Abstract

BOSIO, Martín et al. Hipertensión arterial pulmonar asociada a enfermedades del tejido conectivo. Rev. amer. med. respiratoria [online]. 2013, vol.13, n.2, pp.84-94. ISSN 1852-236X.

The classification of pulmonary arterial hypertension (PAH) and the knowledge on its physiopathology have evolved over the years. The present definition of pulmonary arterial hypertension is when the tension at rest, measured through right heart catheterization, is 25 or more mm Hg. The pulmonary arterial hypertension includes a heterogeneous group of diseases such as the idiopathic PAH and the PAH associated to connective tissue diseases. Although the PAH associated to connective tissue diseases belongs to the same group of idiopathic PAH and both have a similar prevalence, there are relevant clinical and epidemiological differences. The objective of this paper is to describe the epidemiological, physiopathologic and clinical characteristics and the treatment of the PAH associated to connective tissue diseases.

Keywords : Pulmonary arterial hypertension; Diseases of the connective tissue.

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