Pulmonary Valve Stenosis

Overview

In pulmonary valve stenosis, the heart’s pulmonary valve is thick and its opening is smaller than normal. As a result, blood can’t flow normally from the heart through the valve and to the lungs.

When the pulmonary valve is narrowed, the lower right chamber of the heart (the right ventricle) tries to squeeze harder to push blood out into the pulmonary vessels. The extra effort causes the right ventricle’s muscles to become bigger and thickened, making the ventricle stiffer and harder to fill. Initially, depending on how severely the valve is narrowed, enlarged right ventricle muscles may cause no symptoms or ill effects.

However, if the narrowing is severe or if the narrowed valve places undue pressure on the heart, over time, the right ventricle will become fatigued and may begin to fail under its workload. The inflow valve to the right ventricle (tricuspid valve) may leak significantly due to the very high pressure generated in the ventricle. Individually, or in combination, worsening stiffening of the ventricle or tricuspid valve leakage may place additional stress on the right upper chamber (right atrium). If severe, this may result in swelling (edema) of the abdomen, as well as of the head, neck, and extremities, leading to a buildup of fluid in the tissues.

The initial indication of pulmonary valve stenosis may be an abnormal heart murmur that's heard with a stethoscope during a physical exam with a doctor. To confirm a diagnosis of pulmonary valve stenosis, diagnostic testing would include an electrocardiogram (ECG or EKG) and an echocardiogram (echo), with the echo usually confirming the diagnosis.

Treatment

Mild pulmonary valve stenosis usually is very stable over time and typically requires no medical therapy. Sometimes, as a child grows, this stenosis may improve independently.

Moderate pulmonary valve stenosis could progress to severe. Both moderate and severe pulmonary valve stenosis require medical treatment, either with catheter-based balloon valvuloplasty or surgery:

• Balloon valvuloplasty – In balloon valvuloplasty, which has become the standard of care to relieve pulmonary valve stenosis, an interventional cardiologist inserts a thin, flexible tube (catheter) with a balloon on its tip in a large vein in the groin and guides it through the vein into the heart’s right ventricle. The catheter is placed across the narrow opening of the pulmonary valve, and the balloon is inflated. As the balloon opens, it stretches the valve open, forcing it to widen so more blood can flow through. Balloon valvuloplasty works best if the stenosis results from fused valve leaflets, or cusps. If the valve leaflets are extremely thickened (dysplastic), balloon valvuloplasty may not be successful due to the rubbery nature of the valve. Therefore, surgical repair may then be necessary.
• Surgical repair – Improved blood flow through the pulmonary valve can be accomplished by surgically separating the valve’s leaflets or removing tissues obstructing the valve. Surgical valve repair requires opening the chest, stopping the heart, and placing the patient on a heart-lung bypass machine to keep the blood circulating through the body during the procedure.