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Abstract

A 46-year-old woman presented to the hospital with subacute dyspnea on exertion and syncope. She had three syncopal episodes in the months prior, all of which occurred after standing from seated position and were associated with complete loss of consciousness. Echocardiogram showed severe concentric left ventricular hypertrophy (LVH) with preserved systolic function (Panels A and B). Cardiac magnetic resonance (CMR) imaging was performed which confirmed asymmetric septal hypertrophy measuring 28-mm in thickness (Panel C, arrow). Patchy, late gadolinium enhancement was also seen within the myocardium (Panel D, arrows). A diagnosis of hypertrophic cardiomyopathy (HCM) was made. The patient had no family history of HCM or sudden cardiac death (SCD), but did report her mother and maternal aunt both had heart murmurs. Telemetry during hospitalization showed several runs of asymptomatic, non-sustained ventricular tachycardia (NSVT). An implantable cardioverterdefibrillator was placed and the patient was discharged in stable condition. Three days later, the patient was brought in by ambulance in cardiac arrest and passed away shortly after several rounds of cardiopulmonary resuscitation. This case highlights some of the risk factors associated with increased risk of SCD in patients with HCM which include younger age of diagnosis, recent syncope, massive LVH, presence of NSVT, and evidence of late gadolinium enhancement on CMR.

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