Pulmonary valve stenosis

Overview

Pulmonary valve stenosis is a narrowing of a valve, called the pulmonary valve, that regulates the flow of blood from the right ventricle of the heart to the lungs. The defect may force the heart to pump harder, leading to an enlarged heart.

The heart consists of four chambers: two upper chambers, called atria, where blood enters the heart, and two lower chambers, called ventricles, where blood is pumped out of the heart. The flow between the chambers is controlled by a set of valves that act as one-way doors. Normally, blood is pumped from the right side of the heart through the pulmonary valve and the pulmonary artery to the lungs, where the blood is filled with oxygen. From the lungs, the blood travels back to the left chambers. The oxygenated blood is pumped to the rest of the body through a major blood vessel, called the aorta.

The pulmonary valve has three leaflets that work to open and close the valve. Pulmonary stenosis occurs when there is only one or two leaflets or if the leaflets are stuck together.

Signs & symptoms

Children and adults with mild pulmonary valve stenosis often do not have any symptoms. However, if the condition is severe, symptoms may include:

• Low energy
• Poor feeding
• Rapid breathing
• Tiring early with exercise
• Turning blue, a condition called cyanosis

Diagnosis

Because there may be no symptoms, the first sign of pulmonary valve stenosis often is a heart murmur, an extra sound heard during a chest examination. To diagnose pulmonary valve stenosis, the following tests may be performed:

• Echocardiogram, a test that uses sound waves to create a moving picture of the heart
• Electrocardiogram (ECG or EKG), a test that records the electrical activity of the heart
• Exercise stress test to test the effect of exercise on the heart

Treatment

Treatment for pulmonary valve stenosis depends on the severity of the condition and age of the patient.

Babies, children and young adults may be treated in our Pediatric Cardiac Catheterization Laboratory with a procedure called balloon valvuloplasty. This procedure involves inserting a catheter — a thin, flexible, plastic tube — into a blood vessel in the leg and then threading it through the blood vessel to the heart. A balloon at the tip of the catheter is inserted into the narrow opening in the valve and inflated to stretch the valve and separate the valve leaflets.

Balloon valvuloplasty has been very successful in treating pulmonary valve stenosis. In some cases, however, the valve is unusually thick and valvuloplasty is not effective. Then, surgery may be required.

It is important to note that patients who have pulmonary valve stenosis should take antibiotics to avoid heart infection during surgery or dental work.