Tetralogy of Fallot is one of the more common forms of congenital heart disease. Many affected patients develop cyanosis.


Embryologic basis: anterior deviation of the infundibular septum


Primary 4 features (tetralogy):

(1) pulmonary stenosis with outflow obstruction from the right ventricle

(2) right ventricular hypertrophy

(3) ventricular septal defect (VSD), usually located in the posterior portion of the septum just below the aortic valve

(4) rightward shift of the aortic root, so that it overrides the ventricular septal defect


Additional findings may include:

(1) anomalies of the aortic arch (right aortic arch, other)

(2) anomalies of the coronary arteries

(3) anomalies of the pulmonary blood supply (pulmonary atresia, anomalous blood supply from the aorta, other)

(4) insufficiency of the aortic valve if valve annulus is enlarged


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