Rafa and Valerie knew little about congenital heart defects as they started a meeting with pediatric and fetal cardiologist Courtney Wein.

They were expecting their first child and midway through their pregnancy during the anatomy scan, the sonographer spotted an abnormality with their baby’s heart. The family was referred to Johns Hopkins All Children’s Fetal Care program. 

Wein, who works in the Fetal Heart program at Johns Hopkins All Children’s Hospital in Florida, met the family and performed an expert evaluation of the fetal heart. She discussed with the family that their baby had a cardiac malformation called coarctation of the aorta that would require a cardiac surgery shortly after birth.

“She was the first person to really explain it to us,” Valerie says. “She walked us through what it was and helped us get a grasp on what was happening, despite our initial shock and confusion. I loved her from the beginning. She explained the diagnosis in a way we could understand and was also aware of the emotional impact it had on us. I felt like she was taking care of me as well as our son.”

Fetal Heart Program

Congenital heart defects are the most common birth defect, affecting about 40,000 births a year in the United States.

The Fetal Heart program at Johns Hopkins All Children’s is designed to identify congenital heart conditions during pregnancy and develop a treatment plan, bringing together any specialists necessary for the pre- and post-natal care. Fetal Heart services during pregnancy are available in Brandon, New Port Richey, North Port, St. Petersburg, Sarasota and Tampa.

Rafa and Valerie, who live in Odessa, Florida, began to meet with Wein every four to six weeks at the Johns Hopkins All Children’s outpatient care location in Tampa. During these visits, Wein would monitor fetal cardiac development in order to help formulate the best delivery plan for the family.

“I really had an opportunity to meet with this family on multiple occasions and monitor the progression of the cardiac malformation” Wein says. “This family has always been fabulous. They were very eager to understand and learn how they could best provide for their future child.”

“I have nothing but amazing things to say,” Valerie says. “It must be so incredibly hard to do her job, and she does it so well. From the beginning, I felt I could trust her. I don't know how we got this lucky to have her as our son’s doctor. If you have to go through something this difficult – you want someone like Dr. Wein on your team.” 

Lucca’s Conditions 

Baby Lucca was born Dec. 13, 2024, weighing 7 pounds, 1 ounce. There were several aspects to his congenital heart disease. He was noted to have a hypoplastic aortic arch with a coarctation of the aorta, bicuspid aortic valve and multiple muscular ventricular septal defects.  

The aorta is the largest artery in the body. It forms an arch at the top of the chest and carries oxygen-enriched blood from the heart for circulation throughout the body. Hypoplastic aortic arch means the arch is too narrow. The condition often is related to coarctation of the aorta, which means a discrete narrowing of a specific area of the aorta. These narrowed areas obstruct blood flow from the heart to the rest of the body. The ventricular septal defects are holes in the wall between the bottom two chambers of the heart, they allow blood to pass from the left to right and can lead to heart failure. All these conditions require surgical correction in such a small infant. 

When babies such as Lucca have these conditions in the womb, it is not a problem because they receive oxygen from the placenta through the umbilical cord. A channel known as the ductus arteriosus exists between the aorta and the pulmonary artery, which allows the baby’s blood to bypass the lungs. Typically, that hole closes naturally shortly after birth. 

Because of the conditions restricting Lucca’s blood flow to the body, doctors anticipated the need for a medication that would keep the ductus arteriosus open until his aorta could be surgically repaired. 

“That ductus arteriosus is really important because it helps to keep blood flow going to the lower half of the body when a coarctation is present,” Wein says. 

The Surgical Repair 

Lucca was 6 days old when he had open-heart surgery. It was a hard day for Rafa and Valerie. 

“Saying goodbye to him in the hallway as they were wheeling him into the operating room was the hardest thing I've ever had to do,” Valerie says. “That day was the longest day of my life.” 

James Quintessenza, M.D., is co-director of the Johns Hopkins All Children’s Heart Institute and chief of cardiovascular surgery. He has more than 35 years of experience as a pediatric heart surgeon. The plan was to proceed with a somewhat high-risk complete repair as the best chance for the baby’s survival. 

Working with Allison Stitsinger Welch, PA-C, Quintessenza proceeded with the open-heart procedure to reconstruct the entire aortic arch, including the severely narrowed segment and closed the significant holes in the heart chambers. “Operating on a baby this small is a challenge and requires precise teamwork,” Quintessenza says. “Our team performed really well in caring for Lucca and his family.”  

Recovery and Moving Forward

Heart surgery at 6 days old brings challenges for the baby and parents. Lucca needed to be on a ventilator for a few days after the surgery as part of his recovery. At one point, he did develop an arrhythmia, a fast heart rate. The cardiovascular intensive care unit team quickly and efficiently responded to stabilize him.

“There were many moments of high stress, fear and doubt for us as parents,” Valerie says. “But the doctors and nurses did a great job explaining things to us as they were happening and reassuring us that these setbacks weren't necessarily setbacks. They were a normal part of the healing process.” 

Surgical repair of a hypoplastic aortic arch, coarctation of the aorta and closure of multiple ventricular septal defects has significant risks but a high success rate. Lucca went home on Jan. 1 just 13 days after his repair. He is now off the oxygen, and Wein expects by February he will begin transitioning off his remaining medication. 

Rafa and Valerie particularly credit several nurses, including Valentina Linhart, Rebecca Dorsey, Kaitlyn Bush-Bartlett, Jordyn Smith, Meagan Viteri and Taryn Spence, as well as doctors Arabela Stock, Reema Patel, Christine Diiorio, Joy Johnson, Kenneth Coca and physician assistants Allie Welch and Jamie Andre with patiently providing the support and reassurance they needed every step of the way. 

“We will never forget the way in which they took care of Lucca and us as parents,” Valerie says. “These nurses and doctors have the biggest hearts and brains. They got us through the hardest days of our lives, and we are forever grateful.

“It feels to me like people who work with kids in this way are a very unique and special breed of human. Everyone was so warm and loving. I could tell that it wasn’t just a job for them. They felt genuine love, care and concern for our son.

“I feel so grateful that we had Johns Hopkins All Children’s nearby, and that they have the necessary technology, equipment, services and people available to heal our baby’s heart. Whatever their HR department is doing to find and hire these nurses, team members and doctors is working wonderfully!”