Three members of the family - mother, daughter and son - are subsequently undergoing surgery to implant defibrillators to lessen the risk of sudden cardiac death due to erratic electrical heart function. The surgery for Mary Hill, the mother in the family, will take place at Saint Louis University Hospital next Monday, Sept. 29.
"I'm just glad this was discovered before anything more happened in our family," says Mrs. Hill, who has suffered a few bouts of rapid, irregular heartbeat and whose father developed atrial fibrillation in his later years. "It's odd that we're the first family in the whole world to be diagnosed with this condition, but we're glad it was identified and that it's treatable."
The condition is related to the duration of electrical activity during each heartbeat, called the QT interval, which can be measured in a standard electrocardiogram (ECG). "It's been well-known for many years that a prolonged QT interval can lead to sudden cardiac death," says cardiologist Preben Bjerregaard, MD, professor of internal medicine at Saint Louis University School of Medicine and Director of the Electrophysiology and Pacemaker Service at Saint Louis University Hospital. "But we've found that a persistent short QT interval may be just as deadly."
In 2000, Dr. Bjerregaard first published his findings about the Collinsville, Ill., family in Cardiology, a Swiss medical journal. And at the meeting of the American College of Cardiology earlier this year, he heard from colleagues in Germany and Italy who noted similar short QT intervals in two different families. In one of those families, six people had died suddenly.