Grow Fierce: Saving Olivia

Olivia Belfonti wasn’t even born yet when her parents, Tricia and Arnold, knew there was trouble—serious trouble—with their baby. Tricia was 19 weeks pregnant when an ultrasound showed that Olivia’s heart had only one ventricle. Instead of the normal arrangement, where one side of the heart pumps oxygen-rich blood from the lungs to the rest of the body and the other side pumps oxygen-depleted blood back to the lungs to pick up new oxygen, Olivia’s heart had only one big chamber.  “We were told by another hospital here in Connecticut that Olivia’s diagnosis was ‘incompatible with life,’ and we should ‘consider our options,’” says Tricia.

But then the Belfonti’s pediatrician suggested they talk to Felice Heller, MD, a cardiologist at Connecticut Children’s. “Dr. Heller said, ‘We treat these all the time,’” Tricia says.

Connecticut Children’s is, in fact, one of the top hospitals in the country treating congenital pediatric heart defects, according to U.S. News & World Report. It is, perhaps, not surprising that the first hospital thought that Olivia’s condition was unsurvivable: Until the 1970s, there was no method for correcting the problem. But then a French surgeon named Fontan developed a technique that worked in many cases, and surgeons have been improving upon the procedure ever since.  That’s the procedure that Olivia underwent. Because a baby is still growing, the process happens in three stages during the first four years of life. The first surgery, which Olivia had when she was 48 hours old, places a shunt in the system, a small tube that creates a back door for blood to flow into the lungs. The second surgery takes place between two and four months of age, when the blood return from the upper body is connected to the lungs. The final surgery, typically performed between the age of 2 and 4, connects the body’s lower blood supply to the lungs. All the procedures were performed by Connecticut Children’s cardiovascular surgeon Dennis Mello, MD.

The goal, says Dr. Heller, “is to have the blue blood bypass the heart altogether and go straight to the lungs, then let the single pump that is there send the oxygenated blood to the rest of the body. The pressure in the veins goes up a bit to push the blue blood to the lungs, but somehow it works.” And certainly it’s worked for Olivia.

Today, she is a student at St. Paul High School in Bristol, where she’s taking all honors courses. She’s active in student government and the social media task force. And she’s on the school cheerleading squad—a remarkable testament to the effectiveness of the treatment she got at Connecticut Children’s and a far cry from the “incompatible with life” prognosis her parents first received.

That’s not to say it’s all smooth sailing. Children born with this condition who undergo this procedure have to be cautious about many otherwise ordinary aspects of life. Alcohol is not an option, pregnancy is a very high-risk proposition, and any new medication or medical treatment needs to be coordinated with the pediatric cardiologist. Perhaps most serious is the fact that children who have this procedure are prone to cirrhosis of the liver. “Olivia will always need cardiac care,” Tricia says, “and eventually she will need additional work done on her heart. She also sees hepatologist Karan Emerick, MD, who is monitoring her liver, which has started to show signs of fibrosis (a lead-in to cirrhosis of the liver). That diagnosis last summer, while we expected it eventually due to her anatomy, rocked us to our core.” And Olivia has a leaky valve in her heart that may need additional surgery. But within the limitations of her condition, she can expect to live a full and relatively normal life. In fact, some patients who had this procedure in the early days are now in their 50s, and the number of survivors of congenital heart defects grow every year.

The survival of so many babies means that there is now a very large cohort of adults with unusual hearts that baffle adult cardiologists. Not only do these cardiologists not recognize the structure and function of these unorthodox hearts, but they also don’t understand the many medical considerations that come with them. This situation creates an entirely new field of medicine: adult congenital heart disease specialists—pediatric or adult cardiologists with special training who follow these patients through their adulthood. And Connecticut Children’s is on the leading edge of this field, too, with two of the first certified adult congenital heart disease specialists in the country.  They now treat more than 70 Fontan patients of all ages.

And that’s very good news for Olivia, who will be cared for the rest of her life. It’s also good news for her mother, Tricia, who wrote the following letter on the first anniversary of her final procedure:

“A year ago there was a little girl who was 3. She was very limited in her activities. She couldn’t walk up the driveway. A flight of stairs would leave her winded. Blue lips and purple fingers and toes were the norm. March 24, 2008, changed all that. That was the day of Olivia’s final surgery at Connecticut Children’s. Within two weeks, she was running up the driveway. She could sprint 3/4 of a mile. She runs down the driveway, arms extended, and shouts, “I can fly!” while her blonde hair flies behind her. The little girl who was limited now takes swim lessons and attends gymnastics class. She has boundless energy. She bounces and twirls around the house nonstop to the point that we actually ask her to stop jumping. And then we laugh and rejoice that she can jump and twirl. Life is good.”

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