Abstract 14774: Improved Prognosis Of Giant Cell Myocarditis
Background: Idiopathic giant cell myocarditis (GCM) is a rare form of inflammatory heart disease. Prior reports have shown a life expectancy less than 13 months from the time of diagnosis without a cardiac transplant.
Aims: To assess the natural history and prognosis of GCM in the era of combined immunosuppressive medication and intracardiac defibrillator (ICD) therapy.
Methods: A retrospective review of the 32 patients (age 48 ± 11, 22 female) diagnosed with histologically verified GCM in Finland between 1991-2011.
Results: The presenting symptoms of GCM were congestive heart failure in 10 (31 %) patients, distal AV block in 10 (31 %), ventricular tachycardia (VT) in 7 (22 %), sudden death in 1 (3 %) and symptoms consistent with acute myocardial infarction (chest pain and ECG findings) in 4 (13%). GCM was dianosed from endomyocardial biopsies (EMB) in 25 of the 32 cases. The remaining diagnoses were made at autopsy (5/32) or from explanted hearts (2/32). The majority of patients diagnosed at EMB (19/25) were given triple therapy with corticosteroid, azathioprine and cyclosporine and the rest (6/25) received two immunosuppressants. Intracardiac defibrillators (ICD) were implanted in 17/25 patients (68%). No patient receiving targeted GCM-therapy died but 8 (32%) underwent transplantation during a follow-up ranging from 1 to 89 months (mean, 32 months). 17 (68%) patients experienced sustained VTs during follow-up. One patient had recurrence of GCM in transplanted heart after 4.8 years.
Conclusions: Aggressive immunosuppression may stabilize the disease course in most patients with GCM diagnosed during the native heart's lifetime. Sustained VTs remain a problem, however, and ICDs are frequently needed. In our experience, one third of patients do not respond to the current triple-drug immunosupression and need an early transplantation.
- Advancing heart failure
- Immunosuppressive therapy
- Ventricular arrhythmia
- Implantable cardioconvert defibrillator
- © 2011 by American Heart Association, Inc.