Treatment of PH is directed toward improving symptoms, exercise capacity and, when possible, survival. The therapy used for PH depends upon its underlying cause. PH can improve with treatment of associated heart or lung disease.
Non-medication Treatments
Important non-medication treatment includes: oxygen therapy, giving up smoking, removal of deleterious drugs and low level exercise. When PH develops into heart failure, diuretics, digitalis, and sodium restriction can be helpful. Because people with idiopathic pulmonary arterial hypertension (PAH) are at higher risk for pulmonary blood clots, the blood thinner warfarin is used to prevent blood clots.
Medication and Other Treatments
People whose pulmonary blood pressure “responds” to intravenous vasodilator treatment during RHC are candidates for calcium channel blocker therapy (CCB). People with idiopathic, familial, or connective tissue associated PH who do not improve with CCB treatment or do not respond to vasdilator challenge during RHC are candidates for oral medications.
These medications include: phosphodiesterase-5 (PDE-5) inhibitors (sildenafil, tadalafil) or endothelin receptor antagonists (ERA’s) (bosentan, ambrisentan, sitaxsentan). PDE-5 inhibitors and ERA’s can improve exercise capacity, and sometimes, slow the progression of the disease. It is unclear if these drugs provide benefit to people with PH associated with lung or heart disease.
Medications called prostanoids may improve symptoms and, sometimes, survival. Prostanoids can be given by nasal inhalation (iloprost), injection (treprostinil), or via IV infusion (epoprostenol). While the prostanoids can provide significant improvement, they have many side effects. They should be managed at PH centers experienced with their use. Surgical removal of blood clots can benefit people with chronic thromboembolic pulmonary hypertension (CTEPH).
Finally, lung or combined heart/lung transplantation is appropriate in a select group of people who have progressive disease and have failed traditional therapy.
