Pulmonary valve stenosis

Causes

Narrowing of the pulmonary valve is usually present at birth (congenital). It is caused by a problem that occurs when the unborn baby (fetus) is developing. The cause is unknown, but genetics may play a role.

Narrowing that occurs in the valve itself is called pulmonary valve stenosis. There may also be narrowing just before or just after the valve.

The defect may occur alone or with other heart defects that are present at birth (congenital). The condition can be mild or severe.

Pulmonary valve stenosis is a rare disorder.

In some cases, pulmonary valve stenosis runs in families.

Symptoms

Many cases of pulmonary valve stenosis are mild and do not cause symptoms. These infants are usually found to have a murmur on a routine heart examination.

When the valve narrowing (stenosis) is moderate to severe, the symptoms include:

• Abdominal distention
• Bluish color to the skin (cyanosis) in some patients
• Chest pain
• Fainting
• Fatigue
• Poor weight gain or failure to thrive in infants with severe blockage
• Shortness of breath
• Sudden death

Symptoms may get worse with exercise or activity.

Exams and Tests

The health care provider may hear a heart murmur when listening to your heart using a stethoscope. Murmurs are blowing, whooshing, or rasping sounds heard during a heartbeat.

Tests used to diagnose pulmonary stenosis may include:

• Cardiac catheterization
• Chest x-ray
• ECG
• Echocardiogram
• MRI of the heart

The health care provider will grade the severity of the valve stenosis to plan treatment.

Treatment

Sometimes, treatment may not be needed if the disorder is mild.

When there are also other heart defects, medications may be used to:

• Help blood flow through the heart (prostaglandins)
• Help the heart beat stronger
• Prevent clots (blood thinners)
• Remove excess fluid (water pills)
• Treat abnormal heartbeats and rhythms

Percutaneous balloon pulmonary dilation (valvuloplasty) may be performed when no other heart defects are present.

• This procedure is done through an artery in the groin.
• The doctor sends a flexible tube (catheter) with a balloon attached to the end up to the heart. Special x-rays are used to help guide the catheter.
• The balloon stretches the opening of the valve.

Some patients may need heart surgery to repair or replace the pulmonary valve. The new valve can be made from different materials. If the valve cannot be repaired or replaced, other procedures may be needed.

Outlook (Prognosis)

Patients with mild disease rarely get worse. However, those with moderate to severe disease will get worse. The outcome is good with successful surgery or balloon dilation. Other congenital heart defects may be a factor in the outlook.

Most often, the new valves can last for decades. Others wear out and will need to be replaced.

Possible Complications

• Abnormal heartbeats (arrhythmias)
• Death
• Heart failure and enlargement of the right side of the heart
• Leaking of blood back into the right ventricle (pulmonary regurgitation) after repair

When to Contact a Medical Professional

Call your health care provider if:

• You have symptoms of pulmonary valve stenosis
• You have been treated or have untreated pulmonary valve stenosis and have developed swelling (of the ankles, legs, or abdomen), difficulty breathing, or other new symptoms

Alternative Names

Valvular pulmonary stenosis; Heart valve pulmonary stenosis; Pulmonary stenosis; Stenosis - pulmonary valve; Balloon valvuloplasty - pulmonary

References

Carabello BA. Valvular heart disease. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 75.

Marelli AJ. Congenital heart disease. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 69.

Update Date: 6/7/2012

Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Shabir Bhimji, MD, PhD, Specializing in Cardiothoracic and Vascular Surgery, Midland, TX. Review provided by VeriMed Healthcare Network. David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.