Rontrell’s story: a family’s love faces down a rare heart condition

Demetriana and Randell Journee were thrilled when they learned that Demetriana was expecting their third child. The news came in the early days of the COVID-19 pandemic in 2020, when Demetriana was already five months along. Despite everything going on in the world around them, the couple felt that this was a blessing, and that a baby would make their family, which included an 11-year-old son and 4-year-old daughter, complete.

Because Demetriana was already five months pregnant when she found out she was expecting, one of the first ultrasounds she had was the anatomy scan, a comprehensive ultrasound typically performed at 20 weeks gestation to check on the development of the baby’s organs, bones, and overall growth.

It was during this ultrasound when the couple learned that their baby, a boy, had an irregularity with his heart.

“They could tell that he didn’t have all of the chambers, so they referred us to Children’s Hospital New Orleans to get a full workup,” Demetriana recalled.

The couple, who lives in Chalmette, Louisiana, made an appointment as soon as possible. Once there, they met with Dr. Shannon Powell, a pediatric and fetal cardiologist at Children’s Hospital New Orleans. After extensive tests and state-of-the-art imaging, Dr. Powell determined that their baby had a congenital heart condition called heterotaxy syndrome, which is characterized by complex congenital defects. In this case, these included right atrial isomerism (the right-left axis of the heart is malformed and the heart instead has two right sides), an unbalanced atrioventricular canal defect (meaning he was missing the middle parts of his heart and his left ventricle was small), double outlet right ventricle (in which both great arteries arise from the right ventricle) and d-transposition of the great arteries (the aorta and the pulmonary arteries are switched in position), as well as other structural abnormalities.

“This is an extremely rare condition and requires highly specialized care,” said Dr. Powell, who is also the Director of fetal cardiology at the Heart Center at Children’s Hospital New Orleans. “Because babies born with these abnormalities can have serious complications immediately after birth, our team created a comprehensive plan to monitor both the mother and her baby for the remainder of her pregnancy and to prepare for the surgeries the baby would need in the days and months after delivery.”

The Journees were in disbelief, but knew they would do everything they could to help their baby overcome the obstacles he would face in the first days and years of his life. The couple continued to see Dr. Powell throughout the remainder of the pregnancy, and they went to regular appointments at Children’s Hospital New Orleans to be monitored closely.

“We came regularly for stress tests with our obstetrician and to check his heart structures and cardiac function with Dr. Powell.” Demetriana said. “They also did a great job of preparing me for everything that was to come, including what would happen immediately after the delivery.”

On November 17, 2020, Demetriana gave birth to a beautiful baby boy, Rontrell Demetrius Journee. Because she delivered at her local hospital, Rontrell was airlifted shortly after his birth to Children’s Hospital New Orleans where he was placed under the care of experts in the cardiac intensive care unit (CICU).

“I was able to spend a few moments with him before the helicopter arrived, which I was so thankful for,” Demetriana said.

During that first week, as Demetriana recovered from her C-section, she stayed in close contact with her husband and her other two children, all of whom already loved baby Rontrell with their whole hearts.

“His siblings were both so worried about him,” Demetriana recalled. “My oldest understood more about what was going on and we reassured him that he would be okay. That was a very difficult first week.”

The team of specialists continued to monitor Rontrell closely in the CICU. Then, at just one week old, Rontrell had his first major surgery. Performed by Dr. Timothy Pettitt, pediatric cardiothoracic surgeon at Children’s Hospital New Orleans, the surgery aimed to limit some of the extra blood flow in his pulmonary artery.

“In this surgery, we place a band around the pulmonary artery to reduce the diameter of the pulmonary artery, which reduces blood flow and helps limit pulmonary over-circulation and pulmonary edema,” Dr. Pettitt explained. “We sometimes perform this procedure as the first stage of palliation.”

The surgery was a success, and it provided Dr. Pettitt and the surgical team precious time for Rontrell to grow bigger and stronger before his next stage of palliation with his second open-heart surgery.

Rontrell was able to go home and live with his family between the two surgeries. He remained on low levels of oxygen and was monitored closely, but the Journees were beyond happy to have their youngest son home at last.

“We watched him carefully and he had routine appointments with his cardiologist, but otherwise he was very much like a normal baby,” Demetriana said. “He was eating well and I enrolled him in an early steps program to make sure he didn’t fall behind on milestones.”

A little over six months after he was born, it was time for him to undergo his second of three planned surgeries. On June 8, 2021, Dr. Pettitt performed a bilateral bidirectional Glenn procedure, where he directed the blood flow from the upper body into the lungs directly, rather than having that blood go through the heart first and then into the lungs.

“The purpose of this procedure is to help offload the heart by allowing the blood from the head and upper extremities to passively flow to the lungs,” Dr. Pettitt explained. “This surgery precedes his final surgery, the Fontan operation, planned at 3 to 5 years of age. This surgery involves directing the blood flow from the trunk and lower extremities to the lungs as the last stage of offloading the heart. Blood will then effectively be pumped to the body by his heart but will passively get to the lungs.”

Just as he did after his first surgery, Rontrell recovered well. He’s had some setbacks, including vocal cord paresis (a known risk after a procedure such as this), becoming ill with both COVID-19 and RSV in the first year of his life, and other concerns that his cardiologists are following closely. He was also born without a spleen, also not uncommon in babies with heterotaxy syndrome, and will need to be on some medications for the rest of his life. But despite these challenges, Demetriana is confident that her baby is in the right place, getting exactly the care he needs.

“Children’s has been amazing,” Demetriana said. “The entire team of doctors, physicians, nurses—everyone has gone above and beyond for us.”

Demetriana said she will be forever thankful for the lifesaving care Children’s Hospital of New Orleans provided them, and for the youngest Journee, who turns two this November, and who made their family complete.

“Rontrell is doing great. He walks, he talks, and we’re doing potty training right now,” Demetriana said. “He’s very independent—he likes to do things for himself, feed himself, and he’s a climber. He tries to do everything any child would and I definitely have to monitor him.”

In a few years, Rontrell will undergo the Fontan procedure. If all goes well, it will be his final major surgery to palliate the structural abnormalities he was born with. And although he may never be able to run as fast or as far as other kids his age, Demetriana is confident that with the love of their family, and the support of the experts at Children’s Hospital New Orleans, he’ll be able to conquer any challenge that comes his way.

“If you look at him, you would never be able to tell what he’s been through,” Demetriana said. “He’s extremely strong and has been able to overcome all of his obstacles. And we know this is just the beginning.”

To learn more about the Heart Center at Children’s New Orleans, please visit: https://www.chnola.org/our-services/heart-center/