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Semin Respir Crit Care Med 2013; 34(05): 560-567
DOI: 10.1055/s-0033-1355439
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Idiopathic Pulmonary Arterial Hypertension

Rogerio Souza
1   Department of Pulmonary, Heart Institute, University of Sao Paulo Medical School, Sao Paulo, Brazil
,
Carlos Jardim
1   Department of Pulmonary, Heart Institute, University of Sao Paulo Medical School, Sao Paulo, Brazil
,
Marc Humbert
2   University of South Paris - Kremlin-Bicetre, Paris, France
› Author Affiliations
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Publication History

Publication Date:
13 September 2013 (online)

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Abstract

Idiopathic pulmonary arterial hypertension (IPAH), formerly called primary pulmonary hypertension, is a rare disease (incidence and prevalence rates of approximately one and six cases per million inhabitants, respectively) with different clinical phenotypes. A group of diverse conditions manifest pulmonary arterial hypertension (PAH) and share similar pathological and/or clinical findings with IPAH. By definition, IPAH is diagnosed only after alternative diagnoses have been ruled out. Extensive investigation is needed to determine if PAH is associated with thyroid diseases, infectious diseases, autoimmune conditions, exposure to certain drugs (particularly anorexigens), certain genetic mutations, and so on. The presence of genetic abnormalities and risk factors (such as specific drug exposures) reinforces the “multiple hit” concept for the development of pulmonary hypertension. Fortunately, within the past two decades, therapeutic options have become available for IPAH, resulting in improved survival and clinical outcomes. At least seven different compounds have been registered for PAH treatment. However, even with aggressive PAH-specific therapy, mortality rates remain high (∼40% at 5 years). Given the high mortality rates, the use of combinations of agents that work by different pathways has been advocated (either as “add-on” therapy or initial “up front” therapy). Further, new therapeutic agents and treatment strategies are on the near horizon, aiming to further improve survival from the remarkable progress already seen.

Keywords

idiopathic pulmonary arterial hypertension - pulmonary arterial hypertension - heritable PAH - epidemiology