EDITORIAL
Improving survival in idiopathic PAH
Improving survival in idiopathic pulmonary arterial hypertension: revisiting the "kingdom of the near-dead"
Southwest Ontario Pulmonary Hypertension Clinic, Divisions of Respirology and Cardiology, Departments of Medicine, London Health Sciences Center, and The Centre for Critical Illness Research, Lawson Health Research Institute, University of Western Ontario, London, Ontario Canada
Correspondence to:
Correspondence to:
Dr S Mehta
Division of Respirology, London Health Sciences Center - South Street Hospital, London, Ontario, Canada N6A 4G5; sanjay.mehta@lhsc.on.ca
Recent advances in the treatment of idiopathic PAH have resulted in increased survival
Keywords: pulmonary hypertension; survival analysis; epoprostenol; bosentan; endothelin
| The first 150 words of the full text of this article appear below. |
Idiopathic pulmonary arterial hypertension (PAH), formerly known as primary pulmonary hypertension, is characterised by raised pulmonary artery pressure and pulmonary vascular resistance in the absence of underlying significant cardiopulmonary or other medical disease. Idiopathic PAH is a progressive disorder that usually culminates in right ventricular failure and death. Moreover, patients with idiopathic PAH are often severely limited on exertion by dyspnoea and fatigue, and thus suffer from a poor quality of life.
Despite clinical recognition of idiopathic PAH for more than 100 years, there have been few effective therapeutic options until recently. Given the previous absence of effective pharmacological therapy, surgical removal of the disease by lung transplantation has been an important therapeutic option for patients with idiopathic PAH. Lung transplantation is associated with rapid and sustained improvement in pulmonary haemodynamics.1 Moreover, in individual patients with idiopathic PAH who do not respond to medical treatment,
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