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 Overview of Heart Defects 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 . For this disorder in adults, see Pulmonic Stenosis Pulmonic Stenosis Pulmonic (pulmonary) stenosis is a narrowing of the pulmonary valve opening that blocks (obstructs) blood flow from the right ventricle to the pulmonary artery. It is often present at birth... read more .)
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 ), 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 that is pumped from the left side of the heart to the rest of the body. The more oxygen-poor blood (which is blue) that flows to the body, the bluer the body appears.
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 (see figure Heart Failure: Pumping and Filling Problems Heart Failure: Pumping and Filling Problems ).
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.
Echocardiography Echocardiography and Other Ultrasound Procedures Ultrasonography uses high-frequency (ultrasound) waves bounced off internal structures to produce a moving image. It uses no x-rays. Ultrasonography of the heart (echocardiography) is one of... read more (ultrasonography of the heart) confirms the diagnosis.
Electrocardiography Electrocardiography Electrocardiography (ECG) is a quick, simple, painless procedure in which the heart’s electrical impulses are amplified and recorded. This record, the electrocardiogram (also known as an ECG)... read more (ECG) and chest x-rays X-Rays of the Chest Anyone thought to have a heart disorder has chest x-rays taken from the front and the side. Typically, the person is standing upright, but chest x-rays can be done with people lying in bed if... read more are typically done. Results are usually normal, but sometimes the ECG shows thickening of the right side of the heart.
Drugs, such as a prostaglandin, to keep the ductus arteriosus open
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 drug 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.
The following are some English-language resources that 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