The conotruncal defects are a collection of congenital malformations of the cardiac outflow tract(s). The ontology of these cadiac defects is somewhat difficult… there are many overlapping definitions.
Persistent truncus arteriosus
Failure of the truncus arteriosis to divide into the pulmonary trunk and the aorta leaves a patent common trunk. It has previously been classified by the development and position of the pulmonary trunk and/or arteries. The newer classification is in three parts:
- Aortic dominance with both pulmonary arteries arising from the trunk.
- Aortic dominance with one pulmonary artery absent from the trunk.
- Pulmonary dominance with obstruction of the aortic arch.
Previously included was type IV persistent truncus arteriosus, defined by aortopulmonary collateral circulation or pulmonary arteries arising distally from the aorta. This is now included as part of Tetralogy of Fallot.
Tetralogy of Fallot
This is the most common congenital cardiac defect, it is a cyanotic heart disease. The tetralogy is characterised by:
- Ventricular septal defect.
- Overriding aortic root.
- Right ventricular outflow tract obstruction, pulmonary valve stenosis.
- Right ventricular hypertrophy.
Transposition of the great vessels
Technically is any abnormal spacial arrangement of the the great vessels. More commonly meant is transposition of the great arteries which the ICD-11 defines as:
LA85.1 “A congenital cardiovascular malformation in which the morphologically right ventricle or its remnant connects to the aorta and the morphologically left ventricle or its remnant connects to the pulmonary trunk.”1
Double outlet right ventricle
Also coming in various forms, the principle is as the name infers.