Mortality in Primary and Secondary Myocarditis

Todd C. Pulerwitz, MD; Thomas P. Cappola, MD; G. Michael Felker, MD; Joshua M. Hare, MD, FACC; Kenneth L. Baughman, MD, FACC; Edward K. Kasper, MD, FACC


Am Heart J. 2004;147(4) 

In This Article

Abstract and Introduction

Background: Lymphocytic myocarditis presents as a primary disorder or in association with a systemic disease. Whether primary and secondary myocarditis have the same prognosis is unknown.
Methods: Patients (n = 171) referred to the Johns Hopkins Cardiomyopathy service from 1984 to 1998 with newly diagnosed cardiomyopathy were observed for an average of 5.9 years after an original diagnosis of biopsy-proven myocarditis or until reaching the end point of death. Giant-cell myocarditis was excluded from this study. Myocarditis was classified as secondary when a systemic disease was present at the time of presentation; otherwise, myocarditis was classified as primary. Survival rates among patients with primary and secondary myocarditis were compared with Kaplan-Meier analysis and Cox proportional hazard models incorporating clinical variables, including baseline hemodynamics and treatment with immunosuppressive therapy.
Results: The mortality rate associated with secondary myocarditis varied substantially depending on the underlying systemic disorder. Peripartum myocarditis, when compared with idiopathic myocarditis, had a reduced mortality rate (relative hazard, 0.23 [0.06-0.98]; P <.05), which was attenuated after controlling for confounding variables (relative hazard, 0.62 [0.13-2.98]; P = .55). In contrast, human immunodeficiency virus myocarditis had a particularly poor prognosis (relative hazard, 6.70 [3.51-12.79]; P <.05), even after controlling for confounding variables. Myocarditis associated with systemic inflammatory disorders showed a trend toward increased mortality rate (relative hazard, 2.46 [0.65-9.38]; P = .19). For both primary and secondary myocarditis, advanced age and pulmonary hypertension were important clinical predictors of death.
Conclusions: The prognosis of patients with secondary myocarditis, when compared with patients with idiopathic myocarditis, seems most affected by the primary disease process.

Lymphocytic myocarditis (as opposed to giant-cell myocarditis, which is a distinct, rare, and frequently fatal disorder of relatively young, healthy adults[1]) has a variable clinical course,[2] ranging from relatively benign chronic active myocarditis[3] to fulminant heart failure requiring urgent treatment with support devices, cardiac transplantation, or both.[4,5] Although the Dallas criteria[6] provide a histologic definition of the disease, clinical information must be integrated into a strategy to predict outcome. For example, in idiopathic myocarditis, a fulminant clinical presentation was shown to have a favorable long-term prognosis,[5] whereas pulmonary hypertension is associated with a poor prognosis in patients with nonfulminant myocarditis.[7]

In addition to primary (idiopathic or post-viral) myocarditis, myocarditis also occurs in association with systemic diseases, particularly inflammatory or infectious diseases,[2] and may adversely affect patient outcome. Whether these patients have similar outcomes to patients with primary myocarditis is unknown. The purpose of this study was to determine the outcome of common secondary causes of myocarditis compared with that of idiopathic or primary myocarditis using a strategy that incorporates clinical and hemodynamic characteristics.


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