Study note

Tetralogy of Fallot: developmental basis and shunt mechanism

THE CONTEXT
Abnormal bulbar septal development produces anatomical abnormalities that elevate right ventricular pressure and permit right-to-left shunting.

Developmental abnormality

FeatureDetails
Affected structure
Tetralogy of Fallot results from abnormal development of the bulbar septum.
Normal relationship
The bulbar septum separates the ascending aorta from the pulmonary artery and normally aligns and fuses with the outflow part of the interventricular septum.

Right ventricular outflow obstruction

FeatureDetails
Usual location
Obstruction is most often subvalvular and muscular.
Other locations
Obstruction may be valvular, supravalvular or a combination of these forms.
Dynamic component
Subvalvular obstruction may suddenly increase with sympathetic stimulation.

Septal defect and haemodynamic consequence

FeatureDetails
Defect size
The ventricular septal defect is usually large, with an aperture similar to the aortic orifice.
Pressure and shunting
The combination elevates right ventricular pressure and causes right-to-left shunting of cyanotic blood across the ventricular septal defect into the aorta.
Explore this chapter
NEETx
Learn. Practise. Remember.