Document Detail


Pulmonary arterial hypertension in connective tissue diseases.
MedLine Citation:
PMID:  22748903     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs). Approximately 10% of patients with systemic sclerosis develop PAH, the prevalence being much lower in other CTDs. However, PAH is an important contributor to morbidity and mortality in all forms of CTD. Despite similarities in presentation, hemodynamic perturbations, and pathogenesis, patients with CTD-associated PAH (CTD-PAH) usually have a poorer response to PAH-specific medications and poorer prognosis than patients with idiopathic PAH (IPAH). Select patients with CTD-PAH may be candidates for lung transplantation, but results are less favorable than for IPAH because of comorbidities and complications specifically associated with CTD.
Authors:
Stephen C Mathai; Paul M Hassoun
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Review    
Journal Detail:
Title:  Heart failure clinics     Volume:  8     ISSN:  1551-7136     ISO Abbreviation:  Heart Fail Clin     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-07-03     Completed Date:  2012-11-16     Revised Date:  2013-07-12    
Medline Journal Info:
Nlm Unique ID:  101231934     Medline TA:  Heart Fail Clin     Country:  United States    
Other Details:
Languages:  eng     Pagination:  413-25     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Elsevier Inc. All rights reserved.
Affiliation:
Pulmonary Hypertension Program, Division of Pulmonary and Critical Care Medicine, Department of Medicine, Johns Hopkins University, 1830 East Monument Street, Baltimore, MD 21205, USA.
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MeSH Terms
Descriptor/Qualifier:
Autoantibodies
Genetic Predisposition to Disease
Hemodynamics
Humans
Hypertension, Pulmonary / drug therapy,  etiology,  pathology*
Lupus Erythematosus, Systemic / complications,  pathology*
Polymorphism, Genetic
Prognosis
Protein-Tyrosine Kinases / antagonists & inhibitors
Risk Factors
Scleroderma, Systemic / complications,  pathology*
Time Factors
Grant Support
ID/Acronym/Agency:
K23 HL092287/HL/NHLBI NIH HHS; P50 HL084946/HL/NHLBI NIH HHS; P50 HL084946-05/HL/NHLBI NIH HHS
Chemical
Reg. No./Substance:
0/Autoantibodies; EC 2.7.10.1/Protein-Tyrosine Kinases
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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