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Congenital heart disease‐associated pulmonary arterial hypertension: preliminary results from a novel registry

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Background/Aims:  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.

Document Type: Research Article


Affiliations: 1: Department of Cardiology, Starship Children's Hospital, Auckland, New Zealand 2: Pulmonary Hypertension and Transplant Unit, The Prince Charles Hospital, Brisbane, Australia 3: Department of Cardiology, Royal Melbourne Hospital, Melbourne

Publication date: 2012-08-01

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