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Hypertrophic cardiomyopathy sudden death risk — ABIM Board MCQ

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HardCardiovascularHypertrophic cardiomyopathy sudden death riskABIM Board

A 34-year-old man with hypertrophic cardiomyopathy has recurrent presyncope. His father died suddenly at age 39. Cardiac MRI shows maximal septal thickness 31 mm with patchy late gadolinium enhancement. Ambulatory monitoring shows nonsustained ventricular tachycardia. Which of the following would you recommend?

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Correct answer: A — Implantable cardioverter-defibrillator discussion

Marked LV hypertrophy, family history of sudden death, nonsustained VT, syncope or presyncope, and extensive fibrosis increase sudden-death risk in hypertrophic cardiomyopathy. Shared decision-making about an ICD is appropriate.

Reference: 2020 AHA/ACC Hypertrophic Cardiomyopathy Guideline