Pulmonary valve stenosis is a heart valve disorder that involves the pulmonary valve.
This valve separates the right ventricle (one of the chambers in the heart) and the pulmonary artery. The pulmonary artery carries oxygen-poor blood to the lungs.
Stenosis, or narrowing, occurs when the valve cannot open wide enough. As a result, less blood flows to the lungs.
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.
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:
Symptoms may get worse with exercise or activity.
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:
The health care provider will grade the severity of the valve stenosis to plan treatment.
Sometimes, treatment may not be needed if the disorder is mild.
When there are also other heart defects, medications may be used to:
Percutaneous balloon pulmonary dilation (valvuloplasty) may be performed when no other heart defects are present.
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.
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.
Call your health care provider if:
Valvular pulmonary stenosis; Heart valve pulmonary stenosis; Pulmonary stenosis; Stenosis - pulmonary valve; Balloon valvuloplasty - pulmonary
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.
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.
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