| Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. | |
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MedLine Citation:
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PMID: 20585011 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND: Novel therapies have recently become available for pulmonary arterial hypertension. We conducted a study to characterize mortality in a multicenter prospective cohort of patients diagnosed with idiopathic, familial, or anorexigen-associated pulmonary arterial hypertension in the modern management era. METHODS AND RESULTS: Between October 2002 and October 2003, 354 consecutive adult patients with idiopathic, familial, or anorexigen-associated pulmonary arterial hypertension (56 incident and 298 prevalent cases) were prospectively enrolled. Patients were followed up for 3 years, and survival rates were analyzed. For incident cases, estimated survival (95% confidence intervals [CIs]) at 1, 2, and 3 years was 85.7% (95% CI, 76.5 to 94.9), 69.6% (95% CI, 57.6 to 81.6), and 54.9% (95% CI, 41.8 to 68.0), respectively. In a combined analysis population (incident patients and prevalent patients diagnosed within 3 years before study entry; n=190), 1-, 2-, and 3-year survival estimates were 82.9% (95% CI, 72.4 to 95.0), 67.1% (95% CI, 57.1 to 78.8), and 58.2% (95% CI, 49.0 to 69.3), respectively. Individual survival analysis identified the following as significantly and positively associated with survival: female gender, New York Heart Association functional class I/II, greater 6-minute walk distance, lower right atrial pressure, and higher cardiac output. Multivariable analysis showed that being female, having a greater 6-minute walk distance, and exhibiting higher cardiac output were jointly significantly associated with improved survival. CONCLUSIONS: In the modern management era, idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension remains a progressive, fatal disease. Mortality is most closely associated with male gender, right ventricular hemodynamic function, and exercise limitation. |
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Authors:
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Marc Humbert; Olivier Sitbon; Ari Chaouat; Michèle Bertocchi; Gilbert Habib; Virginie Gressin; Azzedine Yaïci; Emmanuel Weitzenblum; Jean-François Cordier; François Chabot; Claire Dromer; Christophe Pison; Martine Reynaud-Gaubert; Alain Haloun; Marcel Laurent; Eric Hachulla; Vincent Cottin; Bruno Degano; Xavier Jaïs; David Montani; Rogério Souza; Gérald Simonneau |
Publication Detail:
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Type: Clinical Trial; In Vitro; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't Date: 2010-06-28 |
Journal Detail:
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Title: Circulation Volume: 122 ISSN: 1524-4539 ISO Abbreviation: Circulation Publication Date: 2010 Jul |
Date Detail:
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Created Date: 2010-07-13 Completed Date: 2010-07-30 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0147763 Medline TA: Circulation Country: United States |
Other Details:
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Languages: eng Pagination: 156-63 Citation Subset: AIM; IM |
Affiliation:
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Service de Pneumologie et Réanimation Respiratoire, Faculté de Médecine, Hôpital Antoine Béclère, Assistance-Publique-Hôpitaux de Paris, Université Paris-Sud 11, 92140 Clamart, France. marc.humbert@abc.aphp.fr |
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Cardiac Output Female Follow-Up Studies Genetic Diseases, Inborn / drug therapy, mortality*, physiopathology Humans Hypertension, Pulmonary / drug therapy, mortality*, physiopathology Incidence Male Middle Aged Prevalence Prospective Studies Sex Factors Survival Rate |
| Comments/Corrections | |
Comment In:
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Circulation. 2010 Jul 13;122(2):106-8
[PMID:
20585007
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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