Wednesday, April 25, 2012

Cyanotic Heart Disease: Differential Diagnosis #5

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:

DiseaseNotes
TTruncus arteriosus1 vessel
TTransposition of great arteries2 vessels switched; "egg on a string" heart
TTricuspid atresia3 leaflet valve
TTetralogy of Fallot4 findings: Pulmonary stenosis, RVH, VSD, aortic override; "boot shaped heart"
TTotal 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.

TAPVR Type I (Supracardiac)
Source: LearningRadiology