Congenital Aortic Stenosis

Congenital aortic stenosis is relatively common, accounting for about 8% of congenital heart disease. Three forms are recognized depending on severity and whether the defect is limited to the aortic valve.

Types

• Congenital aortic valvular stenosis
• Aortic stenosis
• Aortic atresia

Congenital Aortic Valvular Stenosis

Malformation of the aortic valve may cause obstruction of blood flow through the aorta. Congenital aortic valvular stenosis is characterized by fused, thick valve leaflets. Valvular stenosis leads to development of a hyperplastic left ventricle.

Aortic Stenosis

Congenital aortic stenosis (or "hypoplastic aorta") is caused by unequal division of the truncus arteriosus. Aortic stenosis leads to development of a hyperplastic left ventricle.

Severe aortic stenosis or atresia may be associated with hypoplastic left heart syndrome. Source: Wikimedia Commons

Aortic Atresia

Aortic atresia is an extreme form of aortic stenosis, also caused by unequal division of the truncus arteriosus. Aortic atresia leads to the development of a hypoplastic left ventricle and compensatory shunting of mixed blood (oxygenated and deoxygenated) around the aortic atresia:

• patent foramen ovale (with a Left-to-Right shunt to bypass the left ventricle)
• persistent ductus arteriosus (PDA; with a Right-to-Left shunt to supply the systemic circulation)