INFLAMMATORY CARDIOMYOPATHY: The Controversy of Diagnosis and Management

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Myocarditis and dilated cardiomyopathy can occur as isolated entities or in association with systemic inflammatory diseases. The catastrophic results of a damaged myocardium include congestive heart failure, cardiogenic shock, and sudden arrhythmogenic death. Congestive heart failure remains one of the most prevalent and costly health problems in the United States. Over 3 million people in the United States are affected, with an estimated $8 billion per year spent in the treatment of heart failure.43 It has become clear that determining heart failure etiology is extraordinarily important because many diseases can be effectively treated or, at least, ameliorated, with ensuing reduction in morbidity and mortality.

Considerable controversy exists regarding lymphocytic myocarditis, including its etiology, immunology, diagnostic approach, and relationship to dilated cardiomyopathy. Often a diagnosis of myocarditis is rendered when patients present with cryptogenic heart failure and a peri-presentation “viral” syndrome. This observation raises the controversy of the relationship between inflammatory heart disease and heart failure. Etiology, diagnosis, and treatment options for this devastating illness and the surrounding debates are addressed. Lymphocytic myocarditis as an isolated disease is reviewed first, followed by myocarditis associated with systematic inflammatory illnesses.

Section snippets

Etiology and Immunology

Lymphocytic myocarditis, histologically, is characterized by infiltration of the myocardium by lymphocytes and macrophages adjacent to myocyte injury or necrosis. It is intuitive that these inflammatory cells are the culprits in causing cardiac cell injury that leads to necrosis. Clinically, left ventricular systolic function may remain normal or, more frequently, may be subclinically impaired, with subsequent development of symptoms of heart failure or arrhythmias. In acute myocarditis,

Systemic Lupus Erythematosus

Cardiac involvement has been reported in 18% to 38% of cases of systemic lupus erythematosus (SLE).31 Cardiovascular involvement is the third leading cause of death in SLE.33 Pericarditis is the most common manifestation, found in 20% to 30% of patients with lupus.33 Coronary arteritis and occlusions are rare. Libman-Sacks endocarditis is reported in 13% to 74%.3 Myocarditis is also infrequent; it is reported in less than 10% of the cases.3 Domenech et al reported that cardiomyopathy is more

CONCLUSION

In the past decade, advances in immunology have greatly added to our understanding of myocarditis. Therapy with empiric steroids has been categorically futile. Hopefully, more selective immunosuppressive therapies will yield better outcomes. Further research in basic immunology and molecular biology will likely lead to exciting future therapies targeted at the cellular level.

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    Address reprint requests to Karen B. James, MD, Cleveland Clinic Foundation, 9500 Euclid Avenue, F25, Cleveland, OH 44195

    *

    From the Department of Cardiology (KBJ, RS, JBY), and Department of Pathology (NR), Cleveland Clinic Foundation, Cleveland, Ohio

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