Article
作者: Riccini, Clara ; Adler, Eric D. ; Hernández Pérez, Francisco José ; Paleček, Tomáš ; Veronese, Giacomo ; Groh, Matthieu ; Vandenbriele, Christophe ; Gawaz, Meinrad ; Palazzini, Matteo ; Camici, Paolo G. ; Trankle, Cory ; Potena, Luciano ; Baldovini, Chiara ; Arias, Anibal Martin ; Rudi, Wolf-Stephan ; Linke, Axel ; Gilotra, Nisha A. ; Moroni, Francesco ; Elsanhoury, Ahmed ; Gentile, Piero ; Thiele, Holger ; Couto-Mallon, David ; Turco, Annalisa ; Klingel, Karin ; Freund, Anne ; Grosu, Aurelia ; Ruschitzka, Frank ; Emdin, Michele ; Sionis, Alessandro ; Jellinghaus, Stefanie ; Mäyränpää, Mikko I. ; Uribarri, Aitor ; Abbate, Antonio ; D’Alessandris, Nicoletta ; Gustafsson, Finn ; Bromage, Daniel I. ; De Luca, Leonardo ; Domínguez, Fernando ; Schroeder, Jan W. ; Huang, Florent ; Schmidt, Matthieu ; Lehtonen, Jukka ; Corda, Marco ; Merlo, Marco ; Cannatà, Antonio ; Adorisio, Rachele ; Lala, Anuradha ; Narducci, Maria Lucia ; Rossi, Valentina A. ; Kuchynka, Petr ; Bernasconi, Davide P. ; Shih, Jeffrey ; Poloczková, Hana ; Bollano, Entela ; Weislova, Ivana ; Martín Centellas, Alba ; Basso, Cristina ; Pasqualucci, Daniele ; Martínez-Sellés, Manuel ; Caterino, Anna Laura ; Manuylova, Tatiana ; Kamal, Mohamed ; Halushka, Marc K. ; Raineri, Claudia ; Grabmaier, Ulrich ; Cavallini, Claudio ; Cicchitti, Vincenzo ; Garascia, Andrea ; Mistrulli, Raffaella ; Crespo-Leiro, Maria G. ; Ammirati, Enrico ; Tschöpe, Carsten ; Gaillet, Antoine ; Frea, Simone ; Ianni, Umberto ; Cooper, Leslie T. ; Foà, Alberto ; Krejčí, Jan ; Macomb, Elaine P. ; Hong, Kimberly ; Melenovský, Vojtěch ; Lovell, Jana P. ; Shimokawa, Hiroaki ; Vidal-Burdeus, María ; Ihle, Joshua ; Sinagra, Gianfranco ; Greulich, Simon ; Rågback, Johanna
BACKGROUND::No large registries of patients with acute eosinophilic myocarditis (EM) are available. However, EM is perceived as a cardiac disease with high mortality, affecting mainly young and middle-aged adults according to small series and case reports. Awareness of the clinical presentation, associated systemic conditions, treatments, and outcomes of this uncommon condition is an unmet need.
METHODS::In this international, multicenter, retrospective cohort study, 53 centers screened 193 patients with histologically proven acute EM between 1992 and 2023. After the exclusion of patients with insufficient data (n=10), symptoms lasting >30 days (n=19), or histological diagnosis not confirmed after review (n=8), 156 patients were included.
RESULTS::
Median age at presentation was 48 years (first to third quartile, 34–59 years) with male predominance (67.3%), and only 2 were pediatric cases (≤16 years of age; 1.3%). The main signs and symptoms at presentation were dyspnea (75.6%), fever (61.3%), and chest pain (53.2%). Unexpectedly, peripheral eosinophilia was reported in only 57.4% of cases, with a median cell count of 630 eosinophils/μL. The median left ventricular ejection fraction at presentation was 32% (first to third quartile, 25%–48%). The disorders most frequently associated with EM were eosinophilic granulomatosis with polyangiitis (22.4% of cases) and hypersensitivity forms (14.1%). Idiopathic/undefined forms accounted for 44.9% of cases, and miscellaneous causes accounted for 18.6%. In-hospital death or need for heart transplantation (HTx) occurred in 23 patients (14.7%; 22 deaths and 1 HTx), despite 43.6% being treated with temporary mechanical circulatory support and 92.9% being treated with immunosuppressive agents. Estimated rates of death or HTx at 1 and 3 years were 19.0% and 23.8%. Increased age, decreased left ventricular ejection fraction on admission, and no immunosuppressive therapy during hospitalization were independent predictors of death or HTx. A nonsignificant higher occurrence of deaths or HTx was observed in the hypersensitivity form (46.1%) compared with the eosinophilic granulomatosis with polyangiitis–associated form (13.1%) at 3 years (
P
=0.15).
CONCLUSIONS::Acute EM can often present without peripheral eosinophilia, and rates of in-hospital and midterm mortality or HTx are high. Endomyocardial biopsy is required to reach the final diagnosis of EM because relying on peripheral eosinophilia can lead to missing diagnosis. In-hospital immunosuppression is associated with HTx-free survival, although tailored immunosuppressive therapies are needed to improve outcomes.
REGISTRATION::
URL:
https://www.clinicaltrials.gov
; Unique identifier: NCT06447935.