Not long ago this little girl would have died. Texas Children's gave her the gift of life
Corrected on 8/26/07: An earlier version of the article "Saving Makenna's Heart" incorrectly described the effect of detaching one of two large veins to the heart and reconnecting it elsewhere. The effect was to reduce, not increase, the pumping burden on the heart's right side.
It is a steamy summer afternoon in Houston, where 4-year-old Makenna Franks has been in open-heart surgery at Texas Children's Hospital for more than five hours. All went well. Now the groggy little girl is wheeled into the cardiovascular intensive-care unit, where nurses crowd around her and exchange greetings with her parents, Brandi and Bobby Franks. Like many of the more than 20,000 kids admitted each year, Makenna has been here before. This is her third major heart surgery. The goal of this early August visit is to make it her last one.
She was born with hypoplastic left heart syndrome, a condition affecting about 1 in every 3,000 to 6,000 babies in which the left side of the heart and the aorta carrying blood to the body fail to develop properly. Makenna's left ventricle—the main pumping chamber—was all but absent, two valves were defective, and her aorta was more of a soda straw than a pipe. Just 10 to 15 years ago two options were available for most babies like her: Take them home and keep them comfortable while they lived out their few days or weeks, or give them a new heart. Realistically, the outcome in either case was death, given the tiny supply of infant hearts and shortage of transplant expertise.
No longer. Charles Fraser, the Crocs-wearing surgeon who runs Texas Children's congenital heart surgery program, is among the handful of specialists who routinely keep HLHS babies, as well as others with serious heart defects, alive and thriving. The overall mortality rate at Texas Children's for children with such conditions is less than 2 percent—roughly the death rate at good centers for adult heart bypass surgery. And while HLHS babies may tire a little more readily as they grow than do other children and adults, the outlook for them is hopeful.
NICU on wheels. This hopeful outlook assumes that these kids wind up at a hospital like Texas Children's. Not all pediatricians recognize the condition, and arranging transportation or covering costs could be difficult. Makenna was lucky to be born at a suburban Houston hospital that has a close working relationship with Texas Children's.
Early in the morning after her birth in June of 2003, a pediatrician came in. "She was very quiet," recalls Brandi. "Then she mentioned a heart murmur. Then she said 'profound.'" That afternoon, Makenna headed for Texas Children's in an ambulance fully staffed and equipped as a rolling neonatal ICU, her parents behind her battling rush-hour traffic. The same night, doctors snaked a catheter into her heart to keep open a hole between the upper chambers that normally closes shortly after birth, to allow some oxygenated blood to flow.
Three days later, Makenna had her first major surgery—an open-heart operation called a Norwood, for the surgeon who first performed it in 1979—to cut and reroute major blood vessels. That relieved the defective left side of her heart from duty and bought her time to build up strength. A second operation was performed six months later in which the superior vena cava, one of two large veins to the heart, was detached and reconnected elsewhere, placing less of the pumping burden on the right side.
Fraser unquestionably had the chops to do Makenna's final operation. He came to Texas Children's in 1995 at age 37 after working in Australia and at the Cleveland Clinic with Roger Mee, a legendary maestro in the art of children's heart repair. "He was why I became a pediatric heart surgeon," says Fraser, a likable plain talker with four children of his own who reads the Bible every day but also worships at the altar of his alma mater, the University of Texas—the Longhorns' football team in particular.
Today Fraser would perform a Fontan (named for another innovator), separating the second large vein, the inferior vena cava, from the heart and extending it to the pulmonary artery with a piece of Gore-Tex tubing. The architecture of Makenna's heart and plumbing is now far from normal—but it is working well. "She's pink!" exclaims her mother, a first-grade teacher expecting twins, when she gets her first look at Makenna after surgery. "She is, isn't she?" marvels her father, a youth minister.
When Fraser was new to Texas Children's, the Norwood survival rate was zero. He did his first one there, on a newborn boy, the month after he started. He finished up and went to get a gurney, but one wasn't waiting outside as usual. "When I asked where it was," says Fraser, "they told me, 'What's the point? He's going to die anyway.'" That baby is now 12 years old.