If you were born with a heart condition that required surgery to repair your pulmonary valve and its nearby structures when you were an infant or child, the valve may begin to allow blood to “leak” back into your heart as you get older. A properly functioning pulmonary valve keeps blood flowing from the heart’s lower right pumping chamber (right ventricle) into the pulmonary artery and on to the lungs.

If the pulmonary valve begins to allow significant amounts of blood to leak back into the heart, then the right pumping chamber must begin to work harder to pump more blood than usual. In other words, the right ventricle is forced to pump the blood that is flowing normally into the chamber from the right atrium plus the blood that is leaking back. And, the more blood the valve allows to back up into the heart, the more work the heart’s right chamber must do. Over time, the extra work becomes burdensome for the heart. Its ability to pump blood may deteriorate, leading to heart failure.

Ideally, before your heart’s function is affected, your doctor will recommend that your pulmonary valve be fixed or replaced. Its repair or replacement will help maintain the proper function of the right ventricle and ensure your continued well-being.

Congenital Heart Conditions That May Require Pulmonary Valve Replacement in Adulthood

Many forms of congenital heart disease that are repaired in infancy and childhood involve surgery on the pulmonary valve and right ventricular outflow tract (RVOT).

The RVOT is the part of the heart that takes blood from the lower pumping chamber (right ventricle) to the blood vessels (pulmonary arteries) that carry blood from the heart to the lungs. In the lungs, the blood unloads carbon dioxide and wastes from the body and picks up oxygen. The RVOT includes the area underneath the pulmonary valve, the pulmonary valve itself and a portion of the main pulmonary artery (before it divides into the right and the left lung arteries).

Among the conditions that may require surgical procedures on the pulmonary valve and RVOT are:

• Tetralogy of Fallot. Babies born with Tetralogy of Fallot often have narrowings in all three of the areas described above. Repair may involve surgical enlargement of the entire passageway. During this procedure, the surgical incision that cuts through the pulmonary valve opening may leave it somewhat leaky.
• Pulmonary atresia and truncus arteriosus. Repair of these conditions may involve the placement of a valved tube (conduit) or human tissue tube (homograft) between the right ventricle and pulmonary artery. Placement of a tube is most often needed when there is no connection between the right ventricle and the pulmonary artery. Over time, the valves inside the tubes may deteriorate, leading to a leaky or regurgitant pulmonary valve.
• In addition, a number of operations, such as the Rastelli, Ross and Damus-Kaye-Stanzel procedures performed to make other congenital heart repairs, may also involve the placement of a valved tube (conduit) or human tissue tube (homograft) between the right ventricle and pulmonary artery. The valves inside the tubes may deteriorate, leading to a leaky or regurgitant pulmonary valve.

Symptoms of a Leaky Pulmonary Valve

Mild to moderate pulmonary valve leakage (or pulmonary regurgitation) may produce no symptoms. More significant leakage may lead to fatigue, particularly with exercise, and/or chest pain.

How Doctors Evaluate a Leaky Pulmonary Valve

Due to the shape of the right ventricle, evaluating the function of the right side of your heart is more difficult than assessing left heart function. Your doctor will use echo and, in some cases, a magnetic resonance imaging (MRI) or echocardiography to measure the size of the right side of the heart and assess its function with reasonable accuracy.

Treatment Options for a Leaky Pulmonary Valve

Depending on the results of your cardiac evaluation, your doctor may recommend medications to reduce fluid retention, although there is, as yet, no medication for the leaky valve itself. Treatments to address the valve problem include surgery or a less invasive, nonsurgical procedure (transcatheter pulmonary valve replacement). Below we review each option.

Medications for a Fluid Retention Caused by a Leaky Pulmonary Valve

Currently, there is no proven drug therapy for a leaky pulmonary valve. If you are retaining fluid, with swelling in your ankles and legs due to right heart failure, your doctor may prescribe diuretics (drugs that increase fluid removal from your body through urination). Diuretics may relieve swelling due to fluid retention, but they do not help fix the leaky valve.

Surgery to Replace a Leaky Pulmonary Valve

Surgical replacement of the pulmonary valve is considered when the right ventricle becomes significantly enlarged (generally greater than twice the size of the left ventricle) or when the tricuspid valve (the valve bringing blood from the right atrium into the right ventricle) also becomes leaky – especially if you have started to have symptoms.

Surgical replacement of the pulmonary valve can be performed with a low rate of complications. Because it is generally performed on patients who have had previous operations for complex congenital heart conditions, surgery on other heart problems may be undertaken as well. The extent of any operation will depend on each patient’s specific problems.

Transcatheter Pulmonary Valve Replacement

In certain situations, it may be possible to replace the pulmonary valve without open-heart surgery. In these cases, a tissue valve may be sewn onto a metal stent and the stented valve placed on a small, thin tube with a balloon on its tip (balloon catheter). The catheter is inserted into a vein (usually in the groin) and guided to the site in the heart where the valve is to be placed. The balloon is then inflated, and the stent is expanded. The valve on the expanded stent (which is a little larger than the passage in which it is placed) is secured within the pulmonary valve area. Once in place, the valve begins to function.

Valves replaced via catheter are currently available in Europe, Canada and other countries. They have not yet been approved for use in the United States by the U.S. Food and Drug Administration.

Follow-Up Care After Treatment of a Leaky Pulmonary Valve

Regular follow-up care is required for people who have had a leaky pulmonary valve. Of particular importance is monitoring the size of the heart’s right ventricle and leakage (regurgitation) of the pulmonary valve. A number of tests, including an echocardiogram, electrocardiogram, computerized axial tomography (CAT) scan, and magnetic resonance imaging (MRI), may be recommended.

In people with congenital heart defects, acquired heart disease can increase the chance of heart attack, heart failure, sudden death and stroke. Fortunately, acquired heart disease can be largely prevented by reducing or eliminating risk factors such as smoking, poor diet, high blood pressure and high cholesterol and lack of physical activity. Therefore, a heart-healthy lifestyle is strongly recommended.

Lifestyle Considerations for People Who Have Had a Leaky Pulmonary Valve

People who have been treated for a leaky pulmonary valve require ongoing treatment by a cardiologist, who should advise you about the kind and level of activity that is appropriate for you, given your condition. It is always wise to consult a cardiologist before trying new activities.