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The little boy is almost 2 years old now. And, if you happen to ask him who’s Superman, his little index finger will point to the place where his new heart beats.
It's anyone's guess whether Zach Shepardson really thinks of himself as the Man of Steel. What's for sure is, at just 22 months, the Milton toddler is part of a growing number who are surviving congenital heart defects. He will need life-long monitoring to insure that his heart continues to work well.
It used to be that babies born with serious heart defects like little Zach died in childhood. But, with the advent of such medical interventions as cardiopulmonary bypass, myocardial protection and other surgical techniques, that's rarely the case any more, said Dr. Brian Kogon, chief of cardiothoracic surgery at Children's Healthcare of Atlanta's Sibley Heart Center. Not only are children living longer, he said, they're entering adulthood.
That’s the good news. The bad news is that they’re needing care for that long, too.
“We’re now recognizing the subsequent consequences of our life-saving procedures,” Kogon said.
For example, Kogon said that the majority of children with valvular heart disease often need further treatment later on.
“The complex repairs done in childhood often do not last a lifetime and will need future re-repair or replacement,” he said in response to emailed questions. “In addition, the children with complex single ventricle heart disease that survive into adulthood will often develop heart and liver failure in their 30s and 40s. Some of these patients will go on to require heart transplant.”
It is for these reasons, he said, CHOA recently partnered with Emory Healthcare to launch the Congenital Heart Center of Georgia, one of the largest programs in the United States and the first in Georgia to provide continuous comprehensive care from before birth through adulthood.
Of the nearly four million infants born in the United States each year, approximately 3 percent have some type of birth defect, Kogon said.
Indeed, congenital heart defects are the most common birth defect, occurring in about one in 110 births, or nearly one percent of births. Thus, nearly 40,000 infants are affected each year in the U.S.
Last year alone, CHOA performed 880 heart operations, Kogon said.
“We evaluate more than 35,000 children with suspected or confirmed congenital and acquired heart defects,” he said. “Children’s has performed 292 heart transplants since the program’s inception in 1988 — and 21 in 2013 alone — making us one of the largest pediatric heart transplant centers in the country.”
Kogon said the hospital treats the entire spectrum of congenital heart disease, from the most simple to the most complex. That includes among other things congenital cardiac surgery, interventional cardiac catheterization, non-invasive imaging, and cardiac critical care. Patients also have access to specialized outpatient clinics that address cardiac genetics, heart failure, maternal/fetal issues, pulmonary hypertension and telemedicine.
“In conjunction with Emory, we have the ability to treat all aspects of congenital heart disease from fetal to adult life,” Kogon said.
Although studies show that approximately 40 percent of congenital heart disease patients stop seeing heart specialists between the ages of 13 and 21, Kogon said that current guidelines recommend lifelong care and observation for these patients.
Under the newly formed Congenital Heart Center of Georgia, he said CHOA's cardiac team will collaborate with physicians from Emory's Adult Congenital Heart Center to provide a more seamless transition from pediatric to adult care.
They also plan to educate patients about the importance of consistent monitoring and continued care so that they live longer, more productive and healthier lives.
“At all stages of a patient’s life and condition, we will also be able to offer the latest treatments and access to the most progressive clinical trials,” Kogon said.
That’s the source of much of the Shepardsons’ hope these days.
“We met familes at CHOA from as far away as Mississippi who will need this type of care,” said Zach’s father, John Shepardson. “We feel really blessed to live only a half hour away from the best place to get the care he’ll need over the long haul.”
Within hours of his birth on June 13, 2012, doctors diagnosed Zach with tricuspid atresia, an absent pulmonary valve, and heart block.
His heart was about the size of a walnut and his blood vessels were even smaller – about the size of the tip of sharp pencil. For weeks after his surgery, he was so sick his color was closer to purple than pink, and he was so weak he barely ever smiled.
Most patients with this type of heart disease proceed through a series of three palliative operations, but Zach’s heart function deteriorated to a point that the traditional pathway was not possible, Kogon said.
“We knew he would never come home without a new heart,” said his mother, Ashley.
“That was difficult to come to grips with,” John Shepardson added. “All you can do is pray for the donor family who must endure such loss for your baby to live.”
Just 39 days later on Dec. 6, it happened. Kogon traded Zach’s heart for a new one, and the little boy came home to spend Christmas with his mom, dad and two big brothers. For his first birthday, they gave him a Superman themed party.
As he toddled around his family kitchen last week, there were few clues to Zach Shepardson’s journey.
His skin color is normal. He no longer needs a feeding tube to eat. And when you ask him who’s Superman, he points to himself and a big smile marches across his face.
