Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis.
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История · было → стало
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Ammirati Enrico, Palazzini Matteo, Lehtonen Jukka, Potena Luciano, Mäyränpää Mikko I, Rågback Johanna, Foà Alberto, Uribarri Aitor, Thiele Holger, Vidal-Burdeus María, Freund Anne, Gustafsson Finn, Tschöpe Carsten, Elsanhoury Ahmed, Ihle Joshua, Rudi Wolf-Stephan, Grabmaier Ulrich, Merlo Marco, Melenovský Vojtěch, Weislova Ivana, Jellinghaus Stefanie, Linke Axel, Baldovini Chiara, Adorisio Rachele, Kuchynka Petr, Paleček Tomáš, Krejčí Jan, Poloczková Hana, Caterino Anna Laura, Gilotra Nisha A, Lovell Jana P, Macomb Elaine P, Shih Jeffrey, Hong Kimberly, Rossi Valentina A, Ruschitzka Frank, Cavallini Claudio, Riccini Clara, Kamal Mohamed, Huang Florent, Groh Matthieu, Gentile Piero, Garascia Andrea, Lala Anuradha, Shimokawa Hiroaki, Vandenbriele Christophe, Sionis Alessandro, Schmidt Matthieu, Grosu Aurelia, Bollano Entela, Turco Annalisa, Crespo-Leiro Maria G, Couto-Mallon David, Cannatà Antonio, Bromage Daniel I, Narducci Maria Lucia, Cicchitti Vincenzo, Ianni Umberto, De Luca Leonardo, Mistrulli Raffaella, Frea Simone, Raineri Claudia, Schroeder Jan W, Arias Anibal Martin, Emdin Michele, Corda Marco, Pasqualucci Daniele, Greulich Simon, Gawaz Meinrad, Manuylova Tatiana, Martínez-Sellés Manuel, Hernández Pérez Francisco José, Martín Centellas Alba, Domínguez Fernando, Gaillet Antoine, D'Alessandris Nicoletta, Trankle Cory, Halushka Marc K, Moroni Francesco, Abbate Antonio, Basso Cristina, Sinagra Gianfranco, Veronese Giacomo, Camici Paolo G, Adler Eric D, Bernasconi Davide P, Klingel Karin, Cooper Leslie T
Аннотация
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.