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Congenital Pulmonary Stenosis
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Important Resources for Congenital Pulmonary Stenosis:
Congenital pulmonary stenosis is relatively common. Isolated pulmonary stenosis accounts for about 8% of congenital heart disease, and pulmonary stenosis also occurs as part of some malformation syndromes, e.g., Tetralogy of Fallot. Three forms of pulmonary stenosis are recognized, depending on severity and whether the defect is limited to the pulmonary valve.
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Types
- Congenital pulmonary valvular stenosis
- Pulmonary stenosis
- Pulmonary atresia
Congenital Pulmonary Valvular Stenosis
Malformation of the pulmonary valve may cause obstruction of blood flow through the pulmonary trunk. Congenital pulmonary valvular stenosis is characterized by fused, thick valve leaflets. Valvular stenosis leads to development of a hyperplastic right ventricle.
Pulmonary Stenosis
Congenital pulmonary stenosis (or "hypoplastic pulmonary trunk") is caused by unequal division of the truncus arteriosus. Pulmonary stenosis leads to the development of a hyperplastic right ventricle.
Pulmonary Atresia
Pulmonary atresia is an extreme form of pulmonary stenosis, also caused by unequal division of the truncus arteriosus. Pulmonary atresia leads to the development of a hypoplastic right ventricle and compensatory shunting of mixed blood (oxygenated and deoxygenated) around the pulmonary atresia:
- patent foramen ovale (with a Right-to-Left shunt to bypass the right ventricle)
- persistent ductus arteriosus (PDA; with a Left-to-Right shunt to supply the pulmonary circulation)
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