Tina Morton knew something wasn’t right in the 19th week of her pregnancy.
Last year, Morton, 32, was sent by her obstetrician for a routine ultrasound.
“We were excited to find out if we were having a girl or a boy,” she remembers thinking as the assistant rubbed the transducer across her belly.
But then the assistant stopped, and the mood in the room changed. The technician called Morton’s doctor.
“She couldn’t tell me anything, legally, but as a mom you know. I was listening to some of the terminology, and my husband looked at me and said, ‘What’s wrong?’ I said, ‘Something is wrong with her heart.’ ”
Morton is a nurse. She knew she would need a field echo ultrasound to check the tiny baby’s heart. She wound up with a team of cardiac doctors at the Heart Institute of Joe DiMaggio Children’s Hospital in Hollywood. Daughter Addison, now 5 months old and thriving, was born with a malfunctioning left ventricle, and underwent open heart surgery when she was 10 days old.
Today, advances in early detection and treatment for children with heart disease allows for more happy outcomes like Addison’s.
“We now can actually look at the fetus heart rate as early as 12 weeks,” said Dr. Kak-Chen Chan, chief of pediatric cardiology at DiMaggio’s Heart Institute. “We have certain screening views that allow technicians to get an idea, a landscape, [to tell] are we dealing with a fetal heart that is likely to be normal or abnormal.”
The hearts, at that early stage, are not even the size of a nickel. Chan treated Addison.
“Before [Addison] was born we knew what was going on with the cardiac team. And my doctors all had meetings, so we had a course of action, so to speak. She was born and they were all ready to go,” Morton said from her home in Pembroke Pines.
“She came out perfect and pink. I would have gone home, been discharged with her, and she would have crashed at home had I not known because of them being so proactive doing this testing ahead of time. It takes an hour out of the time you’re pregnant.”
Baby Addison’s story is a familiar one now, experts say.
“It’s a whole lot more common than most people realize,” said Dr. John Rhodes Jr., director of cardiology at Miami Children’s Hospital. “The number of patients that have heart disease that are born every year . . . is not increasing. But we know more about it, and have a better way of treating it. There are more therapies for children that otherwise wouldn’t have those options.”
Among these options: Heart valves that can be implanted via a catheter, rather than through open-heart surgery, to treat patients with valve disorders.
“There is a lot of opportunity to do preventative care and do things in less-invasive ways,” said Rhodes.
Miami Children's is participating in a study to assess the safety and effectiveness of a heart valve in young patients with a dysfunctional right ventricular outflow tract, or RVOT. Doctors there implanted three young patients with the valve in 2013. A similar valve has been used via the aorta in senior citizens. Miami Children's uses the pulmonary valve in children and young adults. By summer 2015, Miami Children’s expects to release its data to the FDA for approval of the Edwards SAPIEN transcatheter device, Rhodes said.
Rhodes likes the Melody valve, by Medtronic, an FDA-approved transcatheter pulmonary valve used to treat children with RVOT disorder. Each year, about 7,000 children are born in the United States with this disorder, he said.
To visualize the problem: When the ventricle is not working properly, the blood that travels from the heart to the lungs washes back and forth like in a washing machine. The valve doesn’t close, and the problem leads to shortness of breath and fatigue. Patients are usually born with the disorder; adults tend to have aortic valve issues that are lipid-related.
The Melody valve is on a stent and implanted via the leg. “They are better by the next day,” Rhodes said of patients who receive the device, citing two recent procedures. One was performed seven years ago on a child in Miami who was 11. He is now 18 and attending culinary school.
“At 11, this kid was not able to go to school. Can’t play on the playground for PE. Can’t run like other kids and gets picked on by other kids. This is life-changing for this small group of people,” Rhodes said.
Janet Romano’s son, Dylan, 6, also received the Melody valve.
Dylan, who lives with his parents and older brother in West Palm Beach, got his in September. He was born with a malfunctioning pulmonary valve, as well as two holes in the heart, and underwent his first open-heart surgery at Miami Children’s when he was 2 weeks old. With his new valve, which will help him stave off further heart surgeries until after age 10, “he’s an active boy who plays soccer and chases his brother around,” his mom said.
