Defining New Paradigms for Arrhythmia and Sudden Cardiac Death in Children

Lay Summary: Heart disease is the number one killer of Americans, and has been so for over 100 years. While major efforts in the biomedical sciences have focused on understanding the pathogenesis of cardiovascular disease in adults, far less attention has focused on efforts to diagnose and treat heart disease in babies and young children. For the past decade, our research program has focused on understanding cardiovascular disease in the young- particularly how defects in the cardiac electrical system can cause irregular heart beats (or arrhythmia), fainting, and even sudden cardiac death. Funding from the Saving Tiny Hearts Society has propelled these efforts. More specifically, funds from two years of Society Funding resulted in the identification of a new genetic form of cardiac arrhythmia, termed sinus node bradycardia in children in the US and across the world. Unlike typical children that display heart rates in utero of 150 beats a minute, the hearts of these children beat at 80 beats per minute. When these children are born, instead of having heart rates of 75 beats per minute, their rates are far too low at 38 beats per minute. Unfortunately, this inability to control heart rate may lead to syncope (fainting) and lack of blood to the brain. Funding from StHS has not only provided seed funding to understand this form of arrhythmia, but also resulted in a new genetic test for the disease. Children at risk of this disease may now be diagnosed even before birth and treated by the implantation of a cardiac pacemaker following birth. This wouldn’t have happened without support from StHS.

Just as important as this discovery is the use of these funds to train the next generation of scientists that will dedicate their lives to pediatric heart disease research. Countless fellows, graduate and medical students, and undergraduates have been impacted by these funds. These funds are the lifeblood of their training. For this we all thank you.