pediatrics
Other Congenital Cardiac Anomalies
Pulmonic valve stenosis in children
Pulmonic valve stenosis (see also Pulmonic Stenosis ) as an isolated finding is a common congenital defect (7 to 10% of congenital heart defects) and leads to obstruction of outflow from the right ventricle. This obstruction results in right ventricular hypertrophy and eventually right ventricular failure. The pathology may be commissural fusion of a bileaflet or trileaflet valve or leaflet dysplasia. Clinical findings include a prominent early systolic ejection click and a normal or wide split second heart sound (S2) with a soft pulmonary component and a harsh systolic ejection murmur (intensity varying directly with severity of stenosis), which is loudest at the upper left sternal border. In neonates with critical pulmonary stenosis, cyanosis is present due to right-to-left atrial shunting through a patent foramen ovale. Treatment is usually transcatheter balloon dilation. If the stenosis is moderate to severe, surgical relief of the stenosis is very effective, but surgery is usually reserved for those valves that are not amenable to transcatheter balloon dilation. 