AHA Scientific Sessions 2010 edition:2010 location:Chicago, US date:14-20 November 2010
Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) usually results from a systemic-to-pulmonary shunt. Eisenmenger’s syndrome (ES) is characterised by severe irreversible PAH and reversal of a previous systemic-to-pulmonary shunt. This study aimed at evaluating predictors of adverse outcome in patients with ES.
In 2006, all ES patients, older than 18 years, were selected through the local databases of ten centres in Belgium and prospectively followed. Demographic, clinical, biochemical and treatment data were entered into a web based registry. A composite endpoint, defined as all-cause mortality, lung transplantation, hospitalisation due to cardiopulmonary causes and the need for additional pulmonary hypertensive treatment, was used as independent variable. Uni- and multivariate Cox regression analysis was performed.
A total of 58 patients were included in the registry. Twenty-five patients reached the prespecified endpoint over a median follow-up time of 3 years. According to univariate Cox regression analysis, NYHA classification (P=0.031), creatinine (P=0.006) and the use of oral anticoagulation (P=0.009) correlated with adverse outcome. Multivariate analysis showed that NYHA classification (HR 2.25; 95%CI 1.08-4.68; P=0.03), creatinine (HR 4.47; 95%CI 1.44-13.90; P=0.01) and oral anticoagulation (HR 3.45; 95%CI 1.43-8.33; P=0.006) were independent predictors of adverse outcome.
Higher NYHA classification and higher creatinine levels were predictive for adverse outcome in patients with Eisenmenger’s syndrome. To our knowledge, this is the first study indicating that the use of oral anticoagulation is also an independent predictor of adverse outcome in patients with Eisenmenger’s syndrome.