A number of entities can present as cyanotic congenital heart disease. These can be divided into those with increased (pulmonary plethora) or decreased pulmonary vascularity:
-
increased pulmonary vascularity
- total anomalous pulmonary venous return (TAPVR) (types I and II)
- transposition of the great arteries (TGA)
- truncus arteriosus (types I, II, and III)
- large AVSD
- single ventricle without pulmonary stenosis
-
decreased pulmonary vascularity
- tetralogy of Fallot
-
hypoplastic right heart syndrome: pulmonary vascularity dependent on systemic collaterals
- tricuspid atresia
- hypoplastic right ventricle
- pulmonary atresia or severe stenosis
- Ebstein anomaly with atrial septal defect
- many other combined and infrequent anomalies such as
- double outlet right ventricle (DORV) with pulmonary stenosis
- single ventricle with pulmonary stenosis
- Uhl anomaly
- pentalogy of Cantrell
The most important cyanotic congenital heart defects can be remembered with the five Ts mnemonic.
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