Treatment of pulmonary hypertension

Pulmonary hypertension leading to right heart failure can be related to primary lung disease or hypoventilation. Idiopathic pulmonary hypertension is a progressive disease with poor prognosis. Therapy of idiopathic pulmonary hypertension includes: oxygen, calcium channel blockers, diuretics, anticoagulants, prostanoids, endothelin receptor antagonists and phosphodiesterase-5 inhibitors. Patients with pulmonary arterial hypertension (PAH) and pulmonary hypertension due to chronic thrombotic or embolic disease should be treated with vasodilatators. The potent vasodilatators are: prostacyclin PGI2, prostacyclin analogue and endothelin receptor antagonists. For patients with idiopathic PAH classified as NYHA III (New York Heart Association) bosentan is recommended, whereas for patients classified as NYHA IV--epoprostenol. Combination therapy is an emerging therapeutic option in PAH. In BREATH-2 (Bosentan Randomised Trial of Endothelin Antagonist Therapy for PAH) study the efficacy and safety of combining bosentan and epoprostenol given orally was investigated. No significant difference was established between treatment groups in 6-minutes walking distance or NYHA functional class. However other study investigating the combination of bosentan and prostacyclin analogue showed clinical improvement. Additional bosentan therapy may also reduce the epoprostenol dose and therefore decrease its side-effects. Interventional procedures: atrial septostomy and lung transplantation are indicated in patients with advanced NYHA class III and IV symptoms and refractory to available medical treatment. However, currently no management potent enough to cure pulmonary arterial hypertension is available. The introduction of new class of drugs allowed for the improvement of quality of life and overall survival. The choice of drug depends on a variety of factors including accessibility, approval status and patient's preferences.