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September 4, 2026Circulation64 citationsOpen Access

Features and Outcomes of Histologically Proven Myocarditis With Fulminant Presentation

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KKKoshiro KanaokaKOKenji OnoueSTSatoshi Terasaki

Key Result

Among patients with histologically proven fulminant myocarditis requiring catecholamine or mechanical support, the cumulative risk of death or heart transplantation at 90 days was 29%.

Key Points

  • To determine the clinical characteristics, natural history, and prognostic predictors of 90-day death or heart transplantation in patients presenting with histologically confirmed fulminant myocarditis.
  • Multicenter retrospective cohort study of N=344 patients with histologically proven fulminant myocarditis receiving catecholamines or mechanical circulatory support across 235 Japanese hospitals (April 2012 to March 2017).
  • Evaluated 90-day cumulative incidence of death or heart transplantation and independent clinical and pathological predictors using Kaplan-Meier estimation and multivariable Cox proportional hazards regression.
  • The cumulative risk of death or heart transplantation at 90 days was 29% (n=98) across a median follow-up of 600 days (interquartile range, 36 to 1599 days).
  • Multivariable Cox regression linked older age, nonsinus rhythm, admission left ventricular wall motion <40%, and admission ventricular tachycardia or fibrillation to significantly worse 90-day survival.
  • Severe histologic damage (≥50% damaged cardiomyocytes) was significantly associated with worse 90-day prognosis in patients with lymphocytic myocarditis.

Study Design

Type

Cohort (n=344)

Multicenter

Yes

Structured PICO

P
Population
344 patients (median age 54, 40% female) hospitalized with histologically proven myocarditis requiring catecholamine or mechanical support across Japan, followed for a median of 600 days.
O
Outcome
Death or heart transplantation within 90 dayshard clinical

In patients with histologically proven fulminant myocarditis, the 90-day risk of death or heart transplantation is 29%, with older age, nonsinus rhythm, low LV wall motion, and VT/VF on admission predicting worse survival.

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.

Expert Takes1 quote

“A comprehensive assessment of both clinical and pathologic findings may help the prediction of outcome in patients with FM. Our results showed that about half of the patients with clinically diagnosed FM did not undergo endomyocardial biopsy, and increasing the implementation of biopsy may improve the management of FM.”

Koshiro Kanaoka, Researcher, Nara Medical Universityauto_pipelineSupportiveView source
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Cite This Study

Kanaoka et al. (2022) conducted a cohort in Histologically proven myocarditis with fulminant presentation (n=344). Fulminant myocarditis presentation was evaluated on death or heart transplantation within 90 days. Among patients with histologically proven fulminant myocarditis requiring catecholamine or mechanical support, the cumulative risk of death or heart transplantation at 90 days was 29%.

synapsesocial.com/papers/6a9b5700c5d8c3c656de5f80https://doi.org/10.1161/circulationaha.121.058869
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