Treatment
Pulmonary Arterial Hypertension

Drug therapies
Surgery

While general treatment for IPAH and secondary PAH is similar, treatment may vary, partly because treating the underlying other disease in secondary PAH is an important part of controlling the PAH. In addition, direct treatments for the PAH may be less effective in secondary PAH than in IPAH.

The main goals of all PAH treatment are to treat any underlying disease, reduce symptoms to give the patient a better quality of life, and delay hospitalization for serious complications. Increasing blood supply to the heart and reducing its workload, as well as slowing the changes in the pulmonary artery, form the central targets for these therapies.

Lifestyle changes are an early and safe component of all PAH treatment. In addition to receiving ongoing care from a pulmonologist experienced in treating PAH, this consists of:

  • relaxation and stress reduction
  • walking as an exercise, particularly early in the disease
  • nonphysical activities and hobbies later in the disease
  • avoiding high altitudes, including air travel
  • for women, avoiding pregnancy

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Drug therapies

Conventional therapy

Conventional treatment for PAH may include blood pressure lowering drugs like diuretics -- drugs that cause people to lose water through increased urination. These drugs decrease blood pressure and reduce fluid in the lungs. Oral anticoagulants are another important basic treatment, to prevent blood clots from forming in the remodeled pulmonary arteries. Finally, oxygen therapy helps some patients lessen symptoms and maintain their activity level.

Patients are referred to as vasoreactive (vass-oh-ree-ACK-tiv) when their pulmonary arteries still have the ability to respond to the body's natural signals. For these people, an expanding family of drugs can help reduce pulmonary arterial pressure by causing the vessels to expand.

Specific PAH vasoactive drugs

Recent developments in therapeutic options mirror an increase in our understanding of the pathobiological causes of this disease.  Treatment options involve correcting abnormalities in three pathobiological pathways: nitric oxide pathway, prostacyclin pathway, endothelian pathway. These therapies differ in their indications, routes of administration, and adverse effects. 

Prostacyclins - The earliest members of this family were very effective but expensive and difficult to administer. The drug had to be kept chilled and administered continuously through a permanent catheter in a vein, which can become infected. Improved versions with much less demanding administration can be inhaled through a nebulizer or injected intravenously under the skin (subcutaneously -- though this method may be painful). Prostacyclins dilate pulmonary arteries, inhibit smooth muscle growth, and inhibit blood clotting.

Endothelin receptor antagonists - This family of drugs also widens the arteries and inhibits smooth muscle growth. These drugs can be given as an oral pill.

Nitric oxide inhalation -  Inhalation of a mixture of oxygen and nitric oxide will selectively dilate the pulmonary arteries.

Phosphodiesterase inhibitors - Drugs that fall into this category are available in the oral form.  They selectively expand the pulmonary arteries and inhibit smooth muscle growth.

Less common treatments for PAH include high-dose calcium-channel-blocker drugs, which cause the heart to beat less forcefully. These work in only a small portion of patients who have IPAH, and can be extremely dangerous to those who are not vasoreactive.

Increasingly, doctors are treating PAH patients who do not respond to single drug use with combinations of drugs. There are a wide number of clinical trials that are evaluating the use of combination medications for the treatment of PAH. 

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Surgery

Surgery may be an option for some patients with PAH.

In a procedure called atrial septostomy (AY-tree-al sep-TOH-sto-mee), a surgeon creates a hole in the central wall of the heart. While it relieves some of the pressure on the right side of the heart and the pulmonary artery, it reduces oxygen levels in the blood, which may eventually worsen the PAH.

Lung transplantation may be an option for some PAH patients with advanced disease that is not responding to other treatments. Some of these patients may require a heart and lung transplant.

Finally, in a new procedure called pulmonary thromboendarterectomy (PULL-moh-na-ree throm-boh-end-ar-ter-ECK-toh-mee, or PTE), the surgeon removes the inner lining of the pulmonary artery, to widen it and remove blood clots. This is a major, high-risk procedure with death rates as high as 24 percent from the surgery alone. But immediate survival is much better (death rates as low as 5 percent) at expert surgical centers, and 75 to 80 percent of patients who survive the surgery are still alive five years later. This suggests that the procedure may be very effective when performed by an expert surgeon.

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