Pulmonary Hypertension: Treatment

Reviewed by Marjorie Patricia George, MD

Treatment of pulmonary hypertension is directed toward improving your symptoms and ability to move; delaying progression of the disease; and, most important, helping you have a better quality of life. The treatment used for pulmonary hypertension depends upon its underlying cause.

 

Non-medication Treatments for Pulmonary Hypertension

Important non-medication treatment includes:

 

Medication and Other Treatments for Pulmonary Hypertension

People whose pulmonary blood pressure “responds” to inhaled vasodilator treatment during right heart catheterization may be candidates for calcium channel blocker therapy. People with pulmonary arterial hypertension (PAH) 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, or PAH due to other causes may be candidates for vasodilator therapy.
Pulmonary hypertension medications come in different forms: oral, inhaled, intravenous (through an IV catheter) and subcutaneous.

Pulmonary hypertension medications include:

  • Phosphodiesterase-5 (PDE-5) inhibitors (sildenafil or tadalafil)

  • Soluble guanylate cyclase stimulator (riociguat)

  • Endothelin receptor antagonists (ERAs) (bosentan, ambrisentan, macitentan)

  • Prostacyclin pathway medications (epoprostenol, treprostinil, selexipag).

 

Medications in the prostacyclin pathway may improve symptoms, exercise tolerance and sometimes survival. These medications can be given orally (selexipag, treprostinil); by inhalation (treprostinil); by subcutaneous injection (treprostinil); or via IV infusion (epoprostenol, treprostinil). While these medications can provide significant improvement, they require judicious dosing and have many side effects. They should be managed at pulmonary hypertension programs experienced with their use.

In treating pulmonary arterial hypertension, patients are often started on multiple medications at the same time or in rapid succession to target multiple pathways in this disease and achieve the best response in the shortest amount of time.

In people who have pulmonary hypertension due to chronic blood clots, known as chronic thromboembolic pulmonary hypertension (CTEPH), surgical removal of the scar tissue or balloon pulmonary artery angioplasty is always considered, as this can be of greatest benefit. In patients with CTEPH, lifelong treatment with a blood thinner is crucial to prevent more blood clots.

When pulmonary hypertension develops into heart failure, diuretics, digoxin and sodium restriction can be helpful.

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

 

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