Humbert M, Sitbon O, Simonneau G. Related Articles, Links
Treatment of pulmonary arterial hypertension.
N Engl J Med. 2004 Sep 30;351(14):1425-36. Review. No abstract
available. PMID: 15459304
Related articles: Medline Risk
factors for pulmonary arterial hypertension.*
Humbert M, Nunes H, Sitbon O, Parent F, Herve P, Simonneau G.
Clin Chest Med. 2001 Sep;22(3):459-75.
Service de Pneumologie et Reanimation Respiratoire, Centre des Maladies Vasculaires Pulmonaires, Hopital Antoine Beclere, Universite Paris-Sud, Clamart, France.
The present limitations in knowledge of the potential risk factors for PPH undoubtedly are attributable to the facts that PPH is a rare disease with an unknown pathogenesis and lacking
large case series. Moreover, definite epidemiologic data are rare and ideally should be obtained from epidemiologic surveys such as large case-control studies. The increased incidence of
the disease in young women, the familial cases, the association with autoimmune disorders, and the recent discovery that mutation of the PPH1 gene may not be restricted to familial PPH support
the hypothesis that the development of pulmonary hypertension likely implies an individual susceptibility or predisposition, which is probably genetically determined. It is also now commonly
believed that the development of pulmonary hypertension in some of these predisposed individuals could be hastened or precipitated by various expression factors (some of them yet unrecognized),
such as ingestion of certain drugs or diets, portal hypertension, or HIV infection.
PMID: 11590841
Medline Clinical classification
of pulmonary hypertension.
Simonneau G, Galie N, Rubin LJ, Langleben D, Seeger W, Domenighetti G, Gibbs S, Lebrec D,
Speich R, Beghetti M, Rich S, Fishman A.
J Am Coll Cardiol. 2004 Jun 16;43(12 Suppl S):5S-12S.
Department of Pulmonary and Critical Medicine, University of Paris Sud, Paris, France.
In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to
individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and
widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug
Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain
the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary pulmonary hypertension" and
to replace it with "idiopathic pulmonary hypertension"; to reclassify pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis; to update risk factors and associated
conditions for pulmonary arterial hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-pulmonary shunts.
PMID: 15194173
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