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.
(See also Overview of Heart Defects. For this disorder in adults, see Pulmonic Stenosis.)
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), causing right-to-left shunting. 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 Heart Failure: Pumping and Filling Problems).
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.
Echocardiography (ultrasonography of the heart) confirms the diagnosis.
Electrocardiography (ECG) and chest x-rays are typically done. The ECG usually shows thickening of the right side of the heart if the narrowing is moderate to severe.
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.
American Heart Association: Common Heart Defects: Provides an overview of common birth defects of the heart for parents and caregivers
American Heart Association: Infective Endocarditis: Provides an overview of infective endocarditis, including summarizing antibiotic use, for parents and caregivers
