Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis
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AMMIRATI, Enrico; Matteo PALAZZINI; Jukka LEHTONEN; Luciano POTENA; Mikko I MAYRANPAA; Johanna RAGBACK; Alberto FOA; Aitor URIBARRI; Holger THIELE; Maria VIDAL-BURDEUS; Anne FREUND; Finn GUSTAFSSON; Carsten TSCHOPE; Ahmed ELSANHOURY; Joshua IHLE; Wolf-Stephan RUDI; Ulrich GRABMAIER; Marco MERLO; Vojtech MELENOVSKY; Ivana WEISLOVA; Stefanie JELLINGHAUS; Axel LINKE; Chiara BALDOVINI; Rachele ADORISIO; Petr KUCHYNKA; Tomas PALECEK; Jan KREJČÍ; Hana POLOCZKOVÁ; Anna Laura CATERINO; Nisha A GILOTRA; Jana P LOVELL; Elaine P MACOMB; Jeffrey SHIH; Kimberly HONG; Valentina A ROSSI; Frank RUSCHITZKA; Claudio CAVALLINI; Clara RICCINI; Mohamed KAMAL; Florent HUANG; Matthieu GROH; Piero GENTILE; Andrea GARASCIA; Anuradha LALA; Hiroaki SHIMOKAWA; Christophe VANDENBRIELE; Alessandro SIONIS; Matthieu SCHMIDT; Aurelia GROSU; Entela BOLLANO; Annalisa TURCO; Maria G CRESPO-LEIRO; David COUTO-MALLON; Antonio CANNATA; Daniel I BROMAGE; Maria Lucia NARDUCCI; Vincenzo CICCHITTI; Umberto IANNI; De Luca LEONARDO; Raffaella MISTRULLI; Simone FREA; Claudia RAINERI; Jan W SCHROEDER; Anibal Martin ARIAS; Michele EMDIN; Marco CORDA; Daniele PASQUALUCCI; Simon GREULICH; Meinrad GAWAZ; Tatiana MANUYLOVA; Manuel MARTINEZ-SELLES; Hernandez Perez Francisco JOSE; Martin Centellas ALBA; Fernando DOMINGUEZ; Antoine GAILLET; Nicoletta D'ALESSANDRIS; Cory TRANKLE; Marc K HALUSHKA; Francesco MORONI; Antonio ABBATE; Cristina BASSO; Gianfranco SINAGRA; Giacomo VERONESE; Paolo G CAMICI; Eric D ADLER; Davide P BERNASCONI; Karin KLINGEL a Leslie T COOPER
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Circulation, Philadelphia, Lippincott Williams Wilkins, 2026, 0009-7322
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 (quartile 1-3, 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/mu L. The median left ventricular ejection fraction at presentation was 32% (quartile 1-3, 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 92.3% 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.
AMMIRATI, Enrico; Matteo PALAZZINI; Jukka LEHTONEN; Luciano POTENA; Mikko I MAYRANPAA; Johanna RAGBACK; Alberto FOA; Aitor URIBARRI; Holger THIELE; Maria VIDAL-BURDEUS; Anne FREUND; Finn GUSTAFSSON; Carsten TSCHOPE; Ahmed ELSANHOURY; Joshua IHLE; Wolf-Stephan RUDI; Ulrich GRABMAIER; Marco MERLO; Vojtech MELENOVSKY; Ivana WEISLOVA; Stefanie JELLINGHAUS; Axel LINKE; Chiara BALDOVINI; Rachele ADORISIO; Petr KUCHYNKA; Tomas PALECEK; Jan KREJČÍ; Hana POLOCZKOVÁ; Anna Laura CATERINO; Nisha A GILOTRA; Jana P LOVELL; Elaine P MACOMB; Jeffrey SHIH; Kimberly HONG; Valentina A ROSSI; Frank RUSCHITZKA; Claudio CAVALLINI; Clara RICCINI; Mohamed KAMAL; Florent HUANG; Matthieu GROH; Piero GENTILE; Andrea GARASCIA; Anuradha LALA; Hiroaki SHIMOKAWA; Christophe VANDENBRIELE; Alessandro SIONIS; Matthieu SCHMIDT; Aurelia GROSU; Entela BOLLANO; Annalisa TURCO; Maria G CRESPO-LEIRO; David COUTO-MALLON; Antonio CANNATA; Daniel I BROMAGE; Maria Lucia NARDUCCI; Vincenzo CICCHITTI; Umberto IANNI; De Luca LEONARDO; Raffaella MISTRULLI; Simone FREA; Claudia RAINERI; Jan W SCHROEDER; Anibal Martin ARIAS; Michele EMDIN; Marco CORDA; Daniele PASQUALUCCI; Simon GREULICH; Meinrad GAWAZ; Tatiana MANUYLOVA; Manuel MARTINEZ-SELLES; Hernandez Perez Francisco JOSE; Martin Centellas ALBA; Fernando DOMINGUEZ; Antoine GAILLET; Nicoletta D'ALESSANDRIS; Cory TRANKLE; Marc K HALUSHKA; Francesco MORONI; Antonio ABBATE; Cristina BASSO; Gianfranco SINAGRA; Giacomo VERONESE; Paolo G CAMICI; Eric D ADLER; Davide P BERNASCONI; Karin KLINGEL a Leslie T COOPER. Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis. Circulation. Philadelphia: Lippincott Williams Wilkins, 2026, roč. 153, č. 9, s. 634-652. ISSN 0009-7322. Dostupné z: https://doi.org/10.1161/CIRCULATIONAHA.125.074797.
