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Congenital Heart Disease Associated Pulmonary Arterial Hypertension: Preliminary Results From a Novel Registry.
MedLine Citation:
PMID:  22212153     Owner:  NLM     Status:  Publisher    
Objectives:  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 ANZ registry in 2010 to document the incidence, demographics, presentation and outcomes for these patients. Design:  This multicentre, prospective, web-based registry enrolls patients with CHD associated PAH being followed in a tertiary centre. The inclusion criteria stipulated patient age >16 years, a measured mPAP >25mmHg at rest or echocardiographic evidence of PAH or a diagnosis of Eisenmenger syndrome, and followed since 1/1/2000. A single observer collected standardised data during a series of site visits. Results:  Of the first 50 patients enrolled, 30 (60%) are female The mean age (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. 14 (28%) patients were in WHO functional Class II and 36 (72%) in Class III at the time of diagnosis. 47 of 50 (94%) had congenital systemic-pulmonary shunts and 36 (72%) never underwent intervention. 13 (26%) had Down's syndrome. Confirmation of PAH by recent cardiac catheterization 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.
Ml Rose; G Strange; I King; S Arnup; S Vidmar; F Kermeen; L Grigg; Rg Weintraub; Ds Celermajer
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-12-29
Journal Detail:
Title:  Internal medicine journal     Volume:  -     ISSN:  1445-5994     ISO Abbreviation:  -     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2012-1-3     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101092952     Medline TA:  Intern Med J     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2011 Sydney South West Area Health Service. Journal compilation © 2011 Royal Australasian College of Physicians.
Royal Children's Hospital, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia; Department of Epidemiology and Preventative Medicine, Monash University, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Pulmonary Hypertension and Transplant Unit, The Prince Charles Hospital, Brisbane, Australia; Department of Cardiology, Royal Melbourne Hospital, Melbourne, Australia; Department of Medicine, The University of Sydney, Sydney, Australia.
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