Mortality in hypertrophic cardiomyopathy – Cardiology MCQ – Answer

Mortality in hypertrophic cardiomyopathy – Cardiology MCQ – Answer

Disease specific mortality in hypertrophic cardiomyopathy –  Correct answer: a) 0.5% per year

Hypertrophic cardiomyopathy (HCM) is the commonest among heritable heart diseases, with prevalence of about one in 500 individuals [1]. HCM is often highlighted as the leading cause of sudden death in young adults and athletes [2].

Still the absolute annual mortality in HCM is not high with availability of current treatment modalities including the implantation of cardioverter-defibrillator to prevent sudden death. It has been reported to be as low 0.5% per year by Barry J. Maron, the leading authority on hypertrophic cardiomyopathy [3]. After interrogating databases in two HCM referral centers (Minneapolis Heart Institute and Tufts Medical Center), data on 1653 patients between 1992 and 2013 were gathered. Of these 87% had survived at the end of follow up. 72% of the deaths were unrelated to HCM, the predominant cause of death being cancer in older subjects.

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  1. Maron BJ, Gardin JM, Flack JM, Gidding SS, Kurosaki TT, Bild DE. Prevalence of hypertrophic cardiomyopathy in a general population of young adults. Echocardiographic analysis of 4111 subjects in the CARDIA Study. Coronary Artery Risk Development in (Young) Adults. Circulation. 1995 Aug 15;92(4):785-9.
  2. Maron BJ, Doerer JJ, Haas TS, Tierney DM, Mueller FO. Sudden deaths in young competitive athletes: analysis of 1866 deaths in the United States, 1980-2006. Circulation. 2009 Mar 3;119(8):1085-92.
  3. Maron BJ, Rowin EJ, Casey SA, Garberich RF, Maron MS. What Do Patients With Hypertrophic Cardiomyopathy Die from? Am J Cardiol. 2016 Feb 1;117(3):434-5.