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Cardiac Imaging

E2693. Climbing the Wall: Beyond Wall Thickness in Hypertrophic Cardiomyopathy

Esterson Y,  Saba S,  Shah A,  Rahmani N,  Cohen S. Northwell Health System, Manhasset, NY

Address correspondence to Y. Esterson (yonah.esterson@gmail.com)

Background Information: Hypertrophic cardiomyopathy (HCM) is a disorder of the myocardium caused by a mutation in one of the sarcomere genes. HCM is the most common cause of sudden cardiac death in young athletes and may also cause such symptoms as dyspnea and syncope. Although this disease is inherited in an autosomal dominant pattern and is generally characterized by left ventricular hypertrophy, there is substantial phenotypic variability secondary to the variable expressivity and penetrance of the causal mutations. Given the severity of this disease’s potential outcomes, diagnosis and understanding anatomic variation is key.

Educational Goals/Teaching Points: Our aim is to review the morphologic variations in hypertrophic cardiomyopathy seen on cardiac MR, cardiac CT, and echocardiographic imaging, including variations in wall thickness, mitral apparatus variations, and other left ventricular changes (i.e., bands and trabeculations), and to explain their clinical consequences. We explain the pathophysiology and clinical significance of hypertrophic cardiomyopathy and review the anatomy, physiology, and multimodality imaging appearance of the structurally normal heart (myocardial wall thickness and valve apparatus).

Key Anatomic/Physiologic Issues and Imaging Findings/Techniques: We present the myocardium and mitral valve, including anatomy, physiology, and multimodality imaging appearance; hypertrophic cardiomyopathy, including anatomy, pathophysiology, symptomatology, physical examination findings, and diagnostic criteria; morphologic variations in hypertrophic cardiomyopathy, including myocardial variants, mitral apparatus variants, and left ventricular bands/trabeculations. Clinical consequences of variant multimodality imaging findings in hypertrophic cardiomyopathy are shown.

Conclusion: In this presentation, we provide an overview of hypertrophic cardiomyopathy with a focus on the spectrum of morphologic variations associated with this disease as seen on cardiac MRI, cardiac CT, and echocardiography.