Pulmonary stenosis

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Overview

Pulmonary valve stenosis is a narrowing of the valve located between the lower right heart chamber (right ventricle) and the lung arteries (pulmonary arteries). In a narrowed heart valve, the valve flaps (cusps) may become thick or stiff. This reduces blood flow through the valve.

Usually, pulmonary valve disease results from a heart problem that develops before birth (congenital heart defect). However, adults may develop pulmonary valve stenosis as a complication of another illness.

Pulmonary valve stenosis ranges from mild to severe. Some people with mild pulmonary valve stenosis don't notice any symptoms and may only require occasional doctor's checkups. Moderate and severe pulmonary valve stenosis may require a procedure to repair or replace the valve.

Symptoms

Mild pulmonary stenosis is rarely symptomatic. As the narrowing becomes more severe, common heart valve disease symptoms may include:

Chest pain: You may feel discomfort in your lungs and chest.

Shortness of breath: Breathing normally may feel difficult.

Fainting: You may lose consciousness or feel dizzy.

Fatigue: You may tire easily with physical exertion.

Although rare, some adults may develop pulmonary stenosis later in life. Risk factors for non-congenital pulmonary stenosis include:

Carcinoid syndrome, caused by carcinoid tumors in the digestive system

Rheumatic fever, a rare complication of strep throat

Radiation to the chest

Causes

Some people are born with pulmonary artery stenosis and have problems with their heart valves, walls or other parts of their heart as well. Others who have pulmonary artery stenosis at birth have no other heart issues. You can also develop the condition after heart surgery or from diseases that aren’t very common.

Congenital (since birth) causes of pulmonary artery stenosis

In 40% of cases, people are born with pulmonary artery stenosis but don’t have other heart problems.

Pulmonary artery stenosis happens in 2% to 3% of people who have other congenital (since birth) heart defects, such as:

Tetralogy of Fallot: A condition characterized by four problems in your child’s heart, impairing normal blood flow.

Pulmonary atresia: A defect in which the pulmonary valve, which connects the right ventricle to the pulmonary artery, doesn’t form at all. This keeps blood from flowing to your child’s lungs.

Truncus arteriosus: One combined heart artery instead of the normal two lets oxygen-rich blood blend with oxygen-poor blood.

Aortic valve stenosis: A problem with this valve reduces how much blood goes from your child’s heart to their body.

Atrial septal defect: A hole in the wall between your child’s two upper heart chambers (atria) lets blood with and without oxygen mix.

Ventricular septal defect: A hole in the wall that separates your child’s two lower chambers (ventricles) can make too much blood go to their lungs.

Transposition of the great vessels: The two major arteries that carry blood away from your child’s heart are in each other’s places. This impairs normal blood flow and limits how much oxygen can get to your child’s cells.

Patent ductus arteriosus: This is a connection between your child’s pulmonary artery and aorta. If it doesn’t close after birth as it should, too much blood goes to the lungs.

Other causes of pulmonary artery stenosis can include issues such as:

Rubella syndrome, a group of heart and other health problems your child gets from a rubella infection their birthing parent has while pregnant.

Williams syndrome, a group of abnormalities affecting your child’s heart and other organs.

Alagille syndrome, which causes liver problems and heart issues.

Takayasu’s arteritis, a type of inflammation that damages large blood vessels.

Problems that compress your child’s pulmonary artery from the outside.

Surgical causes of pulmonary artery stenosis

Some people get pulmonary artery stenosis after having a surgical procedure. These include:

Lung transplant.

Surgery to fix your child’s congenital heart issue or improve blood flow through their heart.

Pulmonary artery banding. This purposely narrows your child’s artery to reduce blood flow to their lungs.

Risk factors

German measles (rubella). Having German measles (rubella) during pregnancy increases the risk of pulmonary valve stenosis in the baby.

Noonan syndrome. This genetic disorder causes various problems with the heart's structure and function.

Rheumatic fever. ...

Carcinoid syndrome.

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Complications

Possible complications of pulmonary stenosis include:


Infection of the lining of the heart (infective endocarditis). People with heart valve problems, such as pulmonary stenosis, have an increased risk of developing bacterial infections that affect the inner lining of the heart.

Irregular heartbeat (arrhythmia). People with pulmonary stenosis are more likely to have an irregular heartbeat. Unless the stenosis is severe, irregular heartbeats due to pulmonary stenosis usually aren't life-threatening.

Thickening of the heart muscle. In severe pulmonary stenosis, the heart's right ventricle must pump harder to force blood into the pulmonary artery. The strain on the heart causes the muscular wall of the ventricle to thicken (right ventricular hypertrophy).

Heart failure. If the right ventricle can't pump properly, heart failure eventually develops. Symptoms of heart failure include fatigue, shortness of breath, and swelling of the legs and abdomen.

Pregnancy complications. The risks of complications during labor and delivery are higher for those with severe pulmonary valve stenosis than those without the condition.

Prevention

Children with pulmonary stenosis are at increased risk for subacute bacterial endocarditis (SBE). This is an infection of the heart caused by bacteria in the blood stream. It most commonly occurs after a dental or other medical procedure and can usually be prevented by a dose of antibiotic prior to the procedure.