Complex heart surgery saves life of tiny preemie

Jerrick De Leon’s heart may be only the size of a small grape, but in the field of cardiac surgery it’s one of the most important in the world. On Feb. 6, 1-week-old Jerrick became what surgeons at Lucile Packard Children’s Hospital at Stanford believe is the smallest baby ever to survive a type of open-heart surgery called an arterial switch procedure. Jerrick, who was born more than 13 weeks early, weighed only 700 grams, or slightly more than 1.5 pounds at the time of his surgery.

The successful operation marked a dramatic turnaround for Jerrick, who was airlifted to the hospital after doctors in Southern California gave him a zero chance of survival. Barring unrelated complications from his prematurity, Jerrick is now expected to have a normal lifespan.

Jerrick’s surgery was performed by pediatric cardiac surgeon V. Mohan Reddy, MD, who specializes in correcting heart defects in extremely low birth-weight babies. In 2001, Reddy successfully performed a different cardiac repair on a newborn Serena Brown, who weighed only 640 grams at the time of her surgery. At the time she was believed to be the smallest child ever to undergo open-heart surgery.

“Babies this premature are very small and very fragile, with extremely delicate tissue,” said Reddy. “It’s necessary to scale down your hand and arm movements to achieve a very fine degree of accuracy.”

Reddy, who is chief of pediatric cardiac surgery at Packard Children’s Hospital and an associate professor of cardiothoracic surgery and of pediatrics at Stanford’s School of Medicine, added, “Everyone involved in the operation is extremely cautious and the procedure goes very slowly. What would normally take three hours often takes four to five in a baby this small.”

Reddy also performed the operation without stopping Jerrick’s circulation—allowing blood to continue to bathe his fragile brain during the procedure but substantially increasing the technical difficulty.

“Jerrick is doing far better than expected,” said his mother, pediatrician Maria Lourdes, “especially since he was originally given no chance of survival. This miraculous surgery has corrected his heart problem. Now I’m just looking at a premature baby. I don’t know how to express how thankful I am.” Although Lourdes is a physician, she was not directly involved in her son’s care.

Jerrick’s premature birth at 26.5 weeks gestation was necessitated by his mother’s severe pre-eclampsia, a dangerous spike in a pregnant woman’s blood pressure that can be fatal to both mother and baby. After his birth in a Southern California hospital by cesarean section, Jerrick’s doctors noticed the carbon dioxide concentration in his blood was higher than normal. An echocardiogram pinpointed the reason.

The heart normally pumps blood throughout the body in a kind of figure-eight pattern, with the heart in the center: blood circulates first to the lungs and routes back through the heart for an extra push before traveling through the rest of the body, and then returning again to the heart before heading back to the lungs.

In Jerrick’s case, the two arteries routing blood out of the heart were switched during development, severing the connection between the two loops of the figure eight. Oxygenated blood cycled uselessly between the heart and the lungs while the body was bathed in oxygen-poor blood. A naturally occurring connection between these two arteries was the fragile link keeping Jerrick alive, but it normally closes shortly after birth in full-term babies.

Jerrick’s condition, known as transposition of the great arteries, occurs in about 40 of every 100,000 live births. Prenatal diagnosis via a specialized ultrasound is technically possible, but rare. Surgery is always required to correct the defect, usually within the first two weeks of life. However, it was believed that Jerrick was too small and premature to undergo the difficult fix, which involves switching the arteries back to their correct positions.

Waiting for Jerrick to grow wasn’t an option; doctors considered it unlikely that he would live long enough to gain the needed weight and strength. But after delivering the grim news to the baby’s parents, the surgeon in Southern California did more research and discovered that Reddy had success treating extremely tiny infants.

Jerrick was airlifted to Packard Children’s Hospital on Feb. 4, two days before his surgery. The procedure went well enough that Jerrick was recently moved from the hospital’s specialized cardiac care unit to the neonatal intensive care unit, where he can recuperate and gain weight with other tiny preemies.

“A lot of pediatric cardiologists and neonatologists think these kids are inoperable, but I believe that is a myth” said Reddy. “We have the most experience in the world operating on these extremely low birth-weight babies. This experience, coupled with our ongoing research on fetal surgery, gives us an edge when treating children like Jerrick.”

Lourdes’ view of the situation is colored both by being a pediatrician and a mother. “I know all the risks of having a premature baby; I’ve taken care of smaller infants during my residency training,” she said. “But when the pediatrician was telling me about Jerrick’s heart defect, it just wrecked me emotionally. I couldn’t feel anything. Now, as a mom, I feel that my baby is going to be OK.”

From Stanford Medical Center

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