In some cases of pulmonary arterial hypertension and other forms of the disease, surgery can be useful and even curative.

Surgery is occasionally used to treat the underlying condition that is causing some types of pulmonary arterial hypertension. For instance, an operation to correct certain heart abnormalities, such as congenital heart defects, can eliminate the problem that is producing elevated pulmonary blood pressure.

Some types of secondary pulmonary hypertension also benefit from surgery. If the cause of pulmonary hypertension is blood clots that impair blood flow to the lungs, surgeons can sometimes operate to remove the clots. The operation, called a thromboendarterectomy, is a complicated and risky one; it is used only for patients with severe symptoms. To evaluate whether the operation is appropriate, doctors perform a right heart catheterization and a pulmonary angiogram. These tests allow physicians to gauge the extent of blood clots and to assess whether they can be removed with surgery.

Thromboendarterectomy is an open-heart procedure that is done routinely only at a few specialized centers. Surgeons open the chest through the breast bone. The patient is placed on a heart-and-lung by-pass pump. Opening the main pulmonary artery and its first branches, surgeons remove old blood clots and damaged artery linings. Recovery requires several days or more in an intensive care unit. The operation has a death rate between 5 percent and 10 percent. Blood clots must be prevented lifelong after the operation.

According to the Mayo Clinic, the operation may provide a cure for the particular type of pulmonary hypertension caused by abnormal particles in the blood that fail to dissolve, sticking to the blood vessel wall and obstructing normal blood flow instead; removing them can not only reverse right heart failure, but restore normal pulmonary artery pressures.

Liver transplant for cirrhosis can also be helpful for porto-pulmonary hypertension.

When all other treatment options have failed, and the lungs have been badly damaged by the disease, lung or heart-and-lung transplants are sometimes performed. A single lung transplant is a common method used in cases of pulmonary arterial hypertension. Some transplant centers prefer to replace both lungs partly because they believe double transplants cause fewer complications (a recent Johns Hopkins study found that patients with various diseases who received a double lung transplant lived twice as long on the average as people who received a single lung.) Combined heart and lung transplants are done less often, because of their increased risk.

Single lung transplants are usually reserved for patients under 65 who are otherwise in good health and double lung transplants for those under 60. (Heart-lung transplants, because of the greater risk they pose, are usually reserved for patients under 55.) However, as the population ages and transplant success rates improve, these recommended age limits may increase. Doctors carefully screen candidates for transplant surgery in order to ensure that the patients who undergo such operations are those who stand the best chance of surviving surgery and benefiting from it.

The survival rate for transplant surgery has been improving dramatically since the operations were first pioneered in the 1980s. (The latest figures can be obtained at http://www.optn.org). One reason lung transplant surgery has proved successful is the surprising ability of the right ventricle to heal itself after pulmonary blood pressure is normalized. Studies have shown that both its structure and function improve after transplantation.

Still, organ transplants of any kind carry serious risks. The most troublesome complication is rejection. This occurs when the immune system reacts to foreign "fingerprints" on the cells of the transplanted organ and attacks them. Untreated, immune rejection mechanisms can destroy a transplanted organ within days. To prevent rejection, doctors prescribe drugs that suppress the immune system. Anti-rejection drugs are usually given by injection during the first several weeks, and in pill form later. Unfortunately, these drugs can make patients more vulnerable to infections. Over time, recipients can usually reduce the dosage of anti-rejection drugs. However, transplant patients must continue to take the drugs for the rest of their lives.

-- Peter Jaret is a contributing editor for Health magazine and a winner of the American Medical Association's award for medical reporting. His work has appeared in National Geographic, Newsweek, Hippocrates, and many other national magazines. He is also the author of In Self-Defense (Harcourt Brace Jovanovich), Active Living Every Day, and Heart Healthy for Life.

References

Last updated August 20, 2009
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