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European Heart Journal Advance Access originally published online on February 19, 2007
European Heart Journal 2007 28(6):733-740; doi:10.1093/eurheartj/ehl525
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© The European Society of Cardiology 2007. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Myocarditis in hypertrophic cardiomyopathy patients presenting acute clinical deterioration

Andrea Frustaci1,2,*, Romina Verardo2, Marina Caldarulo2, Maria Cristina Acconcia1, Matteo A. Russo3,4 and Cristina Chimenti1,2,4

1 Heart and Great Vessels ‘Attilio Reale’ Department, La Sapienza University, viale del Policlinico 155, 00100 Rome, Italy
2 ‘Molecular and Cellular Cardiology’ Laboratory, National Institute for Infectious Disease ‘L. Spallanzani’, Rome, Italy
3 Pathology and Experimental Medicine Department, La Sapienza University, Rome, Italy
4 IRCCS San Raffaele Pisana, Rome, Italy

Received 8 September 2006; revised 5 January 2007; accepted 18 January 2007; online publish-ahead-of-print 19 February 2007.

* Corresponding author. Tel: +39 06 55170575; fax: +39 06 55170577. E-mail address: biocard{at}inmi.it

See page 661 for the editorial comment on this article (doi:10.1093/eurheartj/ehm008)

Aim We sought to determine whether myocarditis can be a major cause of acute electrical instability or clinical deterioration in HCM patients.

Methods and results A total of 119 HCM patients (69 M/50F, mean age 41 ± 8), 42 with acute clinical deterioration and 77 clinically stable, underwent cardiac catheterization with left ventricular endomyocardial biopsy and gene analysis of major sarcomeric proteins. Endomyocardial tissue was processed for histology, immunohistochemistry, and polymerase chain reaction for the most common cardiotropic viruses. Controls were surgical samples from 50 patients with mitral stenosis. All 119 patients showed histological findings suggestive of HCM. In addition, CD45RO+ lymphocytes (≥14/mm2) with focal necrosis of the adjacent severely hypertrophied and often disorganized myocytes, consistent with an overlapping active myocarditis, were observed in 28 of 42 unstable and none of 77 stable HCM patients. A viral genome was detected in 14 of 28 patients with myocarditis and in none of HCM patients without and in none of controls. No correlation between sarcomeric protein gene mutations and HCM clinical profile was observed.

Conclusion Myocarditis, often viral, represents a common cause of acute clinical deterioration in HCM. Its recognition can potentially affect disease prognosis and treatment.

Key Words: Hypertrophic cardiomyopathy • Myocarditis • Viruses • Arrhythmia • Heart failure


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