What do fruit flies have to do with hypoplastic left heart syndrome?

In addition to the clinical trials, imaging studies, and other research our program conducts to find solutions for people with hypoplastic left heart syndrome (HLHS), our team members also study fruit flies to learn more about congenital heart defects.

Wait, did you just say fruit flies?

That’s right. Fruit flies are used in cardiac research because of the similarities of the fruit fly heart and the human heart. The fly’s heart is in its abdomen and is about 1mm long. The fly heart is a linear tube, just like the human heart during the first 3.5 weeks of embryological development. Roughly 75% of the genes that cause human disease also have functions in the fruit fly.

The first gene shown to be critical for heart development was discovered in the fruit fly. This gene was named “tinman” because flies with mutations in this gene don’t have a heart (just like the Tinman in The Wizard of Oz). Researchers later found its equivalent in humans – NKX-2.5.

Flies are important to researchers because they can live in the lab for up to 9 weeks. One week in a fly lifespan equals about 10 years in a human lifespan. By using flies, researchers can study the effect of age on heart function without waiting 30 years.

Another example of how fruit flies have helped in the field of cardiology is that mutations known to cause Long QT Syndrome in humans (which can cause cardiac fibrillation and arrest in healthy young athletes) also cause cardiac fibrillation in fruit flies. Similarly, diets high in fat or sugar make flies fat and cause heart dysfunction.

So how does this relate to HLHS?

Our research program is examining the genes from family participants and studying impacted genes in fruit flies to see if there is a connection with heart function. We are very excited about this project and will keep you updated on the results!

All of this makes you look at fruit flies in a new light, doesn’t it?

The Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome (HLHS) is a collaborative network of specialists bonded by the vision of delaying or preventing heart failure for individuals affected by congenital heart defects including HLHS. The specialized team is addressing the various aspects of these defects by using research and clinical strategies ranging from basic science to diagnostic imaging to regenerative therapies.