Other treatments include the Impella Device. This one allows the blood in the main pump of the heart to be pulled out and deposited in the main artery so that the heart does not need to work as a pump. The Impella uses a miniaturized flow pump fitted onto a pigtail catheter. This directly unloads the left ventricle and delivers blood to the ascending aorta to simulate the normal physiology of the heart.
When there is a hole in the heart, the most common heart defect, a GSO Gore Patch can be used to close a hole in the upper chambers of the heart, a disorder known as an atrial septal defect, not unlike one of the problems Addison was born with last year. The newer version of the patch has a softer frame, and can be placed via the leg vein.
Extra cellular matrix (ECM) material, which comes from a pig intestine, can be made into a sheet and placed onto a patient’s heart. After eight to 10 months recipients have generated new tissue, and it grows along with them. “[It is] the holy grail of congenital heart disease,” said Dr. Frank Scholl, surgical director of the heart transplant program at Joe DiMaggio. “If we have something that can grow with the patient, that is phenomenal.”
The Heart Institute at DiMaggio performed its first transplant in December 2010. The hospital has performed 14 transplants since then, and is monitoring 10 other cases at centers in South Florida, said Chris Mashburn, director of DiMaggio’s transplant program.
“This population has the potential for a very good outcome,” she said. “The heart grows with the child, so they basically have no restrictions and can do any sport. They need a lifetime of followups with a dedicated center and immunosupresants, but in general they do exceptionally well.”
The advancements in pediatric heart care outpace the donor supply, Mashburn said. But doctors have the ability to keep young patients alive while they await a suitable heart. Waiting periods can range from minutes to a year or more.
One option is the Berlin heart, a ventricular-assist device that does the work of the heart and is worn outside the body. Patients must remain in the hospital while on the Berlin heart. One Berlin implant at DiMaggio was for a girl who was 2 months old.
“She’s learned to sit up and do all her development milestones despite the fact [she] has a device outside the body,” Mashburn said.
Dee Martinez tells the story of her son, Nathan, DiMaggio’s 13th transplant patient, who just turned 14. The Weston boy underwent the procedure in November. Unlike most transplant patients, Nathan’s family was previously unaware that he had a heart problem. In one month’s time, he was on a transplant list.
“Nathan was born with a rare form of muscular dystrophy, and he almost died seven times the first year,” Martinez said. “He’s a fighter. They told us he’d never walk. I said, ‘No. My child will walk. He will play baseball.’ No one told us to follow up with a heart condition, so we let him go.”
But on Oct. 8, Nathan called his mom on his cellphone from his bed because he couldn’t breathe. Mom whisked him to Cleveland Clinic, where he was stabilized. An examiner there determined something was wrong with his heart. Cleveland recommended treatment at DiMaggio.
The next day, DiMaggio’s cardio team told a surprised Martinez that her son would need to be evaluated for a heart transplant. “He was a healthy kid 12 hours ago, what’s going on?” she said.
The evaluation found that Nathan’s heart had been damaged by the muscular dystrophy. He went on the transplant list on Oct. 30, and was discharged with medication. A week later, he was going downhill fast. Nathan told his mother he was afraid to go to sleep because he was frightened he might not awaken.
Nathan was admitted to the hospital on Nov. 6, and was being prepared for a Berlin heart on the 8th as his condition worsened. Mashburn walked into Nathan’s hospital room to tell the family what to expect with the Berlin heart. Suddenly, her phone rang. A donor matched.
“This is the phone call we’ve been waiting for,” Mashburn alerted the parents.
Nathan had his surgery on Nov. 10. When he came to, he expected to find a Berlin heart. When he found out he had a real heart, he placed his hand upon his chest and wept. Six days later he was home, and back in school in January.
“He had one slight episode of small rejection, but other than that he’s called a poster child for transplanting,” Martinez said.
Today, Nathan is playing baseball with his friends, his mother said. “He hasn’t been able to do that since he was 5 or 6. He’s doing things he was never able to do. He’s a different child every day.”
Martinez finds herself looking at her son, who will pass the eighth grade at Falcon Cove Middle School despite missing three months of school, and she says: “Where did you come from?” She laughs. Nathan was able to see and hold his original heart after his surgery.
“All kinds of good stuff is going on clinically,” Scholl said. “We’re able to care for patients that have any type of congenital heart defect, from fetus to adult. Transplant is the beginning of the rest of their lives.”