European Heart Journal Advance Access originally published online on May 5, 2007
European Heart Journal 2007 28(13):1663-1664; doi:10.1093/eurheartj/ehm135
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Myocarditis in hypertrophic cardiomyopathy
Hypertrophic Cardiomyopathy Center
Minneapolis Heart Institute Foundation
920 E. 28th Street, Suite 60
Minneapolis
MN 55407
USA
University of Padua Medical School
Padua
Italy
Tel: +1 612 863 3996, Fax: +1 612 863 3875, E-mail address: hcm.maron{at}mhif.org
We read with considerable interest the paper by Frustaci et al. We only wish to point out to the readership that these authors have reported a group of patients with hypertrophic cardiomyopathy (HCM) who differ distinctly in clinical profile from all other cohorts of patients with this disease reported anywhere in the world, including Italya country with an intense interest in HCM over many years. We refer specifically to the fact that Frustaci et al. have described 119 patients they regard as having HCM in whom fully 35% required emergency admission to the hospital for acute and profound heart failure (average age, 39; due to myocarditis in two-thirds of the cases based on endomyocardial biopsies), and also 20% who experienced sustained episodes of ventricular tachycardia. Both these disease expressions, so common in the experience of Frustaci et al., are in fact so rare within the broad HCM disease spectrum as to be virtually unheard of by most investigators.1,2 Also, the occurrence of the end-stage of HCM at 20% is fully 10 times greater than that reported by other investigators.3 It is particularly interesting that after almost 50 years of intense study, and reports consisting of literally thousands of HCM patients from around the world, a dramatic subset such as this would suddenly emerge at this time in a confined geographical area. It would be of interest if the authors would explain how and why this circumstance may have come about.
For these reasons, we must sound a note of skepticism regarding the identity of the disease that Frustaci et al. have reported, so tightly linked with acute myocarditis.
References
- Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, Shah PM, Spencer WH, Spirito P, ten Cate FJ, Wigle ED. American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy. A report of the American College of Cardiology Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines Committee to Develop an Expert Consensus Document on Hypertrophic Cardiomyopathy. J Am Coll Cardiol (2003) 42:16871713. Eur Heart J 2003;24:19651991.
[Free Full Text] - Maron BJ. Hypertrophic cardiomyopathy: a systematic review. JAMA (2002) 287:13081320.
[Abstract/Free Full Text] - Harris KM, Spirito P, Maron MS, Zenovich AG, Formisano F, Lesser JR, Mackey-Bojack S, Manning WJ, Udelson JE, Maron BJ. Prevalence, clinical profile and significance of left ventricular remodeling in the end-stage phase of hypertrophic cardiomyopathy. Circulation (2006) 114:216225.
[Abstract/Free Full Text]
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