Pathophysiology of Tetralogy of Fallot

Right ventricular outflow tract obstruction (RVOTO):

In the healthy animal, the pulmonary valve is unobstructed allowing blood to flow
into the pulmonary circulation without excessive resistance.
In the unhealthy animal, the pulmonary valve can be obstructed at multiple
levels. This results in a varying degree of RVOTO:

Mild obstruction doesnt typically cause cyanosis because there is

sufficient blood flow into the pulmonary circulation to maintain blood
oxygen saturation.
Severe obstruction causes cyanosis because it opposes blood flow into the
pulmonary circulation, resulting in deoxygenated blood shunting from the
right ventricle to the left ventricle through the ventricular septal defect
into the systemic circulation. (Blood flow may depend on the patent
ductus arteriosus or bronchial collaterals).

Pulmonary artery anatomy:

Tetralogy of Fallot can be complicated further by association with other
pulmonary diseases such as:

Pulmonary atresia the underdevelopment of the right ventricular outflow

tract. (?)
Absent pulmonary valve, leading to severe pulmonary regurgitation and
massive dilation of pulmonary arteries.

Atresia: http://emedicine.medscape.com/article/905119-overview#a7
Absent: http://emedicine.medscape.com/article/899249-overview
Mal-alignment ventricular septal defect (VSD):
In the healthy animal, the septum should close during development to form a
barrier between the right and left sides of the heart.
In the unhealthy animal, the septum doesnt close correctly allowing blood to
shunt from one side to the other. This can be restrictive or non-restrictive:

Anterior mal-alignment VSD in TOF is almost always non-restrictive,

meaning that the pressure in the right and left ventricles can equalise.
This means that it is the RVOTO that determines the degree of shunting.
Restrictive anterior mal-alignment VSD in TOF is a rare anomaly. When
restricted, the patient is susceptible to right ventricular pressure overload
and subsequent right ventricular failure.

Overview: http://bestpractice.bmj.com/bestpractice/monograph/701/basics/pathophysiology.html
Overview: http://emedicine.medscape.com/article/2035949-overview#a6
Restrictive VSD: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3860792/#bib1