The etiology of the Pulmonary Hypertension should be corrected if possible. Congenital heart defects may be treated through surgical procedures or with medicines.

Medical treatment includes medications such as prostacyclins (Flolan ®, Remodulin ®) a steroid that relaxes blood vessels), calcium channel blockers (which also relax the blood vessels), endothelin antagonists (Bosentan ®), which counteract the effects of endothelin, a substance in the body that narrows blood vessels and increases blood pressure, and phosphodiesterase inhibitors (Sildenafil ®) that increase relaxation of the pulmonary arteries.

Other medications that may be prescribed include anticoagulants (blood thinners) to ward off blood clots (often associated with Pulmonary Hypertension), digitalis (to help regulate the heartbeat and reduce swelling of the ankles and legs), and diuretics (to reduce fluid retention). In addition, oxygen may be given if an inadequate supply in the blood (hypoxemia) is a cause of the high pulmonary pressure.

Depending on the etiology of pulmonary hypertension, some patients may improve or stabilize especially if diagnosed early, but overall mortality is still quite high.