IDIOPATHIC GIANT-CELL MYOCARDITIS - NATURAL-HISTORY AND TREATMENT

Background Idiopathic giant-cell myocarditis is a rare and frequently fatal disorder. We used a multicenter data base to define the natural history of giant-cell myocarditis and the effect of treatment, Methods We identified 63 patients with idiopathic giant-cell myocarditis thro ugh journal announcements and direct mailings to cardiovascular center s worldwide. Results The patients consisted of 33 men and 30 women wit h an average age of 42.6 years; 88 percent were white, 5 percent were black, 5 percent were Southeast Asian or Indian, and 2 percent were Mi ddle Eastern. Most presented with congestive heart failure (47 patient s, or 75 percent), ventricular arrhythmia (9 patients, or 14 percent), or heart block (3 patients, or 5 percent), although in some cases the initial symptoms resembled those of acute myocardial infarction (4 pa tients). Nineteen percent had associated autoimmune disorders. The rat e of survival was worse than among 111 patients with lymphocytic myoca rditis in the Myocarditis Treatment Trial (P<0,001); among our patient s, the rate of death or cardiac transplantation was 89 percent, and me dian survival was only 5.5 months from the onset of symptoms. The 22 p atients treated with corticosteroids and cyclosporine, azathioprine, o r both therapies survived for an average of 12.3 months, as compared w ith an average of 3.0 months for the 30 patients who received no immun osuppressive therapy (P=0.001). Of the 34 patients who underwent heart transplantation, 9 (26 percent) had a giant-cell infiltrate in the tr ansplanted heart and 1 died of recurrent giant-cell myocarditis. Concl usions Giant-cell myocarditis is a disease of relatively young, predom inantly healthy adults, Patients usually die of heart failure and vent ricular arrhythmia unless cardiac transplantation is performed. Despit e the possibility of fatal disease recurrence, transplantation is the treatment of choice for most patients. (C) 1997, Massachusetts Medical Society.