Hypertrophic Cardiomyopathy Comes in Different Shapes and Sizes

Scientific Sessions 2018 marks many firsts for me—my first time at Scientific Sessions and my inaugural blog post on the AHA Early Career Voice.  Both are tremendous opportunities.

I specifically sought out the Sunday morning session, “State of the Art in Hypertrophic Cardiomyopathy.”  As an internal medicine resident at Emory, I’ve had several experiences seeing patients with hypertrophic cardiomyopathy (HCM) in the outpatient clinic.  Unlike many other fields of cardiology, HCM is a niche dominated by young, otherwise healthy patients.  The title of this session alludes to how little we know about HCM, and how the practice of managing this complex condition truly is an “Art.”

Much of the session was an exercise in taxonomizing the umbrella term, “HCM,” splitting that pie from a number of interesting angles.  Dr. Sharlene Day divided HCM by obstructive phenotype: obstruction at rest, obstruction with provocation, and no obstruction.  Our approach to therapies has been driven by a focus on relieving obstruction, but strategies for treating symptoms in the absence of obstruction represents an open frontier.  Currently, the MAVERICK-HCM trial is studying the use of a cardiac myosin modulator in this patient population.

Dr. Jodie Ingles compared “familial” versus “non-familial” HCM.  The latter case, she argued, tends to involve men, present later, and portend a lower risk of cardiovascular events.  Discerning which cases of HCM is considered “familial” versus “non-familial,” and whether such a dichotomy truly exists, sparked much debate in the Q&A.

Drs. Elizabeth McNally, Adam Helms, and Jil Tardiff shared similarly thought-provoking insight, highlighting the heterogeneity of genotypes and phenotypes in HCM.  Multiple disparate mechanisms are responsible for producing sarcomere dysfunction, subsequent organic dysfunction, and finally clinical symptoms.  An appreciation for these finer details is necessary to guide a sophisticated approach to management.


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