Epidemiology and Prognosis of Myocarditis and dilated Cardiomyopathy: Predictive Value of Clinical Parameters and Biopsy Findings

  • G. William Dec
Part of the Progress in Inflammation Research book series (PIR)


The true incidence of myocarditis is difficult to assess due to the extreme diversity of its clinical manifestations. Myocarditis has been implicated in sudden death of young adults in 8–12% of cases and has been identified by endomyocardial biopsy as a cause of unexplained dilated cardiomyopathy in 10–12% of cases. Among cases of biopsy-proven myocarditis, the pathological distribution is typically lymphocytic 55%, borderline 22%, granulomatous 15%, and eosinophilic approximately 5%. Newer immunohistochemical techniques are better able to characterize specific inflammatory cellular infiltrates and have increased the sensitivity of biopsy for diagnosing myocarditis. The spectrum of viral genome detected by biopsy has shifted from Coxsackie virus B to adenovirus in the late 1990s and, more recently, to parvovirus B19 and other viruses. Clinical manifestations range from asymptomatic electrocardiographic abnormalities during viral pandemics to frank cardiogenic shock. Although endomyocardial biopsy remains the “gold standard” for establishing the diagnosis, cardiac magnetic resonance imaging with gadolinium enhancement has rapidly emerged as the screening procedure of choice.

The natural history of myocarditis varies based on its initial clinical presentation. Myocarditis masquerading as acute myocardial infarction almost always results in full recovery of cardiovascular function and rapid improvement in segmental or global wall motion abnormalities. Acute dilated cardiomyopathy is the most frequent, clinically relevant presentation. Patients with mildly impaired left ventricular function typically improve within weeks or months, while patients with more marked ventricular dilatation or more longstanding symptoms may have substantial residual cardiac dysfunction. Fulminant myocarditis appears to have an excellent, long-term prognosis with event-free survival rate exceeding 90% at 10 years if aggressive hemodynamic support is provided during the acute phase of the illness. Mortality for biopsy-verified lymphocytic myocarditis averages 20% at 1 year and over 50% at 4 years. The survival rates are similar to observational data for patients with idiopathic dilated cardiomyopathy. Survival with giant cell myocarditis is substantially lower with fewer than 20% of patients surviving 5 years. Important predictors of adverse prognosis with biopsy-proven myocarditis include giant cell histopathology, persistent viral genome on repeat endomyocardial biopsy, elevated myocardial or circulatory Fas and Fas ligand levels, extent of left ventricular enlargement and sphericity, pulmonary hypertension and bundle branch block. Treatment remains supportive as no specific treatment for biopsy-proven myocarditis has yet been shown to be effective in randomized controlled trails.


Dilate Cardiomyopathy Endomyocardial Biopsy Idiopathic Dilate Cardiomyopathy Acute Myocarditis Initial Clinical Presentation 


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Copyright information

© Springer Basel 2010

Authors and Affiliations

  • G. William Dec
    • 1
  1. 1.Cardiology Division, Massachusetts General HospitalHeart Failure and Transplantation UnitBostonUSA

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