@article{2570617, author = {Ammirati, Enrico and Palazzini, Matteo and Lehtonen, Jukka and Potena, Luciano and Mayranpaa, Mikko I and Ragback, Johanna and Foa, Alberto and Uribarri, Aitor and Thiele, Holger and VidalandBurdeus, Maria and Freund, Anne and Gustafsson, Finn and Tschope, Carsten and Elsanhoury, Ahmed and Ihle, Joshua and Rudi, WolfandStephan and Grabmaier, Ulrich and Merlo, Marco and Melenovsky, Vojtech and Weislova, Ivana and Jellinghaus, Stefanie and Linke, Axel and Baldovini, Chiara and Adorisio, Rachele and Kuchynka, Petr and Palecek, Tomas and Krejčí, Jan and Poloczková, Hana and Caterino, Anna Laura and Gilotra, Nisha A and Lovell, Jana P and Macomb, Elaine P and Shih, Jeffrey and Hong, Kimberly and Rossi, Valentina A and Ruschitzka, Frank and Cavallini, Claudio and Riccini, Clara and Kamal, Mohamed and Huang, Florent and Groh, Matthieu and Gentile, Piero and Garascia, Andrea and Lala, Anuradha and Shimokawa, Hiroaki and Vandenbriele, Christophe and Sionis, Alessandro and Schmidt, Matthieu and Grosu, Aurelia and Bollano, Entela and Turco, Annalisa and CrespoandLeiro, Maria G and CoutoandMallon, David and Cannata, Antonio and Bromage, Daniel I and Narducci, Maria Lucia and Cicchitti, Vincenzo and Ianni, Umberto and Leonardo, De Luca and Mistrulli, Raffaella and Frea, Simone and Raineri, Claudia and Schroeder, Jan W and Arias, Anibal Martin and Emdin, Michele and Corda, Marco and Pasqualucci, Daniele and Greulich, Simon and Gawaz, Meinrad and Manuylova, Tatiana and MartinezandSelles, Manuel and Jose, Hernandez Perez Francisco and Alba, Martin Centellas and Dominguez, Fernando and Gaillet, Antoine and D'alessandris, Nicoletta and Trankle, Cory and Halushka, Marc K and Moroni, Francesco and Abbate, Antonio and Basso, Cristina and Sinagra, Gianfranco and Veronese, Giacomo and Camici, Paolo G and Adler, Eric D and Bernasconi, Davide P and Klingel, Karin and Cooper, Leslie T}, article_location = {Philadelphia}, article_number = {9}, doi = {https://doi.org/10.1161/CIRCULATIONAHA.125.074797}, keywords = {biopsy; Churg-Strauss syndrome; eosinophilia; hypersensitivity; myocarditis; treatment outcome}, language = {eng}, issn = {0009-7322}, journal = {Circulation}, title = {Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis}, url = {https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.125.074797}, volume = {153}, year = {2026} }
TY - JOUR ID - 2570617 AU - Ammirati, Enrico - Palazzini, Matteo - Lehtonen, Jukka - Potena, Luciano - Mayranpaa, Mikko I - Ragback, Johanna - Foa, Alberto - Uribarri, Aitor - Thiele, Holger - Vidal-Burdeus, Maria - Freund, Anne - Gustafsson, Finn - Tschope, Carsten - Elsanhoury, Ahmed - Ihle, Joshua - Rudi, Wolf-Stephan - Grabmaier, Ulrich - Merlo, Marco - Melenovsky, Vojtech - Weislova, Ivana - Jellinghaus, Stefanie - Linke, Axel - Baldovini, Chiara - Adorisio, Rachele - Kuchynka, Petr - Palecek, Tomas - 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 - Cannata, Antonio - Bromage, Daniel I - Narducci, Maria Lucia - Cicchitti, Vincenzo - Ianni, Umberto - Leonardo, De Luca - Mistrulli, Raffaella - Frea, Simone - Raineri, Claudia - Schroeder, Jan W - Arias, Anibal Martin - Emdin, Michele - Corda, Marco - Pasqualucci, Daniele - Greulich, Simon - Gawaz, Meinrad - Manuylova, Tatiana - Martinez-Selles, Manuel - Jose, Hernandez Perez Francisco - Alba, Martin Centellas - Dominguez, 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 PY - 2026 TI - Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis JF - Circulation VL - 153 IS - 9 SP - 634-652 EP - 634-652 PB - Lippincott Williams Wilkins SN - 00097322 KW - biopsy KW - Churg-Strauss syndrome KW - eosinophilia KW - hypersensitivity KW - myocarditis KW - treatment outcome UR - https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.125.074797 N2 - 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 (quartile 1-3, 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/mu L. The median left ventricular ejection fraction at presentation was 32% (quartile 1-3, 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 92.3% 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. ER -
AMMIRATI, Enrico; Matteo PALAZZINI; Jukka LEHTONEN; Luciano POTENA; Mikko I MAYRANPAA; Johanna RAGBACK; Alberto FOA; Aitor URIBARRI; Holger THIELE; Maria VIDAL-BURDEUS; Anne FREUND; Finn GUSTAFSSON; Carsten TSCHOPE; Ahmed ELSANHOURY; Joshua IHLE; Wolf-Stephan RUDI; Ulrich GRABMAIER; Marco MERLO; Vojtech MELENOVSKY; Ivana WEISLOVA; Stefanie JELLINGHAUS; Axel LINKE; Chiara BALDOVINI; Rachele ADORISIO; Petr KUCHYNKA; Tomas PALECEK; Jan KREJČÍ; Hana POLOCZKOVÁ; Anna Laura CATERINO; Nisha A GILOTRA; Jana P LOVELL; Elaine P MACOMB; Jeffrey SHIH; Kimberly HONG; Valentina A ROSSI; Frank RUSCHITZKA; Claudio CAVALLINI; Clara RICCINI; Mohamed KAMAL; Florent HUANG; Matthieu GROH; Piero GENTILE; Andrea GARASCIA; Anuradha LALA; Hiroaki SHIMOKAWA; Christophe VANDENBRIELE; Alessandro SIONIS; Matthieu SCHMIDT; Aurelia GROSU; Entela BOLLANO; Annalisa TURCO; Maria G CRESPO-LEIRO; David COUTO-MALLON; Antonio CANNATA; Daniel I BROMAGE; Maria Lucia NARDUCCI; Vincenzo CICCHITTI; Umberto IANNI; De Luca LEONARDO; Raffaella MISTRULLI; Simone FREA; Claudia RAINERI; Jan W SCHROEDER; Anibal Martin ARIAS; Michele EMDIN; Marco CORDA; Daniele PASQUALUCCI; Simon GREULICH; Meinrad GAWAZ; Tatiana MANUYLOVA; Manuel MARTINEZ-SELLES; Hernandez Perez Francisco JOSE; Martin Centellas ALBA; Fernando DOMINGUEZ; Antoine GAILLET; Nicoletta D'ALESSANDRIS; Cory TRANKLE; Marc K HALUSHKA; Francesco MORONI; Antonio ABBATE; Cristina BASSO; Gianfranco SINAGRA; Giacomo VERONESE; Paolo G CAMICI; Eric D ADLER; Davide P BERNASCONI; Karin KLINGEL a Leslie T COOPER. Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis. \textit{Circulation}. Philadelphia: Lippincott Williams Wilkins, 2026, roč.~153, č.~9, s.~634-652. ISSN~0009-7322. Dostupné z: https://doi.org/10.1161/CIRCULATIONAHA.125.074797.