February 14 is, of course, Valentine’s Day, a romantic celebration in an otherwise gloomy month. But, it also marks Congenital Heart Defect Awareness Week (February 7-14), an opportunity to learn more about the most common category of birth defects, and the ones most likely to result in infant death. About 1 percent of newborns have a heart defect, and about one-third of those are serious or life-threatening.
Emma was born in St. Johnsbury on an early January afternoon, the much anticipated first child of a healthy young couple. The pregnancy had been uneventful, and local prenatal screening had not identified anything of concern. She was born crying and vigorous, weighing almost 8 pounds and perfect in every way. The delivery room nurse dried her skin, wrapped her in warm blankets, and handed her to her mother. Within a few minutes, however, the nurse became concerned. The transient duskiness often seen in a newborn wasn’t resolving. The nurse blew oxygen in Emma’s little face, but her skin grew darker. She was rushed to the nursery and the pediatrician was called. A measurement of her blood oxygen level was 77 percent, instead of the normal 97 percent. Her doctor consulted a neonatologist at The University of Vermont Children’s Hospital at the UVM Medical Center, and the neonatal transport team was dispatched to bring Emma to Burlington.
When the transport team arrived a couple of hours later, Emma’s oxygen level had dropped below 70 percent. Suspecting a serious heart abnormality, the transport nurse inserted an IV and began an infusion of prostaglandin E1, a medicine that can relax a large arterial connection present in babies before birth, but which closes spontaneously after birth. Enlarging this vessel can often restore blood oxygen levels in babies with heart defects, and Emma’s rose back toward 80 percent within minutes. The University of Vermont Children’s Hospital NICU and cardiology teams were notified, and after brief additional stabilization, and a quick goodbye to her mother, Emma was on her way to Burlington.
During transport Emma was alert and calm, sucking quietly on a pacifier, but her blood oxygen level once again began drifting slowly down, and by arrival, despite her infusion, it had fallen to 60 percent. An echocardiogram confirmed a serious heart defect. The major arteries were connected to her heart in an abnormal way, circulating oxygenated blood back to the lungs and blue, un-oxygenated blood back to the body. The only way for oxygen to get to her body was through the vessel opened by the prostaglandin infusion, and this mixing was clearly inadequate. Emma immediately had a breathing tube placed, and she was deeply sedated. A small catheter was inserted high in her leg, and threaded up into her heart. Guided by ultrasound, a balloon on the tip of the catheter was filled with about a half a teaspoon of saline, than forcefully pulled through a divider in her heart to tear a hole and allow more oxygenated blood to get to her body. Her blood oxygen level promptly rose to 90 percent and remained there. Two days later she underwent corrective surgery, cutting and reattaching her major arteries in the proper position. She was home within two weeks.
Today, Emma is a healthy 10 year old, playing soccer and basketball, and, except for a thin, pale scar on her chest, just like all the others in her fourth grade class. She, and many others, are the beneficiaries of remarkable progress in infant heart care. Had she been born 50 years ago, she would have died within 24 hours. Today, for the first time in history, there are more adults living with a history of major heart defects than there are children.
Valentine’s Day is the perfect time to recognize this success, and to celebrate tens of thousands of mended little hearts.
Scott Yeager, MD, is a pediatric cardiologist at the The University of Vermont Children’s Hospital at the University of Vermont Medical Center and a professor at the Larner College of Medicine at UVM.