Natural history and prognostic risk factors in endocardial fibroelastosis

A retrospective study was made of 52 patients (16 men, 36 women) with endocardial fibroelastosis diagnosed by strict clinical criteria and confirmed histologically in 18 (35%). Clinical and hemodynamic manifestations at presentation were reviewed from the clinical record. The follow-up period averaged 47 months (range 1 day to 228 months). Actuarial survival rates were 93% at 6 months, 83% at 1 year and 77% at 4 years. Clinical and hemodynamic manifestation included onset at less than 1 year of age (89%), respiratory distress (71%), cardiomegaly on chest roentgenogram (99%), left ventricular hypertrophy with ST-T-wave changes on the electrocardiogram (97%) and reduced contractility with dilatation of the left ventricle (100%). Prognostic risk factors were evaluated comparing 13 patients who died (group 1) with 16 patients who survived greater than 4 years (group 2). Only cardiac index (2.8 +/- 0.8 vs 3.5 +/- 0.5 liter/min/m2) and ejection fraction (18 +/- 12 vs 33 +/- 21%) measured at catheterization were significantly reduced in group 1 compared with group 2 (p less than 0.005 and p less than 0.01, respectively). Careful observation and appropriate management are recommended in all patients, although low ejection fraction and cardiac output at presentation are predictive of poor outcome and support other therapeutic alternatives.