ABSTRACT
Although obstructive lesions of the right ventricular outflow tract (RVOT) are found in 25-30% of children with congenital heart disease such as atrial and ventricular septal defects, isolated pulmonary stenosis at the valvular level with an intact ventricular septum accounts for approximately 8-10% of all congenital heart defects. The clinical presentation is variable, ranging from asymptomatic mild stenosis to complete atresia of the pulmonary valve, occurring in approximately 4.5 per 100 000 live births. Common genetic syndromes associated with pulmonary stenosis include Noonan’s, Williams, and Leopard syndromes.
