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Pulmonary Valve Stenosis in Children


Lee B. Beerman

, MD, Children's Hospital of Pittsburgh of the University of Pittsburgh School of Medicine

Reviewed/Revised Apr 2023

Pulmonary valve stenosis is a narrowing of the pulmonary valve (sometimes called the pulmonic valve), which opens to allow blood to flow from the right ventricle to the lungs.

  • The heart valve between the right ventricle and the artery to the lungs is narrowed.

  • In most children, the only symptom is a heart murmur, but, if the narrowing is severe in an infant, a bluish color to the skin (cyanosis) and signs of right heart failure (such as fatigue and enlargement of the liver) are possible.

  • The diagnosis is suspected based on a heart murmur heard with a stethoscope and is confirmed with echocardiography.

  • Balloon valvuloplasty to open the valve or surgery to reconstruct it is sometimes needed.

In most children with pulmonary valve stenosis, the valve is mildly to moderately narrowed, making the right ventricle pump a bit harder and at a higher pressure to propel blood through the valve. Severe narrowing increases pressure in the right ventricle and may limit the amount of blood that can reach the lungs. When pressure in the right ventricle becomes extremely high, the valve leading into the right ventricle may leak, forcing oxygen-poor blood back into the right atrium and then through a hole in the atrial wall (atrial septal defect Atrial and Ventricular Septal Defects Atrial and ventricular septal defects are holes in the walls (septa) that separate the heart into the left and right sides. Holes can be present in the walls of the heart between the upper heart... read more Atrial and Ventricular Septal Defects ), causing right-to-left shunting Shunting of blood flow About one in 100 babies is born with a heart defect. Some are severe, but many are not. Defects may involve abnormal formation of the heart's walls or valves or of the blood vessels that enter... read more . In right-to-left shunting, oxygen-poor blood from the right side of the heart mixes with oxygen-rich blood from the left side of the heart. The more oxygen-poor blood (which is blue) that flows to the body, the bluer the body appears.

Symptoms of Pulmonary Valve Stenosis in Children

Most children with pulmonary valve stenosis have no symptoms. Severe pulmonary valve stenosis may cause the skin to have a bluish coloration (cyanosis), particularly of the lips, tongue, skin, and nail beds. Newborns and infants are more likely to have cyanosis than are older children. Older children with severe pulmonary stenosis are more likely to may have fatigue and/or shortness of breath with exertion due to heart failure of the right side of the heart (see figure ).

Diagnosis of Pulmonary Valve Stenosis in Children

  • Echocardiography

Doctors often suspect pulmonary stenosis if they hear a certain kind of heart murmur while listening with a stethoscope. A heart murmur is a sound created by turbulent blood flow through narrowed or leaking heart valves or through abnormal heart structures. An extra sound or click also may be heard.

Treatment of Pulmonary Valve Stenosis in Children

  • Medications, such as a prostaglandin, to keep the ductus arteriosus open in newborns

  • Balloon valvuloplasty or surgery

Treatment depends on the severity of the infant's symptoms.

Severe disease that causes cyanosis in newborns is treated by giving a prostaglandin by vein (intravenously). The prostaglandin keeps the ductus arteriosus open, thus sending extra blood to the lungs to increase the level of oxygen in the infant's blood. This medication is usually given until the valve can be repaired with balloon valvuloplasty or a surgical procedure.

For balloon valvuloplasty, a thin tube (catheter) with a balloon at its tip is passed through a blood vessel in the arm or leg into the narrowed valve. The balloon is inflated and used to widen the narrowed opening of the valve.

Doctors usually also do balloon valvuloplasty in infants who do not have cyanosis if the valve is moderately or severely narrowed.

If the valve is very small or markedly thickened, balloon valvuloplasty may not be sufficient. Surgery is then used to open or reconstruct the pulmonary valve.

Children do not need to take antibiotics before visits to the dentist or surgeries.

More Information

The following English-language resources may be useful. Please note that THE MANUAL is not responsible for the content of these resources.

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