Suppose 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. In that case, the valve may begin to allow blood to “leak” back into the heart over time—this is known as pulmonary valve regurgitation. A properly functioning pulmonary valve keeps blood flowing from the heart’s lower right pumping chamber (right ventricle) into the pulmonary artery and onto the lungs.
A properly functioning pulmonary valve keeps blood flowing from the heart’s lower right pumping chamber (right ventricle) into the pulmonary artery and onto the lungs.
If the pulmonary valve begins to allow significant amounts of blood to leak back into the heart, the right pumping chamber must work harder to pump more blood than usual. In other words, the right ventricle is forced to pump the blood flowing normally into the chamber from the right atrium, plus the blood 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. As a result, 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 (CHD) repaired in infancy and childhood involve surgery on the pulmonary valve and right ventricular outflow tract (RVOT). Sometimes, these CHD conditions may require a pulmonary valve replacement later in life.
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 right side of 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 include the following:
- Tetralogy of Fallot – Babies born with Tetralogy of Fallot often have narrowings in all three areas described above. Therefore, repair may involve surgical enlargement of the entire passageway. The surgical incision that cuts through the pulmonary valve opening may leave it somewhat leaky during this procedure.
- 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. The 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, many 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.
Mild to moderate pulmonary valve leakage (or pulmonary valve regurgitation) may produce no symptoms. More significant leakage may lead to fatigue, particularly with exercise, lower extremity swelling, abdominal pain, and/or chest pain.
Due to the shape of the right ventricle, evaluating the function of the right side of your heart is more difficult than assessing the left heart function. Your doctor will use an echocardiogram (“echo”) and, in some cases, cardiac magnetic resonance imaging (MRI), to measure the size of the right side of the heart and assess its function with reasonable accuracy.
Depending on the results of your cardiac evaluation, your doctor may recommend medications to reduce fluid retention, although there’s currently no medication for the leaky valve itself. Treatments to address the valve problem may include a minimally invasive, nonsurgical procedure or open-heart surgery.
- Medications – Currently, there is no proven drug therapy for a leaky pulmonary valve. If you’re 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.
- Transcatheter pulmonary valve replacement (TPVR) – 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 (a tiny, expandable mesh-like metal tube) 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 (a little larger than the passage in which it’s placed) is secured within the pulmonary valve area. Once in place, the valve begins to function.
- Surgery – 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 symptoms.
Surgical replacement of the pulmonary valve can be performed with a low rate of complications. Because it’s generally performed on patients who have had previous operations for complex congenital heart conditions, surgery on other heart problems may also be undertaken. The extent of any operation will depend on each patient’s specific problems.
Regular follow-up care is required for people with a leaky pulmonary valve. Of particular importance is monitoring the size of the heart’s right ventricle and leakage (regurgitation) of the pulmonary valve. Some tests, including an echocardiogram, an electrocardiogram, a computed tomography (CT) scan, and a cardiac MRI, may be recommended.
In people with congenital heart defects, acquired heart disease can increase the chance of heart attack, heart failure, stroke, and even sudden death. Fortunately, acquired heart disease can be primarily 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.