The fetal monitor throughout the night hasn’t indicated that her heart is in distress, nor did a routine ultrasound exam several weeks earlier detect any cardiac abnormality. But the moment Taylor Keaveney is born on a rainy night in April 2010, the dark-blue cast of her skin and her ominously low blood-oxygen level reveal that her life is in peril.
Doctors and nurses at Reston Hospital Center rush the infant from the delivery suite to the neonatal intensive-care unit (NICU) so quickly that her mother, Amanda, exhausted from a long labor, barely catches a glimpse of her baby. Her husband, Joe, is shaken by the sight of his daughter but doesn’t betray his worry to his wife.
Dr. Sharon Karr is at a Virginia pediatric-society meeting in Tysons Corner when she answers a page and then calls the neonatologist at the hospital.
“We have a blue baby here,” the neonatologist tells Karr, a pediatric cardiologist. “We took x-rays, and it looks like she has a total anomalous pulmonary venous return.”
Most babies born with congenital heart defects—occurring in about 1 in 125 live births—are not in immediate danger. But of all the congenital heart defects, three can be true emergencies: a type of hypoplastic left heart, in which there’s only one pumping chamber instead of two; a tight aortic narrowing that can’t be opened with medication; and baby Taylor’s defect, a total anomalous pulmonary venous return.
I take a special interest in Taylor’s case and in congenital heart defects generally because I was born with one. At my request, the administration and medical staff at Inova Fairfax Hospital for Children have allowed me to observe all phases of its heart program. Over several months I witness heart surgeries, activities in the catheterization lab, and mortality-and-morbidity conferences. In those months, I observe the newest technologies for repairing young hearts—and reflect back on my own heart and how it was repaired 50 years ago this year. Medicine has come a long way.
After getting the diagnosis from the neonatologist, Karr quickly leaves her meeting, drives 20 minutes to Reston Hospital Center, and hurries to the NICU. The neonatologists have put Taylor on a ventilator and turned up the oxygen to 100 percent, but she remains dark blue with a blood-oxygen level of 50 percent when it should be in the 90s. Karr puts her stethoscope on the infant’s chest. The first thump of the heartbeat sounds normal, but the second thump is unusually loud.
She slides a wand-like transducer over Taylor’s frail chest to get an echocardiogram image of the heart and to study the flow of blood through the major vessels. Taylor’s left heart appears small and compressed, almost flattened; her right heart is dilated and not functioning normally. The echocardiogram confirms the diagnosis: The pulmonary veins from Taylor’s lungs to her heart never connected during fetal development.
This defect arose when Taylor’s heart began to form during the fifth week of her fetal life. This is the most critical phase of embryonic heart development, when a primitive tube of myocardial cells folds to create the heart’s four chambers.
No bigger than the head of a small nail at first, the heart continues to develop, and by ten weeks’ gestation its four chambers can be visualized by ultrasound. But a genetic glitch can malform the heart’s complex architecture—resulting in a valve that doesn’t open properly, vessels attached to the wrong chambers, a hole in the heart wall, or a condition such as Taylor’s.
“When you consider just how complex the heart is,” Karr says, “it’s almost a miracle that so many develop normally.”
It’s not surprising that Taylor’s heart defect was missed on a routine ultrasound months before her birth, Karr says. In such examinations, technicians usually confirm that the heart has four chambers and normal outflow vessels, but because the pulmonary veins are very hard to image, only a pediatric cardiologist well trained in fetal ultrasound is likely to have detected Taylor’s defect in utero.
Studying the images of Taylor’s miniature heart, Karr sees that the four pulmonary veins—two from the left lung and two from the right—come together in a confluence unconnected to her heart. Moreover, Taylor’s spaghetti-size pulmonary veins are tiny even for an infant, making any surgical repair that much more delicate. Karr knows Taylor will die, and soon, without surgery.
Around 9 pm, Karr calls Dr. Irving Shen, director of pediatric cardiac surgery at Inova, who is on call that night. In his four years at Inova, Shen has earned the trust of Karr and other cardiologists. She reaches him as he’s about to get ready for bed and tells him Taylor’s condition.
“She needs to go to the OR tonight,” Karr says.
Shen asks Karr to bring Taylor to Fairfax Inova and gets on the phone to assemble the cardiac-surgery team. Most are at home after having already put in a long day in the operating room.
In Amanda’s hospital room, Karr tells Amanda and Joe that their daughter is gravely ill. “She has to be operated on right away,” Karr says, “or we could lose her.”