Pulmonic Valve Disease

How the pulmonic valve works

The heart has four valves. The pulmonic valve has three thin leaflets that open when the right ventricle squeezes blood toward the lungs and close to stop it flowing back. It handles lower pressures than the valves on the left side of the heart, so problems tend to be better tolerated and develop more slowly.

There are two types of pulmonic valve disease:

Causes

Pulmonary stenosis

Pulmonary stenosis is usually congenital and is one of the more common congenital heart defects. The leaflets may be fused or thickened. It can occur alone or with other defects, and is common in Noonan syndrome. Rarely, it develops later from rheumatic heart disease or carcinoid syndrome.

Pulmonary regurgitation

A trace of leakage is common and normal. Significant regurgitation is most often seen in adults who had repair of tetralogy of Fallot or pulmonary stenosis as children, because the surgery widened or removed part of the valve. Other causes include pulmonary hypertension, which stretches the valve ring, infective endocarditis, and carcinoid syndrome, in which hormones released by a tumor stiffen the right-sided heart valves.

Symptoms

Mild disease usually causes no symptoms and is found when a doctor hears a murmur. As disease becomes moderate or severe, the right ventricle works harder and symptoms appear:

• shortness of breath, especially during exercise
• tiredness and reduced exercise capacity
• chest pain or pressure
• lightheadedness or fainting with exertion (severe stenosis)
• palpitations from irregular heart rhythms
• swelling of the ankles, legs or belly when the right side of the heart begins to fail

Babies with critical pulmonary stenosis may be blue (cyanosed) and breathless soon after birth, and need treatment in the first days of life.

Diagnosis

1. Examination: stenosis causes a harsh murmur at the upper left chest, sometimes with a click. Regurgitation causes a softer murmur.
2. Echocardiogram: an ultrasound of the heart shows the valve, measures the pressure difference across it (gradient), grades leakage and shows the size and function of the right ventricle.
3. ECG: may show strain or thickening of the right ventricle and rhythm problems.
4. Cardiac MRI: the best test for measuring the size and pumping of the right ventricle, used to time valve replacement in regurgitation.
5. Exercise testing: checks whether symptoms and fitness are truly limited.
6. Cardiac catheterization: measures pressures directly and is often combined with treatment.

Treatment

Monitoring

Mild stenosis or regurgitation rarely progresses much. Regular check-ups with an echocardiogram every few years are usually enough. Adults who had heart surgery as children should be followed in a specialist adult congenital heart disease clinic.

Balloon valvuloplasty

For moderate to severe stenosis, a thin catheter is passed from a vein in the groin to the heart, and a balloon is inflated in the valve to stretch it open. It is highly effective, often permanent in children, and avoids open surgery. Some regurgitation may follow.

Valve replacement

Severe regurgitation that is enlarging the right ventricle, causing symptoms or causing arrhythmias is treated with valve replacement:

• Transcatheter pulmonary valve replacement: a new valve is delivered by catheter, often inside a previous surgical valve or conduit.
• Surgical replacement: with a tissue (biological) valve, through open-heart surgery.

Tissue valves wear out over 10 to 20 years, so further procedures may be needed in life.

Medicines

Medicines do not fix the valve, but diuretics can ease swelling, and medicines control heart rhythm problems.

Complications

• right heart failure
• arrhythmias, including atrial flutter and ventricular arrhythmias, particularly after tetralogy repair
• infective endocarditis of an abnormal or replaced valve
• sudden cardiac death, rarely, in severe untreated disease

Living with pulmonic valve disease

Most people with mild disease live completely normal lives with no restrictions. With moderate or severe disease, your cardiologist will advise on exercise and sports. Good dental hygiene and regular dental check-ups lower the risk of endocarditis; people with replacement valves usually need antibiotics before some dental procedures. Women planning pregnancy should have a specialist review first, because pregnancy increases the load on the heart.

Frequently asked questions

What is pulmonic valve disease?

It is a problem with the pulmonary valve, between the right ventricle and the lung artery. The valve may be narrowed (stenosis), blocking flow, or leaky (regurgitation), letting blood flow back. Most cases are present from birth or follow childhood heart surgery.

Is pulmonary valve disease serious?

Mild disease is usually not serious and often never needs treatment. Severe stenosis or regurgitation strains the right side of the heart and can cause heart failure and rhythm problems, but it can be treated effectively with balloon procedures or valve replacement.

What are the symptoms of pulmonary stenosis?

Mild pulmonary stenosis causes no symptoms. More severe stenosis causes breathlessness and tiredness during exercise, chest pain, lightheadedness or fainting on exertion, and swelling of the legs in advanced cases. Babies with critical stenosis can turn blue soon after birth.

Can you live a normal life with a leaky pulmonary valve?

Yes, most people can. Mild regurgitation is common and harmless. Moderate or severe leakage needs regular monitoring with echocardiograms and MRI, and valve replacement before the right ventricle is damaged. After treatment, most people lead active lives.

How is a pulmonary valve replaced?

It can be replaced through open-heart surgery or, in many people, through a catheter inserted in a groin vein, with no chest incision. The catheter approach is often used to place a new valve inside a previously implanted valve or conduit.