The Belgian Eisenmenger syndrome registry: Implications for treatment strategies?

Objective - Pulmonary arterial hypertension (PAH), associated with congenital heart disease (CHD), usually results from a systemic-to-pulmonary shunt. Eisenmenger syndrome (ES) is characterised by severe irreversible PAH and reversal of a previous systemic-to-pulmonary shunt.A national registry of ES patients was initiated to optimise patient care and to provide epidemiological information regarding PAH and CHD in Belgium. Methods - All ES patients, older than 18 years, were selected through the local databases of ten centres in Belgium. After written informed consent, demographic, clinical, biochemical, technical, and treatment data were entered into the web-based registry. Results - Ninety-one patients were included in the registry. Mean age was 36 +/- 11 years (range 18-59 years). Complete atrioventricular septal defect (N = 26, 28.6%), followed by ventricular septal defect (N = 25, 27.5%) were the commonest defects. Forty-five percent were patients with Down syndrome. Down patients were younger (32 +/- 9 versus 40 +/- 12 years; P = 0.039), had worse functional capacity (class II/III ratio: 15/16 versus 21/8; P = 0.035) and received significantly less specific PAH treatment (7% versus 38%; P = 0.002). Conclusion - Through the national Eisenmenger registry, 91 adult patients with ES were identified (estimated prevalence 11 per million inhabitants). Almost half of them were Down patients. Although having worse functional capacity, significantly less Down patients were receiving specific PAH treatment.