science:
- Cardiology
- Medical Imaging
- Pathology
Background:
- Hypertrophic cardiomyopathy (HCM) is defined by left ventricular hypertrophy (LVH) unexplained by other cardiac or systemic diseases.
- Understanding the diverse morphologic and histologic changes in HCM is crucial for accurate interpretation of cardiac imaging.
- Early detection of HCM-associated findings can lead to timely diagnosis and management, improving patient outcomes.
Purpose of the Study:
- To describe the spectrum of morphologic variations in hypertrophic cardiomyopathy (HCM) beyond myocardial thickening.
- To highlight key imaging features of associated cardiac structures in HCM.
- To emphasize the importance of recognizing these variations for early diagnosis and treatment.
Main Methods:
- Review of imaging findings in patients with hypertrophic cardiomyopathy (HCM).
- Description of characteristic morphologic variations including papillary muscle and mitral valve abnormalities, myocardial crypts, and left ventricular bands.
- Discussion of dystrophic calcification patterns associated with increased myocardial tension.
Main Results:
- Morphologic variations in HCM extend beyond simple myocardial thickening.
- Key findings include abnormalities of papillary muscles and mitral valve, myocardial crypts, and ventricular bands.
- Dystrophic calcification may be present, reflecting increased myocardial wall tension.
Conclusions:
- Recognizing diverse morphologic variations in HCM is essential for accurate diagnosis and management.
- Associated findings may be present even in the absence of significant left ventricular hypertrophy (LVH).
- Comprehensive imaging interpretation aids in the early identification and treatment of hypertrophic cardiomyopathy (HCM).
Abstract:
Hypertrophic cardiomyopathy (HCM) is characterized by left ventricular hypertrophy (LVH) in the absence of another causal disease. Several morphologic and histologic changes have been described. Given the morbidity and mortality associated with HCM, understanding these anatomic variations is key to interpreting imaging. This is especially important since many patients exhibit these associated findings in the absence of LVH and prompt early detection of these variations may lead to early diagnosis and treatment. This article describes the appearance of morphologic variations seen in HCM beyond myocardial thickening including: papillary muscle and mitral valve variants, myocardial crypts, left ventricular myocardial bands, and dystrophic calcification related to increased wall tension.
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