What can be done to treat Pulmonary Arterial Hypertension?

There is currently no cure for Pulmonary arterial hypertension (PAH) but advances in understanding about how the disease develops (see section: Causes of Pulmonary Arterial Hypertension ) mean that licensed treatments have become are available which have helped to improve prognosis and quality of life for patients with this disease.

Treatment options for patients with Pulmonary Arterial Hypertension 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).

General treatments include:¹

• Anticoagulants, which are used to prevent blood clots from forming in the lungs

• Diuretics, (commonly known as water tablets) which reduce fluid overload and reduce swelling

• oxygen therapy, to increase the amount of oxygen available to the blood

• calcium-channel blockers (CCBs), which can relieve constriction in the pulmonary arteries.

Treatments that have been specifically studied in Pulmonary Arterial Hypertension (PAH) include:

• Prostacyclin analogues (or 'Prostanoids'): prostacyclin analogue therapy is used to increase the level of prostacyclin in the body, which is typically lower than normal in people with Pulmonary Arterial Hypertension (PAH). Administering these treatments can be complex as they must be given either intravenously (directly into the bloodstream via a catheter), subcutaneously (under the skin via an infusion pump) or as an inhaled medication, using a nebuliser

• phosphodiesterase 5 inhibitors: these are given as tablets to help the blood vessels in the lungs to relax

• endothelin receptor antagonists: endothelin is thought to be involved in the development of Pulmonary Arterial Hypertension (PAH) and patients with the condition produce excessive levels of this substance. Endothelin receptor antagonists (ERAs) are oral treatments (tablets) that help block the damaging effects of endothelin by binding to one or both of the Endothelin receptors.^2-4

In very severe cases, surgical options may be considered:

• balloon atrial septostomy is an operation that creates a small hole in the wall between the two upper chambers of the heart. This acts as a safety valve if the pressure in the right heart increases suddenly

• heart and lung transplantation can be useful for a small number of patients but its use is limited by the shortage of donor organs.For further information about treatment for Pulmonary Arterial Hypertension (PAH) please speak with your doctor.

References
1. Nazzareno G, Seeger W, Naeije R, et al. Comparative analysis of clinical trials and evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol 2004;43: (Supply S): S76-88
2. Sitbon O, Humbert M, Ja£s X et al. Long-term response to Calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005; 11:3105-11
3. Channick RN, Simonneau G, Sitbon O, et al.  Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study.  Lancet 2001;358(9288):1119-1123
4. Rubin LJ, Badesch DB, Barst RG, et al.  Bosentan therapy for pulmonary arterial hypertension.  N Engl J Med 2002;346(12):896-903
5. Barst RG, Langleben D, Frost A, et al. Sitaxsentan therapy for pulmonary arterial hypertension. Am J Respir Crit Care Med 2004;169: 441-447