Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicentre study

Abstract

BACKGROUND: -Eisenmenger syndrome (ES) is associated with substantial morbidity and mortality. There is no consensus, however, on mortality risk stratification. We aimed to investigate survival and predictors of death in a large, contemporary cohort of ES patients. METHODS: -We identified in a multicentre approach adults with ES under follow-up between 2000 and 2015. We examined survival and its association with clinical, electrocardiographic, echocardiographic and laboratory parameters. RESULTS: -We studied 1098 patients (median age 34.4years, range 16.1-84.4years, 65.1% female, 31.9% with Down syndrome). The majority had a post-tricuspid defect (n=643, 58.6%), followed by patients with a complex (n=315, 28.7%) and pre-tricuspid lesion (n=, 12.7%). Over a median follow-up of 3.1years [IQR 1.4-5.9], allowing for 4361.6 patient-years observation, 278 patients died and six and six underwent transplantation. Twelve parameters emerged as significant predictors of death on univariable analysis. On multivariable Cox regression analysis only age (HR 1.41/10years, 95%CI 1.24-1.59, P<0.001), pre-tricuspid shunt (HR 1.56, 95%CI 1.02-2.39, P=0.041), oxygen saturation at rest (HR 0.53/10%, 95%CI 0.43-0.65, P<0.001), presence of sinus rhythm (HR 0.53, 95%CI 0.32-0.88, P=0.013) and presence of pericardial effusion (HR 2.41, 95%CI 1.59-3.66, P<0.001) remained significant predictors of death. CONCLUSIONS: -There is significant premature mortality amongst contemporary adults with ES. We report, herewith a multivariable mortality risk stratification model based on five simple, non-invasive predictors of death in this population.