G iant-cell myocarditis (GCM) is known as a rare, rapidly progressive, and frequently fatal myocardial disease in young and middle-aged adults. It is attributed to a T lymphocyte-mediated inflammation of the heart muscle and associates with systemic autoimmune diseases in ≈20% of cases.
1,2The most common early manifestations are heart failure, ventricular arrhythmias, and atrioventricular block, but GCM may also disguise as an acute myocardial infarction and rarely presents as an unexpected sudden cardiac death.1-3 The diagnosis of GCM rests fully on microscopy of the heart muscle and even in experienced centers >4 in 10 cases may escape detection until autopsy or cardiac transplantation. Aside from nonspecific measures to combat its symptomatic manifestations, the treatment of GCM relies on immunosuppression. Retrospective observations from the Multicenter GCM Registry 1,4 and a small prospective study with repeat biopsies 5 suggest that cyclosporine-based combined immunosuppression may be able to reduce myocardial inflammation 5 and improve clinical outcome. 1,4,5 Yet, these data are uncontrolled and suffer from lack of details about the treatments given 1,4 and the possibility of survivor bias. 1,4,5 The key problem is that the rarity and seriousness of GCM make controlled treatment trials, let alone use of a placebo arm, virtually impossible. The only such attempt, a cooperative endeavor by 17 centers, was terminated after 6 years because of difficulties in recruiting patients.5 Therefore, carefully studied observational patient series continue to add to the knowledge about GCM. We report here our experience in 32 patients with GCM, of whom 26 received combined immunosuppression. We focus on the diagnosis of GCM and on the outcome of patients with contemporary treatment. Our key observations suggest that repeat and imaging-guided biopsies increase the detection rate of GCM and that combined immunosuppression supported by therapy for heart failure and arrhythmias may result in transplant-free survival in two thirds of patients.
Clinical Perspective on p 22
Methods
PatientsFrom the year 1991 through 2011, 32 patients with histologically verified GCM were seen at the Division of Cardiology, Helsinki University Central Hospital. The majority of diagnoses (29/32) were made after year 2000, that is, during the latter half of the study period. The medical records, laboratory test, imaging studies, and available biopsy material of all patients were retrospectively reviewed and © 2012 American Heart Association, Inc. Background-Giant-cell myocarditis often escapes diagnosis until autopsy or transplantation and has defied proper treatment trials for its rarity and deadly behavior. Current therapy rests on multiple-drug immunosuppression but its prognostic influence remains poorly known. We set out to analyze (1) our experience in diagnosing giant-cell myocarditis and (2) the outcome of patients on combined immunosuppression. Methods and Results-We reviewed the histories, diagnostic procedures, details of treatment, a...