Pulmonary Hypertension: Treatment

Reviewed by Marjorie Patricia George, MD
Treatment of pulmonary hypertension is directed toward improving symptoms, improving exercise capacity and delaying progression of the disease. The treatment used for pulmonary hypertension depends upon its underlying cause. Pulmonary hypertension can improve with treatment of associated heart or lung disease.


Non-medication Treatments

Important non-medication treatment includes:


Medication and Other Treatments

People whose pulmonary blood pressure “responds” to intravenous vasodilator treatment during right heart catheterization may be candidates for calcium channel blocker therapy. People with idiopathic pulmonary arterial hypertension that does not respond to vasodilator challenge during right heart catheterization or who do not improve with calcium channel blocker treatment, or people who have inherited, or connective tissue-associated pulmonary hypertension are candidates for oral medications.

These oral medications include:

  • phosphodiesterase-5 (PDE-5) inhibitors (sildenafil or tadalafil),
  • soluble guanylate cyclase stimulator (riociguat),
  • endothelin receptor antagonists (ERAs) (bosentan, ambrisentan, macitentan).

PDE-5 inhibitors and ERAs can improve exercise capacity, and sometimes, slow the progression of the disease. It is unclear if these medication provide benefit to people who have pulmonary hypertension associated with lung or heart disease.


Prostanoid medications

Medications called prostanoids may improve symptoms and, sometimes, survival. Prostanoids can be given by inhalation (treprostinil), subcutaneous injection (treprostinil) or via IV infusion (epoprostenol, treprostinil). There are also oral medications as well (selexipag, treprostinil). While the prostanoids can provide significant improvement, they have many side effects. They should be managed at pulmonary hypertension centers experienced with their use.

In people who have chronic thromboembolic pulmonary hypertension (CTEPH), surgical removal of blood clots or balloon pulmonary artery angioplasty is always considered, as this can be of greatest benefit.
When pulmonary hypertension develops into heart failure, diuretics, digitalis and sodium restriction can be helpful. In people who have pulmonary hypertension due to chronic blood clots, treating with a blood thinner is very important to prevent more blood clots.

Finally, lung or combined heart/lung transplantation may be appropriate in people who have progressive disease and have failed traditional therapy.

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