Tetralogy of Fallot: Management Strategies by P. Lang | OPENPediatrics

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I'm Peter Lang. I'm a cardiologist at the Children's Hospital in Boston, and we're talking about Tetralogy of Fallot.

What we're going to do now is speak about what I'm going to call a garden variety Tetralogy of Fallot. That is, we're in the midst of discussing different management strategies, and they depend a bit upon how kids present and what their individual anatomy and physiology is. And we're going to take a couple of examples.

Review of Basic Anatomy and Physiology.

And what we're going to do right now is talk about a child with Tetralogy of Fallot who I'm going to say is plain old Tetralogy of Fallot, no bells and whistles. And as we have learned over the years, Tetralogy of Fallot does have Fallot's Four Components.

A ventricular septal defect. Pulmonary stenosis. In this drawing, sub-pulmonary stenosis. An overriding aorta, so it's a bit over the ventricular septum. And right ventricular-- increased right ventricular muscle mass, or right ventricular hypertrophy, because the pressure in the right ventricle is high. There's transmission of high pressure from the left ventricle, and there is the outflow tract obstruction.

We know that, really, this is all because there is a malalignment of the conal septum with the ventricular septum, which creates the VSD, crowds the right ventricular outflow tract between the conal septum and the free wall of the right ventricle, leading to the overriding of the aorta. And as a consequence of that, there is right ventricular muscle hypertrophy.

In what I'm going to call the usual or more typical form of Tetralogy of Fallot, we've got our ventricular septal defect and have a modest amount of right ventricular outflow tract obstruction. In this situation, systemic venous return comes to the right atrium, goes across the tricuspid valve to the right ventricle. And the way I've drawn it, a fair amount of it, if not all, can go out to the pulmonary artery while pulmonary venous return from the lungs and the left atrium goes across the mitral valve and then out the aorta.

And this would be a balanced circulation. The blood in the aorta is fully saturated. There's no admixture of systemic venous blood. Nor is there a lot of, or any-- the way I've drawn it, so far-- of blood going from the left ventricle to the pulmonary artery. So normal pulmonary blood flow, normal systemic blood flow.