Congenital heart disease-associated pulmonary arterial hypertension: preliminary results from a novel registry.
OBJECTIVE
Pulmonary arterial hypertension (PAH) frequently accompanies childhood congenital heart disease (CHD) and may persist into adult life. The advent of specific PAH therapies for PAH prompted formation of a national Australian and New Zealand registry in 2010 to document the incidence, demographics, presentation and outcomes for these patients.


METHODS
This multicentre, prospective, web-based registry enrols patients with CHD-associated PAH being followed in a tertiary centre. The inclusion criteria stipulated patient age ≥16 years, a measured mean pulmonary arterial pressure >25 mmHg at rest or echocardiographical evidence of PAH or a diagnosis of Eisenmenger syndrome, and followed since 1 January 2000. A single observer collected standardised data during a series of site visits.


RESULTS
Of the first 50 patients enrolled, 30 (60%) were female. The mean age (standard deviation (SD)) at the time of PAH diagnosis or confirmation in an adult centre was 27.23 (10.07) years, and 32 (64%) patients are currently aged >30 years. Fourteen (28%) patients were in World Health Organization Functional Class II and 36 (72%) in Class III at the time of diagnosis. Forty-seven of 50 (94%) had congenital systemic-pulmonary shunts, and 36 (72%) never underwent intervention. Thirteen (26%) had Down syndrome. Confirmation of PAH by recent cardiac catheterisation was available in 30 (60%) subjects. During follow up, a total of 32 (64%) patients received a PAH-specific therapy.


CONCLUSIONS
CHD associated with PAH in adult life has resulted in a new population with unique needs. This registry will allow documentation of clinical course and long-term outcomes for these patients.
Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
M L
ML
Rose
G
G
Strange
I
I
King
S
S
Arnup
S
S
Vidmar
C
C
O'Donnell
F
F
Kermeen
L
L
Grigg
R G
RG
Weintraub
D S
DS
Celermajer
D000328:Adult; D005260:Female; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D008297:Male; D011446:Prospective Studies; D012042:Registries; D055815:Young Adult