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).


CONCLUSIONS
Through the national Eisenmenger registry, 91 adult patients with ES were identified (estimated prevalence II per million inhabitants). Almost half of them were Down patients. Although having worse functional capacity, significantly less Down patients were receiving specific PAH treatment.
Van de Bruaene
Alexander
A
Department of Cardiology, University Hospitals Leuven, Belgium.
Delcroix
Marion
M
Pasquet
Agnes
A
De Backer
Julie
J
De Pauw
Michel
M
Naeije
Robert
R
Vachiéry
Jean-Luc
JL
Paelinck
Bernard
B
Morissens
Marielle
M
Budts
Werner
W
D000293:Adolescent; D000328:Adult; D001530:Belgium / Q000453:epidemiology; D004314:Down Syndrome / Q000150:complications; D004541:Eisenmenger Complex / Q000453:epidemiology* / Q000628:therapy*; D006343:Heart Septal Defects; D006801:Humans; D006976:Hypertension, Pulmonary; D008875:Middle Aged; D015995:Prevalence; D012042:Registries