Endomyocardial fibrosis in adults: multimodality imaging phenotype and independent predictors of mortality

Background Endomyocardial fibrosis (EMF) is a rare restrictive cardiomyopathy characterized by endocardial fibrosis and apical obliteration. Data on its long-term evolution and prognosis remain limited. Objectives To describe the clinical profile, multimodality imaging findings, longitudinal evolution and independent predictors of all-cause mortality in adults with EMF. Methods We conducted a retrospective and single-center cohort study of consecutive adults (≥18 years) diagnosed with EMF, between 1997 and 2024. Diagnosis was based on clinical features and characteristic transthoracic echocardiography, with cardiac magnetic resonance (CMR) when available. The primary outcome was all-cause mortality. Secondary outcomes included need for surgery, perioperative mortality, development of atrial fibrillation (AF), and longitudinal changes in clinical, laboratory, and echocardiographic parameters. Survival analyses used Kaplan–Meier curves and Cox regression. Results Eighty-nine patients were included; 79.8% were women and mean age at diagnosis was 62 ± 13 years. Most had heart failure with preserved ejection fraction, left ventricular apical obliteration, left atrial enlargement and valvular dysfunction. Late gadolinium enhancement on CMR was present in 71.6%, predominantly apical. Over a mean follow-up of 8.1 years, 51 patients (57.3%) died. Mortality was higher in surgically treated patients (p = 0.004). AF developed in 42%. In multivariate analysis, independent predictors of mortality were NYHA III/IV (HR 4.66), AF (HR 2.95), lower hemoglobin (HR 0.76 per g/dL), larger left atrial diameter (HR 1.09/mm), and greater interventricular septal thickness (HR 1.80/mm). Conclusions Adult EMF showed a restrictive phenotype with high long-term mortality. Clinical status and structural remodeling—rather than surgery—were the main determinants of prognosis.