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Life-Saving Surgeries for Rare Heart Defect

Young Joe DiMaggio Children's Hospital Patient Is Healthy and Active After Cardiac Surgeries


December 2007 — Several hours after giving birth at Memorial Hospital West, Sandra and her husband Peter learned that their 10-pound baby boy had breathing problems and was put on a ventilator. Christian had tricuspid atresia, a rare heart defect in which the tricuspid valve is closed or absent, resulting in a lack of blood flow to the lungs. "Our baby was transferred toJoe DiMaggio Children's Hospital for heart surgery the next day," Sandra recalls.

Internationally renowned Richard Perryman, MD, Chief of Cardiac Surgical Services, performed the delicate procedure to insert a Blalock-Taussig shunt to improve blood flow. When Christian developed a discontinuity between the two pulmonary arteries a week later, Dr. Perryman put in a second shunt. Just a month old, Christian was then taken home. His medical care, however, was far from over.

Additional Surgery

Like most tricuspid atresia patients, Christian would need to undergo the Fontan procedure, a series of two open heart surgeries that help deliver red, oxygen-rich blood to the body and blue, oxygen-deficient blood to the lungs. The first surgery would be performed when Christian was 9 or 10 months old, and the second when he was 2 years old. Because Christian slept a lot and wasn't interested in eating, he received nutrients through a gastrostomy tube. At 4 months and 9 pounds, he had to be hospitalized and fed intravenously for a week. His skin was purplish from low oxygen levels.

When Peter's Navy leave ended and he returned to his post in Virginia, Sandra stayed with her parents so she could be close to Joe DiMaggio Children's Hospital. Follow-up care was provided by Orlando Arce, MD, pediatric cardiologist on the medical staff at Joe DiMaggio Children's Hospital, Memorial Hospital West and Memorial Hospital Miramar, and it helped Sandra to know he was just a phone call away. "I have his beeper number in my cell phone and know that I can call him any time with questions," she says.

After the first part of the Fontan procedure, Christian had more energy and his skin tone improved. He required speech, occupational and physical therapy, and today is thriving as he awaits the second procedure.

In fact, Sandra, Peter and Dr. Arce decided that Christian was doing well enough that the family could finally live together in Virginia. They will return to South Florida so Dr. Perryman can perform the final surgery.

The doctor says that while tricuspid atresia requires lifelong medical observation, Christian's circulation should be greatly improved after the next surgery.

A Bright Future

Whether further interventions will be required remains to be seen, but Dr. Perryman shares that one girl who underwent the same surgery became the fastest runner in her school. Sandra and Peter are grateful for Christian's medical team. "They explain everything," Sandra says. "The doctors and nurses always make sure we know exactly what's going on and that we understand . I love everyone at Joe DiMaggio Children's Hospital."

For referral to a physician for your child, please call Joe DiMaggio Children's Hospital Physician Referral Service toll-free at (866) JDCH-DOC. We're available 24 hours a day, 7 days a week.