Long-term follow-up and mortality predictors in endomyocardial fibrosis: a 20-year retrospective study at a quaternary hospital

Abstract Background Endomyocardial fibrosis (EMF) is a rare and severe condition characterized by fibrosis of the endocardium and myocardium, typically affecting both ventricles. Its aetiology is considered multifactorial, although the exact mechanisms remain unclear. EMF is a neglected disease, with few clinical reports and limited data on its progression and treatment. Purpose To evaluate the clinical evolution of patients diagnosed with EMF at a quaternary hospital over a 20-year follow-up period. Materials and Methods This retrospective descriptive study included patients diagnosed with EMF between 1997 and 2017 at our institution. Clinical, echocardiographic, laboratory, and magnetic resonance imaging data were analyzed, focusing on the clinical progression of the patients and the need for surgical interventions. Statistical analysis was performed using the Mann-Whitney test for continuous variables and chi-square or Fisher’s exact test for categorical variables. Survival was analyzed using Kaplan-Meier and log-rank tests. Cox regression was used to identify mortality predictors, considering p 0.07 for multivariate analysis. Statistical significance was set at p 0.05. Analyses were conducted using SPSS version 27. Results The study included 89 patients, with a mean age of 62 years, and a predominance of females (79%). The mortality rate was 57.3%, with 36.4% among those who underwent surgery. Atrial fibrillation (AF) was observed in 42% of patients. Significant factors associated with poorer prognosis included: NYHA functional class III/IV HR 4.656 (1.339 – 16.120), p = 0.016, AF HR 2.948 (1.021 – 8.514), p = 0.046, low hemoglobin HR 0.759 (0.598 – 0.964), p = 0.024, increased left atrial (LA) diameter HR 1.086 (1.036 – 1.139), p = 0.001, and increased interventricular septum (IVS) diameter HR 1.802 (1.261 – 2.575), p = 0.001. Conclusion The results indicate no significant difference between clinical and surgical treatments; however, factors such as severe functional class, AF, low hemoglobin, and increased diameters of the IVS and LA were predictors of higher mortality. These findings are crucial for identifying patients with worse prognosis in EMF and may inform future therapeutic approaches and management strategies.Figure 1 Figure 2