Objectives: To identify risk factors associated with mortality in adult patients with Eisenmenger syndrome and to assess the effect of left ventricular dysfunction on mortality in these patients.
Methods and results: One hundred twenty-two adult patients with Eisenmenger syndrome were retrospectively evaluated for signs and symptoms of heart failure, and underwent electrocardiography and laboratory investigations. Available echocardiograms were analyzed and left ventricular function was assessed both qualitatively and quantitatively. There were 47 deaths at 37.8 +/- 12.0 years of age. On univariate analysis clinical signs and symptoms of heart failure, right ventricular hypertrophy on electrocardiography, wide QRS (QRS duration >130 milliseconds), and left ventricular dysfunction (left ventricular ejection fraction [LVEF] <50%) on echocardiography were associated with mortality. On multivariate analysis, signs and symptoms of heart failure, right ventricular hypertrophy on electrocardiography, and LVEF <50% remained strong predictors of death.
Conclusions: Signs and symptoms of heart failure predict mortality in patients with Eisenmenger syndrome. Furthermore, patients with left ventricular dysfunction (LVEF <50%) have higher mortality. A combination of signs and symptoms of heart failure, right ventricular hypertrophy on electrocardiography, and left ventricular dysfunction on echocardiography provides the most powerful predictor of death in patients with Eisenmenger syndrome.