| Congenital Heart Disease Associated Pulmonary Arterial Hypertension: Preliminary Results From a Novel Registry. | |
| | |
MedLine Citation:
|
PMID: 22212153 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
|
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. |
| | |
Authors:
|
Ml Rose; G Strange; I King; S Arnup; S Vidmar; F Kermeen; L Grigg; Rg Weintraub; Ds Celermajer |
Related Documents
:
|
21418543 - Erythrophagocytosis by neutrophils - a rare morphological phenomenon resulting in acqui... 22362283 - Subarachnoid hemorrhage complicated with neurogenic pulmonary edema and takotsubo-like ... 11710863 - Botulinum toxin a for mentalis muscle dysfunction. |
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. |
Affiliation:
|
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. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
|
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Modelling the effect of temperature on the range expansion of species by reaction-diffusion equation...
Next Document: Mixed phenotype acute leukemia of T/myeloid type with a prominent cellular heterogeneity and unique ...