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Treatments for Pulmonary Hypertension

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Bosentan/Tracleer
Inhaled Prostacyclin (Ventavis, Iloprost)
Inhaled Nitric Oxide
Heart/Lung Transplants
Letairis/Ambrisentan
Prostacyclin (Flolan)
Riociquat
Sildenafil Citrate (Revatio, Viagra)
Stem Cell Transplant
Treprostinil (TYVASO)
Warfarin (Coumadin)

Overview

Treatments for pulmonary hypertension (PH) include bosentan (Tracleer), Letairis (Ambrisentan), lung transplants, oxygen therapy, sildenafil citrate (Revatio, Viagra), stem cell transplants, and warfarin (Coumadin).
Pulmonary arterial hypertension. Over recent years, advances in therapies, including the prostacyclins, the endothelin receptor antagonists, and the phosphodiesterase type 5 inhibitors, have resulted in an improved quality of life and outlook for patients with what is often a progressive disease. Current Problems in Cardiology, 2011 Dec.
What can be done to treat Pulmonary Arterial Hypertension? Treatment options fall into two main areas: general therapies that are used to reduce symptoms but which do not have a positive impact on the disease progression, and disease-targeted therapies that have been specifically researched in the area of Pulmonary Arterial Hypertension (PAH). Actelion Pharmaceuticals.
Inhaled Nitric Oxide
Long-term Inhaled Nitric Oxide (iNO) Plus Phosphodiesterase 5 Inhibitors for Severe Pulmonary Hypertension. Inhaled nitric oxide is a potent pulmonary vasodilator, but therapeutic experience in patients with severe pulmonary hypertension is scarce. We suggest iNO therapy alone or in combination with a phosphodiesterase type 5 inhibitor could be a therapeutic alternative for severe pulmonary hypertension. G M Pérez-Peñate. (PubMed) The Journal of Heart and Lung Transplantation Vol 27, Issue 12, Dec 2008, Pages 1326-1332.
Treprostinil (TYVASO)
The potential for inhaled treprostinil in the treatment of pulmonary arterial hypertension (PAH). PAH remains a severe, life-threatening disease in spite of the enormous progress in specific drug therapy over the last decade. Therefore, further improvement of drug therapy will be essential, with clear potential for inhaled treprostinil: a reduction of inhalation frequency and duration would markedly improve quality of life and compliance, and a longer-lasting local prostanoid effect might further enhance the efficacy of inhaled treprostinil. Tobias Gessler, Therapeutic Advances in Respiratory Disease, February 7, 2011.
FDA Approves TYVASO (Treprostinil) Inhalation Solution for the Treatment of Pulmonary Arterial Hypertension (PAH). In the TRIUMPH-1 randomized, double-blind, 12-week placebo-controlled clinical trial, patients taking TYVASO in four daily inhalation sessions achieved a 20-meter improvement in six-minute walk distance over those taking placebo. United Therapeutics plans to launch TYVASO in conjunction with its wholly-owned subsidiary, Lung Rx, Inc., in the United States at the beginning of September 2009. Medical News Today. 07/31/09.
Warfarin (Coumadin)
Effect of Warfarin on Survival in Scleroderma-associated Pulmonary Arterial Hypertension (SSc-PAH) and Idiopathic PAH. Belief Elicitation for Bayesian Priors. This study demonstrates the presence of uncertainty about the effect of warfarin, and provides justification for a clinical trial. Sindhu R. Johnson. Journal of Rheumatology. January 2011, 38 (1).
Anticoagulation in pulmonary arterial hypertension: a qualitative systematic review. Seven observational studies evaluating the effectiveness of warfarin comprising 488 patients were identified. Five studies support the effectiveness of anticoagulation therapy, whereas two do not. Eur Respir J 2006; 28:999-1004.
 
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