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.