The first thing you notice when you enter Nicole and Kevin Keller’s sunny home in Margate, Florida, is the clanging. Following the young couple into the kitchen, you find a bright-eyed, spirited 3-year-old pulling pots and pans out of the cabinets and dumping them onto the floor. Like any other boy his age, Garhett is having the time of his life amid the noise and shiny kitchenware.

But Garhett isn’t like other children his age. Born with a rare and complex heart disease called hypoplastic left heart syndrome (HLHS), he’s already undergone three major surgeries to remodel his heart at Holtz Children’s Hospital of the University of Miami/Jackson Memorial Medical Center. The first was three days after his birth, when his heart was barely larger than a walnut. The second took place at four months and the third at two years. And, although Garhett is doing well, the Kellers know their only child will continue to need lifelong follow-up care.

HLHS, which occurs in one of every 4,000 to 6,000 births, is a complex combination of heart malformations that prevents the left ventricle of a child’s heart, the main pumping chamber, from developing and carrying oxygenated blood to the body. Six years ago, a four-center study showed that only 42 percent of 231 infants with a heart defect like Garhett’s survived one year following surgery. Since that study, these odds have improved significantly. More than 98 percent of the 900-plus children surgically treated at the Children’s Heart Center at Holtz Children’s Hospital for all forms of congenital heart defects in the last five years have survived. During the same period, the cardiac-related survival rate for children who have undergone the first of three surgeries commonly performed for HLHS has been nearly 90 percent at the Children’s Heart Center, compared with 80 to 85 percent at most children’s heart centers, due in large part to the introduction of a modified version of the standard surgical procedure for HLHS.

Eliot Rosenkranz, M.D., associate professor of medicine at the Leonard M. Miller School of Medicine and chief of pediatric cardiothoracic surgery, who performed Garhett’s surgery, attributes the program’s success in correcting life-threatening heart defects in children to a number of factors. “One of the first things we did after I began my position as division chief was to cohort critically ill children with heart defects into a specialized unit. These babies often encounter some subtle and unique difficulties that the hospital’s Cardiac Intensive Care Team is better able to identify and then respond to in a prompt and successful manner.”

Rosenkranz also introduced state-of-the-art surgical techniques to Holtz Children’s Hospital’s pediatric cardiac operating rooms. The surgical team now does complete, rather than staged, repairs when possible. In addition, new procedures such as the modified HLHS operation—which involves putting a more reliable connection between the heart and the main pulmonary artery that provides blood to the lungs—have been introduced.

The expanding field of interventional cardiology, also called cardiac catheterization, now assists in the diagnosis as well as the treatment of heart defects, without major surgery. An interventional cardiologist inserts a thin plastic tube called a catheter through blood vessels in the child’s groin or arm and then guides this up to the heart. The specially trained cardiologists can open narrowed arteries, remove obstructions, and patch holes in the hearts and blood vessels of children even as early as the day of their delivery. Rosenkranz is particularly enthusiastic about “hybrid” surgeries in which a cardiac surgeon and interventional cardiologist collaborate, thereby reducing the time children spend on the operating table. He and other members of the cardiac team have shared these techniques with patients and health care workers in the Dominican Republic. The summer of 2005 marked their third visit there, all through Caribbean Heart Menders, a group of medical volunteers who treat children with heart disease and train local physicians.

Some heart defects take the form of abnormal heart rates and rhythms, known as arrhythmias. The Children’s Heart Center is a leader in pediatric cardiac electrophysiology—the field that addresses these life-threatening abnormalities. Ming-Lon Young, M.D., director of the hospital’s Pediatric Cardiac Electrophysiology program and the interim division director of pediatric cardiology, uses a technique called radio frequency ablation to save young lives by removing abnormal or extra electrical pathways within the heart.

Despite the advent of these remarkable surgical and medical interventions, which can extend life but can never fully restore these damaged hearts, some interventions remain impossible. In her first four months of life, Sophia Caceres seemed normal—sleeping, eating, and gurgling at her parents. Then her mother noticed Sophia had stopped growing and seemed unusually lethargic. Sophia’s mother, Claudia, brought her daughter to the Children’s Heart Center, where she learned that Sophia suffered from dilated cardiomyopathy (a condition characterized by unhealthy heart muscle and an enlarged heart) and heart failure. Without a heart transplant, this blue-eyed, black-haired infant whom pediatric nurse practitioner Ray Lavendera describes as a “little princess” would die.

At the Children’s Heart Center, children such as Sophia are referred to the Pediatric Heart Transplant and Heart Failure Unit. Paolo Rusconi, M.D., who directs the program, works aggressively to repair these tiny hearts with medication before resorting to a heart transplant. The sickest children are placed on the transplant waiting list, and roughly eight children a year have the procedure. Some of these children receive pacemakers or defibrillators. Some require artificial cardiac and pulmonary support from an external machine until a heart becomes available.

When Rusconi became director of the program in 1998, half of the children who came for cardiac transplants died. “There was a tendency to see the transplant as a last resort, so physicians tended to wait until a patient was extremely sick and on multiple intravenous medications and in multiorgan failure,” Rusconi says. “The first thing we did was create the heart failure program, a structure that supported patients with medication and nutrition until they improved or were well enough for transplant.” One medication, which Rusconi has pioneered, is carvedilol, a beta-blocker that has been very effective in adults with cardiomyopathy and congestive heart failure.

This more proactive approach has paid off; the one- and three-year survival rates for children with conditions similar to Sophia are 90 percent or greater, which compares favorably with the national average of 87 percent. Less than 30 percent of the children referred to the unit for a heart transplant evaluation and work-up end up actually needing the transplant.

The community physician who diagnosed Garhett’s HLHS in utero recommended that Nicole Keller deliver at Jackson Memorial Hospital. At birth Garhett was taken to the hospital’s high-risk neonatal intensive care unit and monitored by the pediatric cardiology team prior to surgery. This type of extensive collaboration among medical specialists is one of the keys to success in treating children with heart problems.

“Families are happier, outcomes are better, and costs are lower when pediatric cardiac care specialists work together as an integrated children’s heart center team,” explains Steven Lipshultz, M.D., who chairs the Miller School’s Department of Pediatrics and is chief of staff at the Holtz Children’s Hospital. “During the past few years we have developed an integrated Children’s Heart Center that is one of the few in the world to function this way.”

Faculty from every discipline meet weekly to determine the best course for managing each patient. In many cases, physicians also meet with parents before the child is born to assess the child’s heart defect and plan his or her delivery and surgery. The Children’s Heart Center has operating rooms for heart surgery, a cardiac catheterization laboratory, and a cardiac ICU.

“Pediatrics as a whole is not just dealing with the child, but dealing with the family and the way the disease affects the family,” says Steven Schultz, M.D., director of pediatric cardiac critical care. “When children get mixed up in the adult world as they’re receiving care, they find it’s not such a friendly place.”

“What makes this field so challenging is that every child with heart disease is born with a slightly different grouping of defects, so every medical treatment plan or operation is like a creative masterpiece,” Lipshultz says. “You shed many tears and hold many hands and make many promises. You really develop a covenant with these families that you have to do more, to keep pressing for answers to achieve optimal outcomes for these children by developing new preventive and therapeutic strategies.”

Joan Cochran is a freelance writer in Boca Raton, Florida.