Endomyocardiofibrosis in the Americas Collaborative Study: The EMF-SIAC Registry.

Autor: Scatularo CE; Department of cardiology, Sanatorio de la Trinidad de Palermo, Buenos Aires, Argentina. Electronic address: emmanuelscatularo@gmail.com., Posada Martínez EL; Department of Cardiology, Ignacio Chavez National Institute of Cardiology, Mexico City, Mexico., Alba AC; Department of Cardiology, University Health Network, Toronto, Ontario, Canada., Renedo MF; Department of Heart Failure and Thoracic Transplantation, Fundacion Favaloro, Buenos Aires, Argentina., N Llober M; Department of Cardiology, Hospital Argerich, Buenos Aires, Argentina., Elfman M; Department of Heart failure, Sanatorio Güemes, Buenos Aires, Argentina., Pérez de Arenaza D; Department of Cardiovascular Imaging, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina., Diez M; Department of Heart Failure, Pulmonary Hypertension and Heart Transplantation, Instituto Cardiovascular de Buenos Aires, Buenos Aires, Argentina., Saldarriaga C; Department of Heart Failure, CardioVID Clinic, Medellin, Antioquia, Colombia., Cingolani E; Department of electrophysiology, Cedears-Sinai Medical Center, Los Angeles, California, United States., Sosa Liprandi MI; Department of Heart failure, Sanatorio Güemes, Buenos Aires, Argentina.
Jazyk: angličtina
Zdroj: Current problems in cardiology [Curr Probl Cardiol] 2023 Dec; Vol. 48 (12), pp. 101995. Date of Electronic Publication: 2023 Jul 27.
DOI: 10.1016/j.cpcardiol.2023.101995
Abstrakt: Endomyocardiofibrosis (EMF) is a restrictive cardiomyopathy characterized by subendocardial fibrosis due to eosinophilic myocardial infiltration. EMF may commonly present with heart failure (HF) or atrial fibrillation (AF). Immunosuppression can be effective in early stages, but not in the chronic phase. Our objective was to describe the characteristics of EMF patients in the Americas. This registry is a retrospective multicenter cross-sectional study including patients ≥18 years-old with EMF diagnosed by imaging methods, according to the Mocumbi criteria. Clinical, biochemical, and imaging variables were analyzed. On the 54 patients included, 28 (52%) were male with an age of 47 years. The etiology was idiopathic in 47 (87%) patients, familial in 4 (7%), and secondary to chemotherapy in 2 (3.5%). We detected a history of HF in 41 patients (76%), AF in 19 (35%), and ischemic stroke in 8 (15%). The diagnosis was made by echocardiography in all patients, and 38% had Cardiac Resonance or Computed Tomography. Thirty-five patients (65%) presented a left ventricular ejection fraction ≥50%, 11 (21%) severe mitral regurgitation, and 18 (33%) severe tricuspid regurgitation. In 17 patients (32%) the diagnosis was confirmed by endomyocardial biopsy. Among medical therapy, 72% received beta-blockers, 63% vasodilators, 65% mineralocorticoid antagonists, 7.4% SGLT2 inhibitors, and 11% corticosteroids. Subendocardial resection was performed in 9 (16%) patients and mitral valve replacement in 11 (20%) patients. In conclusion, EMF patients had a high prevalence of HF, AF, and embolic events. The diagnosis was frequently made in an advanced stage when HF management and surgery were the only effective treatments.
Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2023 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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