Features and Outcomes of Histologically Proven Myocarditis With Fulminant Presentation

Abstract

BACKGROUND: Fulminant myocarditis presentation (FMP) is a rare and severe presentation of myocarditis. The natural history of FMP and its clinical features associated with poor outcomes are incompletely understood because there is a lack of generalizable evidence. METHODS: This multicenter retrospective cohort study included patients hospitalized with histologically proven myocarditis who underwent catecholamine or mechanical support from 235 cardiovascular training hospitals across Japan between April 2012 and March 2017. Clinical features and the prognostic predictors of death or heart transplantation within 90 days on the basis of clinical and pathologic findings were determined using the Kaplan-Meier method, log-rank test, and Cox regression analysis. RESULTS: This study included 344 patients with histologically proven FMP (median age, 54 years; 40% female). The median follow-up was 600 days (interquartile range, 36 to 1599 days) and the cumulative risk of death or heart transplantation at 90 days was 29% (n=98). Results from multivariable Cox regression analysis showed that older age, nonsinus rhythm, low left ventricular wall motion (<40%) on admission, and ventricular tachycardia or fibrillation on admission day were associated with worse 90-day survival. Severe histologic damage (damaged cardiomyocytes comprising ≥50% of the total cardiomyocytes) was associated with a worse 90-day prognosis in patients with lymphocytic myocarditis. CONCLUSIONS: The results from analyses of data from this multicenter registry demonstrated that patients with FMP are at a higher risk of death or heart transplantation in real-world settings. These observations inform which clinical and pathologic findings may be useful for prognostication in FMP. REGISTRATION: URL: https://www.umin.ac.jp/ctr ; Unique identifier: UMIN000039763.

Reevaluation of Intravenous Steroid Therapy for Histologically Confirmed Myocarditis with Fulminant Presentation: Insights from the Japanese Registry of Fulminant Myocarditis

“…In a previous study using the same registry, factors such as older age, non-sinus rhythm, low LVEF (<40%) upon admission, and presence of ventricular tachycardia or fibrillation on the day of admission were identified as predictors of reduced 90-day survival. 12 In the current study, we compared outcomes between IS(+) and IS(-) groups, stratified by the number of previously identified risk factors. Although minimal differences in 90-day mortality rates were observed between IS(+) and IS(-) groups among patients with 2, 3, and 4 or more risk factors, the 90-day mortality rate was notably higher in the IS(+) group than in the IS(-) group among patients with 0 or 1 factors (i.e.…”

“…The Japanese Registry of Fulminant Myocarditis is a multicenter, retrospective cohort study conducted in collaboration with 235 cardiovascular training hospitals in Japan, as described in a previous report. 12…”

“…Based on a previous study using the same registry, older age (≥55 years), non-sinus rhythm, low left ventricular ejection fraction (LVEF; <40%) on admission, and ventricular tachycardia or fibrillation on the day of admission were considered as prognostic factors. 12…”

“…We compared patient characteristics between IS(+) and IS(-) groups in accordance with previous studies. 12 To control for potential confounders, we conducted propensity score matching (PSM), in both the entire and LM populations. PSM was performed using a greedy matching algorithm with a multivariate logistic regression model that included all baseline variables.…”

“…10,11 Given the above-mentioned limited data, this multicenter cohort study aimed to determine the prognostic efficacy of intravenous steroids (IS) in patients with FMP using data from the Japanese Registry of Fulminant Myocarditis. 12…”