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LUNG (PULMONARY): MAIN MENU
Lung (Pulmonary) Overview
Aspiration Pneumonia
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Pulmonary Hypertension: MAIN MENU
What is PH?
Primary PH
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PH Disease Correlations
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Treatments for Pulmonary Hypertension
Bosentan/Tracleer
Endothelin Receptors (Sitaxsentan/Ambrisentan)
Inhaled Prostacyclin (Ventavis, Iloprost)
Inhaled Nitric Oxide
Heart/Lung Transplants
Prostacyclin (Flolan, Epoprostenol) Warning
Sildenafil Citrate (Revatio, Viagra)
Stem Cell Transplant
Treprostinil (TYVASO)
Warfarin (Coumadin)
Overview
New Approaches May Help Tame Scleroderma. Some new therapies are beginning to have an impact on the vasculopathic and fibrotic components of the disease. Better understanding of the key signaling pathways in pulmonary arterial hypertension (PAH), such as the endothelin pathway, has led to the development and approval of agents such as bosentan, sildenafil, and ambrisentan. N.Walsh. Skin & Allergy News. Volume 39, Issue 8, Page 65 (August 2008) (Also see: Scleroderma Treatments)
Treatments for pulmonary hypertension (PH) include bosentan (Tracleer), endothelin receptors (Sitaxsentan, Ambrisentan), lung transplants, oxygen therapy, sildenafil citrate (Revatio, Viagra), endothelin receptors (Sitaxsentan, Ambrisentan), stem cell transplants, and warfarin (Coumadin).
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.
Medical Therapy for Pulmonary Arterial Hypertension (PAH). This update provides evidence-based treatment recommendations for physicians involved in the care of patients with PAH. Due to the complexity of the diagnostic evaluation required and the treatment options available, referral of patients with PAH to a specialized center continues to be strongly recommended. Chest. 2007; 131:1917-1928.
Pulmonary arterial hypertension and systemic sclerosis. Pulmonary arterial hypertension is a serious complication of systemic sclerosis and a leading cause of death in patients with it. Recent publications suggest that a prevalence of 10-15% is likely. PubMed. Presse Med. 2006 Dec;35(12 Pt 2):1929-37.
Pulmonary arterial hypertension in collagen disease: findings of the German Network for Systemic Scleroderma (DNSS). The DNSS recommends annual echocardiography for patients with scleroderma and right-heart catheterization if the systolic pulmonary arterial pressure is higher than 35 mmHg. Aggressive immunosuppressive treatment - in the first instance with cyclophosphamide - should be given only if there is also progressive fibrosis. PubMed. Dtsch Med Wochenschr. 2006 Dec;131 Suppl 9:S325-7.
A case of systemic scleroderma complicating pulmonary hypertension. This case was treated from an early stage with steroid pulse therapy and CY pulse therapy, accompanied with oral administration of a PGI2 preparation for the pulmonary hypertension. The dermal symptoms improved, and it was possible to maintain a state of remission. PubMed. Nihon Rinsho Meneki Gakkai Kaishi. 2006 Dec;29(6):378-83. (Also see: Juvenile Scleroderma)
Pulmonary hypertension gets a closer look.   Researchers have found that excesses or deficiencies of certain substances within the pulmonary artery are linked to a progression of the disease, and several medications have been developed to address those problems. Pittsburgh Post-Gazette. 10/11/06.
Successful Treatment of Pulmonary Arterial Hypertension (PH) Associated with Mixed Connective Tissue Disease (MCTD) by Methylprednisolone Pulse Therapy. Our findings suggest the long-term efficacy of methylprednisolone pulse therapy for early PH associated with MCTD. Doppler echocardiography is also very useful to detect early PH in MCTD. S. Ohshima. AB0283 EULAR 2006. (Also see: MCTD)
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. (ScienceDirect) The Journal of Heart and Lung Transplantation Vol 27, Issue 12, Dec 2008, Pages 1326-1332.
Treprostinil (TYVASO)
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. PR Newswire. 07/31/09.
Warfarin (Coumadin)
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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