Pulmonary Hypertension Treatment

As with any disease, early detection improves treatment options for pulmonary hypertension. If an early diagnosis is made, some medications are available to minimize or slow disease progression. In later stages of the disease, when heart failure is present, most medications simply help relieve symptoms.

While a definitive diagnosis may take some time, and the disease may be quite advanced by the time of diagnosis, current treatments for primary pulmonary hypertension have improved the long term prognosis considerably, with some PPH patients surviving fifteen or twenty years or longer.

Medications used in treatment for pulmonary hypertension improve circulation by limiting vessel constriction, enhancing heart muscle contraction, and/or slowing the thickening of the blood vessel wall.

Anticoagulation

Treatment for pulmonary hypertension with oral anticoagulation (blood-thinning) medication has shown to improve PPH survival rates. In fact, anticoagulation is recommended for the majority of PPH patients in the absence of a bleeding disorder or other condition that would make anticoagulation treatment dangerous.

Prostacyclins

Prostacyclins are substances that occur naturally in the body but can also be manufactured as a valuable medication to help slow the progression of PPH. Prostacyclins aid in dilating the blood vessels, which allows blood to circulate more easily. Prostacyclins also improve exercise endurance, and have been shown to enhance survival of PPH patients.

The medications are quick acting but short lived, so they must be continuously administered via intravenous (IV) or subcutaneous (under the skin) infusion. Common prostacyclin used in treatment for pulmonary hypertension are epoprostenol (Flolan®), which is given IV, and treprostinil (Remodulin®), which is given subcutaneously.

Endothelin Receptor Antagonists

Endothelin receptor antagonists, such as bosentan (Tracleer®), are oral heart medications that help stop blood vessels from tightening and becoming thicker. In patients with sclerosis, bosentan also prevents the formation of scar tissue. Pregnant women are not candidates for this medication. All PPH patients taking bosentan are monitored monthly to assess the medication’s affect on the liver.

Phosphodiesterase Inhibitors

Phosphodiesterase inhibitors are enzymes that favor blood vessel dilation. They do so by slowing the breakdown of dilation factors that keep vessels open. These drugs work along the same principle as Viagra® to improve blood flow.

Calcium Channel Blockers

Calcium channel blockers relax the inside walls of the blood vessels to improve the circulation of blood through them. Not all PPH patients respond to calcium channel blockers, but the ten to fifteen percent of individuals who do respond boast a five-year survival rate of ninety percent.

Digitalis

Digitalis improves the strength of heart muscle contraction and helps to lower heart rate in patients with a rapid pulse (tachycardia). Use of digitalis must be strictly monitored due to the medication’s side effects, which include anxiety, dizziness, hallucinations, confusion, depression, and palpitations.

L-Arginine

L-arginine is an amino acid that is necessary for the production of nitric oxide, a gas that relaxes blood vessels causing them to dilate and improve blood flow. L-arginine as a potential treatment for pulmonary hypertension is still under investigation.

Serotonin Inhibitors

Serotonin may stimulate the thickening of the blood vessel walls, which may be the reason the “Fen-Phen” type of appetite suppressants contribute to secondary pulmonary hypertension development. Researchers are currently evaluating the effect of serotonin inhibitor medications in PPH treatment.

PPH and SPH Symptom Relief

If pulmonary hypertension has progressed to the point of heart failure, some medications can relieve symptoms. Diuretics, or water pills, flush excess fluid from the body to minimize edema (swelling of the tissues). Supplemental oxygen is used in some cases to relieve shortness of breath.

Surgical Treatment for Pulmonary Hypertension

If the direct cause of secondary pulmonary hypertension is the formation of blood clots in the lungs, a surgical procedure called thromboendarectomy, may be used to remove the clots. Thromboendarectomy has five-year survival rate of eighty percent.

Another surgical procedure reserved for end-stage treatment for pulmonary hypertension is the single lung transplant or heart-lung transplant. Single lung transplants are less complicated than heart-lung transplants and have a seventy percent survival rate for the first year.

Birth Control for Women

Because of the increased risk of mortality due to PPH during pregnancy, hormonal birth control is recommended for fertile women of childbearing age.

Pulmonary Hypertension and Endocarditis

People with pulmonary hypertension should take antibiotics prior to dental procedures, including cleaning, and any other invasive procedure to prevent bacterial endocarditis (infection of the heart valves).

Resources

American Lung Association. (November 2003). Primary pulmonary hypertension (PPH) fact sheet.

Beers, M. H.