Pulmonary valve stenosis

Pulmonary valve stenosis is a type of heart valve disease that involves the narrowing of the pulmonary valve, which controls the flow of blood from the heart’s right ventricle into the pulmonary artery to carry blood to the lungs. During stenosis, the pulmonary valve’s flaps (also known as cusps or leaflets) are thickened, stiffened or fused together. This narrowed opening of the valve slows or blocks the flow of blood into and through the pulmonary artery.

Due to the slowed blood flow into the valve, the right ventricle is forced to work harder to pump blood to the lungs successfully. Over time, this extra work required of the heart can cause the heart to become strained and go into heart failure.

Pulmonary valve stenosis may also be referred to as pulmonic stenosis.

Pulmonary stenosis symptoms typically depend on how severe the stenosis is. The more narrowed the pulmonary valve becomes, the more your heart and body will feel the symptoms and be impacted by the condition. Many people living with this condition have mild or no symptoms. Mild pulmonary stenosis is rarely symptomatic at all.

Common symptoms of pulmonic stenosis include:

• Chest pain: you may feel discomfort in your lungs and chest
• Shortness of breath: breathing normally may feel difficult, especially during physical activity
• Fainting: you may lose consciousness or feel dizzy
• Fatigue: you may tire easily with physical exertion

There is not a lot known about the direct cause for pulmonary stenosis, however it usually develops before birth when the pulmonary valve does not develop normally while growing inside the womb. This condition accounts for almost 10 percent of all congenital heart disease.

People born with pulmonary valve stenosis need lifelong monitoring by a cardiologist or adult congenital heart disease specialist.

Although rare, some adults may develop pulmonary stenosis later in life. Risk factors for non-congenital pulmonic stenosis include:

• Carcinoid syndrome, which is a condition caused by carcinoid tumors in the digestive system that release chemicals into the blood stream that may damage heart valves
• Rheumatic fever, a rare complication of strep throat
• Radiation to the chest, possibly from treatment for cancer close to the chest
• Rubella, a condition known as German measles that can increase the risk of pulmonary stenosis if experienced during pregnancy

While it is usually diagnosed in childhood, pulmonary stenosis may go undetected until adulthood or develop later in life. If your pulmonary valve is narrowed, your doctor may hear a heart murmur during a routine examination. Diagnosis of pulmonary stenosis requires additional testing using echocardiography and cardiovascular imaging techniques including:

• Transthoracic echocardiogram (TTE)
• Electrocardiogram
• Cardiac magnetic resonance imaging (MRI)
• Cardiac catheterization

At the time of diagnosis, your doctor determines whether your pulmonary stenosis is mild, moderate or severe. The classification is based on the difference between the blood pressure in your right ventricle versus the blood pressure in your pulmonary artery. A severe narrowing causes a bigger difference in blood pressure.

People living with heart valve conditions may feel an impact on their health outside of the possible symptoms. There is an increased risk of complications related to pulmonary stenosis, especially if the condition is left untreated or unmanaged.

People with pulmonic stenosis may develop:

• Endocarditis, which is an inflammation of the inner lining of the heart chambers and valves. This infection is most often bacterial and rarely fungal.
• Ventricular hypertrophy, which is a thickening of the right ventricle caused by an overworking of the heart chamber to compensate for slowed blood flow.
• Cardiac arrhythmia, also known as an irregular heartbeat that results from abnormal electrical signals that control the heartbeat.
• Complications during pregnancy
• Heart failure

Our cardiology team provides regular monitoring of mild and moderate pulmonary stenosis. If your valve was replaced in childhood, our imaging experts and adult congenital heart disease specialists evaluate your prosthetic valve for signs of dysfunction.

If your pulmonary stenosis is severe, your cardiologist may recommend valve replacement or repair. Treatment for pulmonary valves at Penn Medicine includes:

• Pulmonary valve repair and replacement, which allows our expert cardiac surgeons to surgically repair or replace your diseased pulmonary valve.
• Balloon valvuloplasty, which is a catheter-based treatment that uses an expanded balloon to repair your narrowed pulmonary valve.
• Transcatheter pulmonary valve replacement (TPVR), which is a nonsurgical procedure for valve replacement if your previously replaced pulmonary valve is dysfunctional.

In addition to traditional treatment for pulmonary valve stenosis, Penn researchers are at the forefront of innovations in valve treatment. Through clinical trials, patients have access to new therapies for pulmonary valve replacement and repair not available elsewhere.