Describe Congenital Pulmonary Stenosis?

The murmur present since birth. PS may be valvular, sub-valvular, or supravalvular. An obstruction may occur within the RV cavity by an abnormal muscle bundle (i.e. " double chambered RV"). In valvular PS the valve is thickened, with fused or absent commissures and, a small orifice dysplastic valves are frequent in Noonan Syndrome. Supra-valvulal- PS is associated with Congenital Rubella Syndrome and Williain Syndrome. Most patients are acyanotic and well developed. Newborns with critical PS are cyanotic and tachypneic. Majority of patients are asymptoinntic in infancy and childhood. Dyspnea and fatigue are mild as long as the right ventricle maintains normal stroke volume at rest and augments its stroke volume with exercise.

Figure: Congenital pulmonary stenosis

Maximum ESM is over main pulmonary artery (i.e. 2"d Left intercostal space) associated with systolic thrill. The intensity and length of ESM is directly. Proportional to severity of PS. wide spitting of S2 due to delayed closure of pulmonary valve. Ejection click present the interval between S1 and click varies inversely with the degree of PS, more severe the stenosis earlier the click'. ECG shows RVH. Neonates with critical PS may show LVH because of hypoplastic RV and relatively large LV. Sudden death is possible in patients with severe PS during heavy exertion.