Sunday, August 4, 2019

Tetralogy of Fallot: The Basics

Hey guys, this post highlights the important points discussed in the accompanying video.

The four salient anatomic components of TOF results from a specific morphogenetic abnormality - Malalignment of the Infundibular septum, i. e. it deviates anteriorly and cephalad; so that it is not aligned with the trabecular or muscular septum.

1. This leads to a large non-restrictive VSD.

2. Since it encroaches on the RV Outflow tract, it leads to Infundibular stenosis and Overriding or Biventricular Aorta.

3. The non restrictive VSD leads to systemic BP in the RV, leading to development of RVH.
TOF is very commonly seen in Alagille syndrome. It is also associated with mutation in JAG1 gene that is a ligand in the NOTCH signaling pathway which controls the development of neural crest cells, a part of which is present in the endocardial cushions from which the Infundibular or conotruncal septum develops.

That's all!  Do watch the video for a better grasp on this topic.

Also, here is a cute animation of the heart in TOF:

- Vinayak

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