If a child has congenital heart disease, the blood may bypass the pulmonary system. If so, the blood is not fully oxygenated, causing the baby to have a dusky hue (appear cyanotic). Depending on the degree and type of malformation, the child may present anytime from birth up til late adolescence. Five common causes of
cyanotic heart disease are the
1-2-3-4-5 Ts:
| Disease | Notes |
| T | Truncus arteriosus | 1 vessel |
| T | Transposition of great arteries | 2 vessels switched; "egg on a string" heart |
| T | Tricuspid atresia | 3 leaflet valve |
| T | Tetralogy of Fallot | 4 findings: Pulmonary stenosis, RVH, VSD, aortic override; "boot shaped heart" |
| T | Total Anomalous Pulmonary Venous Return | 5 vessels involved; "snowman" heart |
In TAPVR, the pulmonary veins do not drain into the left atrium. In the most common form Type I, the vessels form the left vertical vein and drain superior to the heart to the brachiocephalic, SVC or azygos vein. The left vertical vein forms the snowman's head. In Type II, the pulmonary veins drain to the coronary sinus. In Type III, they drain subdiaphragmatically to the IVC.
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