When a child's heart fails
Date: February 28, 2011
The physician on Maryland’s Eastern Shore, Kevin Karpowicz, understood why the 7-year-old’s mom believed that her daughter might have appendicitis. After all, the child had been complaining of worsening stomach pain, fever and abdominal swelling. But Karpowicz found that the swelling was in the girl’s upper abdomen, close to the liver. He also became alarmed at her heart sounds, which displayed a prominent murmur. He ordered an abdominal scan and spotted an enlarged heart. Now it was Hopkins Hospital time.
When pediatric cardiologist Janet Scheel reviewed Allyson Fowler’s case in Johns Hopkins’ Emergency Department the following day, she found the heart enlargement so pronounced that one of her colleagues exaggerated its dimensions to make his point. “It’s the size of a small European country,” he said.
After an echocardiogram, Scheel knew she was seeing an advanced case of cardiomyopathy. Four of Ally’s heart valves were leaking, yielding a frightening fractional shortening of 7 percent, and her heart showed “a very prominent gallop.” Her pulse was racing to compensate for the leaks. Her liver had swollen in response. Her skin was clammy.
Over the ensuing days, Scheel and other members of the pediatric heart failure team amplified Ally’s cardiac output while trying in vain to identify its seemingly viral source. But the child’s condition was relentless. Two weeks after her admission, Ally became a priority candidate for a heart transplant.
But soon Scheel and pediatric cardiac surgeon Luca Vricella became more alarmed with mounting evidence of their little patient’s accelerating decline. “She was so weak,” Vricella explains, “she could not even open her eyes.”
When an adult’s heart declines, says Vricella, physicians have a ready pipeline for cardiac assist devices that can extend their lives for transplant. But the cardiac device market is not so generous with youngsters, partly because heart failure in children is such a rare occurrence. Both of the companies that make assist devices suitable for children are based in Germany, and neither system is approved for use in the United States by the FDA.
“So we have to get dispensation,” Vricella says, by making a compassionate use appeal to the FDA and key insurance groups. “I found my angel,” says Vricella, “in Kristen Nelson,” a pediatric critical care specialist who’s mastered the appeals process so effectively that the chosen assist package for Ally arrived within three days of Vricella’s request.
Vricella installed the assist device on July 14 in a three-hour procedure that bought Ally the extra window of time that she needed. Her new heart arrived on July 25. By Aug. 2, she was back home, after 42 days in the intensive care unit.
The case of Allyson Fowler provides a useful window on the Hopkins pediatric heart team’s world, say Scheel and Vricella. The focus of the group’s 15 cardiologists and other specialists has produced excellent outcomes for an unusual breadth of cases ranging from infants to adults suffering long-term consequences of childhood cardiac interventions.