Thursday, May 29, 2014

Tetralogy of Fallot

One of the most interesting things I've got to do is intern at my local hospital in a cardiovascular lab. To me, the cardiovascular system is simply phenomenal; from studying it in anatomy and exercise physiology class and seeing it in the “real world”, the way that the cardiovascular system functions and works is breath taking to me. I actually was able to see an open heart surgery recently and it was one of the coolest things I have ever seen. People always want to go see “breath taking” sights: the Grand Canyon, Yellowstone, the Aurora Borealis; if you want to see something that will take your breath away, look no further than the human body.
I’ve come across my fair share of interesting cardiac diseases while working in the hospital. One of the most interesting to me is called Tetralogy of Fallot (TOF). TOF isn’t just single disease, though. Instead, it is four congenital dysfunctions compacted into one: ventricular septal defect, overriding aorta, pulmonary stenosis, and right ventricular hypertrophy. The fact that a child can even survive being born with so much wrong with their heart is astounding, not to mention the fact that we have the ability to surgically fix that many defects. Children’s Hospital of Boston boasts a 92% surgical success rate if performed within the first thirty days of a child’s life, and a 99% surgical success rate if performed after the first thirty days.
Figure A shows the structure and blood flow in the interior of a normal heart. Figure B shows a heart with the four defects of tetralogy of Fallot.


TOF is the most common cyanotic heart defect known. This should come without surprise, as the ventricular septic defect and the overriding aorta would cause oxygenated and deoxygenated blood to mix, pulmonary stenosis making it harder for blood to get to the lungs to become oxygenated, and the right ventricular hypertrophy being caused by blood not flowing properly to the lungs. With so much mixing of deoxygenated blood and difficulty getting blood into the lungs, it shouldn’t be a revelation that the child isn’t getting enough oxygenated blood to their body. Thus, one of the tell-tale signs (as the baby’s patent ductus arteriosus closes) is a bluish tint to the skin.

But while those are the most common defects, there are a laundry list (up to nine others are currently known) of other abnormalities that may occur. A baby can survive this and we are able to repair it. Look no further than the human body.

Other symptoms include those seen with respiratory distress, having a higher chance at facial abnormalities, as well as symptoms from the four (or more) defects that makes up TOF.

But can anyone lead a normal life if they’re cursed by Fallot? Sure it may be repairable, but being repaired and living life are two different things. But you can do more than just lead a normal life, you can excel at it, even become the best. A man who has achieved more with a heart defect than most do without, Shaun White is perhaps the most famous person alive with TOF. With gold medals from the Olympics, winning over ten times in the X games, winning the United States open championship four times in the United States open championship, winning the gold medal in the World Cup three times, and many more feats, Shaun proves that this heart defect doesn't stop you from anything.

Look no further than the human body.

Resources:
http://www.cdc.gov/ncbddd/heartdefects/tetralogyoffallot.html
http://www.childrenshospital.org/health-topics/conditions/tetralogy-of-fallot-tof-tet
http://www.cincinnatichildrens.org/health/t/tof/
http://en.wikipedia.org/wiki/Tetralogy_of_Fallot
http://www.mayoclinic.org/diseases-conditions/tetralogy-of-fallot/basics/definition/con-20043262
https://www.nhlbi.nih.gov/health/health-topics/topics/tof/#
http://www.teamusa.org/Athletes/WH/Shaun-White