ABSTRACT
Pulmonary arterial hypertension (PAH) is a progressive arteriopathy of the pulmonary
circulation that can affect a wide group of patients. Presentation and natural history
of PAH are intimately linked to progressive right ventricular failure. Historically,
survival in PAH has been poor, especially for patients with PAH associated with progressive
systemic sclerosis and human immunodeficiency virus. Oral, subcutaneous, and intravenous
therapies that have emerged during the last decade can improve symptoms and hemodynamics.
This review explores the impact of these therapies on prognosis in PAH, which ultimately
rests on the ability to reverse the pulmonary arteriopathy and preserve right ventricular
function.
KEYWORDS
Pulmonary arterial hypertension - prognosis - epoprostenol
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Murali M ChakinalaM.D.
Department of Medicine, Washington University School of Medicine
660 South Euclid Ave., #8052
St. Louis, MO 63110
eMail: chakinalam@wustl.edu