HEART, AORTA, TRACHEA - Tetralogy of Fallot

Tetralolgy of Fallot consists of the following:
  1. Ventricular septal defect (VSD)
  2. Overriding aorta
  3. Pulmonary stenosis
  4. Right ventricular hypertrophy
The specimen shows an infant heart opened to display a high ventricular septal defect, with over-riding of right ventricle by the origin of the aorta. The pulmonary valve cusps are missing, with replacement by nodules in the endocardium. In addition, as seen in relation to the trachea, the aorta is right-sided. There was also a large atrial septal defect (not displayed).
This child was in respiratory difficulty soon after birth. Radiologically there was evidence of congenital lobar emphysema of right lower lobe. This was treated surgically, but the child died shortly afterwards.