Pulmonary Hypertension

Treatment

Pulmonary Arterial Hypertension
Other Forms of Pulmonary Hypertension

Pulmonary Arterial Hypertension

Pulmonary arterial hypertension is a relatively rare disease and no cure exists for it yet. However, specialized treatment can lower pulmonary pressures, reduce symptoms, increase the capacity to be active, and prolong lifespan. Treatments for pulmonary arterial hypertension include the following:

Medications

Prostacyclin analogues
These medicines enable the vessels in the lungs to expand and allow the blood to move through them with less resistance (vasodilation). The primary drugs in this category are prostacyclin, which is given as a continuous intravenous infusion, and treprostinil, which is given as a continuous infusion under the skin or as a continuous intravenous infusion.

Endothelin receptor antagonists
These drugs, in pill form, help to reverse the effect of endothelin, a substance in blood vessels that causes the vessels to constrict. As a result, these drugs also produce vasodilation. Bosentan is the endothelin receptor antagonist approved for use in pulmonary hypertension; others are being studied.

Phosphodiesterase-5 inhibitors
Sildenafil is a medication that has been FDA-approved to treat pulmonary hypertension. Revatio contains sildenafil, the same active ingredient as the impotence drug Viagra. It works by opening the blood vessels in the lungs, but it is used cautiously because sildenafil has been associated with vision problems and other side effects.

High-dose calcium channel blockers
For some patients, these medications also help to relax the muscles in the blood vessels, and promote vasodilation.

Anticoagulants
These drugs help to prevent the blood from clotting.

Diuretics
These medications help to remove excess fluid from body tissues which may accumulate due to high pressures in blood vessels.

Oxygen
With a higher concentration of oxygen in the air, more oxygen enters the bloodstream even though blood flow is reduced.

Many drugs for treating pulmonary hypertension have strong, serious side effects. The level of dosage must be carefully set and monitored to avoid dangerous consequences.

Surgery

In rare cases, a lung or heart-lung transplant may be an option, at least for younger patients with pulmonary arterial hypertension. See transplant pages for more information.

Other Forms of Pulmonary Hypertension

Because some other disease may be the cause, the underlying disease has to be treated along with the blood vessel disease. Successful treatment of the underlying disease may decrease or cure the pulmonary hypertension. If the underlying disease cannot be cured, the pulmonary hypertension may have to be treated directly, using many or all of the treatment methods used in pulmonary arterial hypertension.

Chronic Thromboembolic Pulmonary Hypertension
This is a type of pulmonary hypertension that is often missed or misdiagnosed. Patients often have a history of blood clots to the lung but some have no such history. The correct diagnosis of this condition is important because it is very treatable by a surgical procedure called pulmonary thromboendarterectomy. This may be recommended for people whose secondary pulmonary hypertension is due to persistent pulmonary emboli (blood clots).