Pulmonary valve stenosis in children

[Hina Muqadas]

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 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 prevents almost any blood from reaching the lungs. When pressure in the right ventricle becomes extremely high, oxygen-poor blood is forced through abnormal paths (usually a hole in the atrial wall [atrial septal defect]) instead of the pulmonary artery, 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 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.