Congenital pulmonary valvar stenosis

Congenital pulmonary valvar stenosis (also known as congenital pulmonary valve stenosis or congenital pulmonic stenosis) is a heart defect present at birth in which the pulmonary valve — the valve that controls blood flow from the right ventricle of the heart to the pulmonary artery and lungs — is abnormally narrowed. This narrowing (stenosis) obstructs blood flow from the heart to the lungs, forcing the right ventricle to work harder to pump blood through the restricted valve. The condition accounts for approximately 8–10% of all congenital heart defects. The pulmonary valve leaflets are often thickened, partially fused (commissural fusion), or dome-shaped, reducing the effective valve opening. Symptoms vary depending on the severity of the stenosis. Mild cases may be asymptomatic and detected only by the presence of a heart murmur during routine examination. Moderate to severe cases can cause exertional dyspnea (shortness of breath with activity), fatigue, chest pain, cyanosis (bluish discoloration of the skin due to reduced oxygen), and in neonates with critical stenosis, heart failure. Right ventricular hypertrophy develops as the heart compensates for the increased workload. In severe or critical cases, particularly in neonates, the condition can be life-threatening without prompt intervention. The primary treatment for moderate to severe congenital pulmonary valvar stenosis is balloon valvuloplasty (also called balloon valvotomy), a catheter-based procedure that dilates the narrowed valve. This procedure has excellent long-term outcomes and is considered the first-line intervention. Surgical valvotomy or valve replacement may be necessary in cases where the valve anatomy is not amenable to balloon dilation, such as when there is significant valve dysplasia. Mild stenosis typically requires no intervention but is monitored with periodic echocardiography. With appropriate treatment, the long-term prognosis is generally very good, though some patients may require re-intervention later in life due to residual stenosis or pulmonary regurgitation.

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