Pulmonic Stenosis

• Pulmonic stenosis without VSD= 8% of all CHD

            • Mostly asymptomatic

            • When symptomatic, cyanosis and heart failure

            • Loud systolic ejection murmur

• Cor pulmonale

• Rare calcification of pulmonary valve in older adults

X-ray

            • Enlarged main pulmonary artery

            • Enlarged left pulmonary artery (jet stream effect)

            • Normal to decreased peripheral pulmonary vasculature

Pulmonic Stenosis. There is post stenotic dilatation of the main pulmonary artery (red arrow) from valvular pulmonic stenosis. This could be presumed to be a mediastinal mass not of vascular origin. A CT scan would be definitive.

Subvalvular pulmonic stenosis

            •Infundibular pulmonic stenosis

                        • Typically in Tetralogy of Fallot

            •Subinfundibular pulmonic stenosis

                        •Associated with VSD (85%)

Valvular Pulmonic Stenosis

            • Classic pulmonic stenosis (95%)

            • Fusion of pulmonary cusps

            • Presents in childhood

            • Pulmonic click

            • Dome-shaped pulmonic valve

            • RX: Balloon valvulo-plasty

Trilogy of Fallot

            • Severe pulmonic valvular stenosis

            • RV hypertrophy

            • ASD with R—L shunt

Supravalvular pulmonic stenosis

            •May be either tubular hypoplasia or localized with poststenotic dilatation

            •May be associated with:

                        • Valvular pulmonary stenosis, supravalvular aortic stenosis, VSD, PDA, systemic arterial stenoses

                        • Williams-Beuren Syndrome: PS, supravalvular AS, peculiar facies

                        • Ehlers-Danlos syndrome

                        • Postrubella Syndrome: peripheral pulmonary stenoses, PDA, low birth weight, deafness, cataracts, mental retardation