Truncus Arteriosus: Evan's Fight to a Healthy Heart

Patient Evan at Johns Hopkins All Children's Hospital
Published in Johns Hopkins All Children's Hospital - Latest News and Stories
text hereEvan with Dr. Courtney Wein

After months of trying, Patricia and Chris found out they were finally pregnant. They were so excited to tell family and friends the news that they were about to be a family of three. What they did not expect, however, was that the 18-week anatomy scan would bring forward new concerns.

“When they came in and told us something was wrong with his heart, we were just completely shocked,” Patricia recalls. 

Doctors quickly referred Patricia to Courtney Wein, D.O., a pediatric and fetal cardiologist at Johns Hopkins All Children’s Heart Institute in St. Petersburg, Florida. Wein and her team performed a fetal echocardiogram for an in-depth evaluation of the baby’s heart and confirmed that he had truncus arteriosus. Truncus arteriosus is a rare and complex congenital heart defect where only one blood vessel (instead of two) comes off of the heart and gives rise to both the lung and body’s arteries. What started off as a difficult diagnosis at first transformed into a journey of mutual trust.

“Dr. Wein really made all the difference,” says Patricia, holding back tears. “She asked if we had a name for our baby boy and from that moment on, she always referred to him as ‘Evan.’ She said, ‘I’m a part of your team now. This is our baby.’ Dr. Wein promised us she'd be with us every step of the way, any day, any time — and she was.”

“Being a mother myself, I know how important this child is to them,” Wein says, adding that when delivering a congenital heart disease diagnosis, the team walks families through, step-by-step, with the utmost patience and care. “I try to give space so it can be a conversation. I show diagrams and discuss what the heart defect is. I give the family time to process and express their thoughts and feelings. Hearing this news is different from what you anticipate when you first learn of your pregnancy, so I want to be open to hear their thoughts and concerns as they digest the news. … No question is silly!”

Patricia and Chris say the Johns Hopkins All Children’s Heart Institute team was always available to their family. Wein and her team meticulously monitored Evan's development with monthly echocardiograms. Their care plan included meetings with surgeons, anesthesiologists, intensivists, nurses, social workers and other clinical and non-clinical teams.

“There was such a wealth of resources that were made available to us. When you're in that situation, you have no idea what to expect and you're in a kind of shock. So, the team at Johns Hopkins All Children’s really helped us navigate the entire process and feel as prepared as you can in that situation,” says Patricia. Now, all that was left was to wait for baby Evan’s arrival.

That day came in March 2023 when Patricia checked into Bayfront Health St. Petersburg’s Baby Place, located on the third floor of Johns Hopkins All Children’s Hospital. Once her induction started and Patricia had contractions, Evan’s heart rate began to drop. After about 24 hours in labor, the team decided it was in the best interest of Patricia and Evan that she proceed with a c-section. While the stork team waited in the wings to rush in if needed, Patricia and Chris were anxious to meet their son. 

They were relieved and overwhelmed that everything seemed normal upon his arrival and there were no emergent complications. 

“He had a full head of hair and was absolutely perfect,” Chris says. 

While Patricia recovered, Chris headed to the cardiovascular intensive care unit (CVICU) where the team cared for Evan, performing the scans and tests that the family knew to anticipate following birth. The team noticed in his first few days of life that Evan was working to breathe, as well as fatiguing overall and feeding poorly. At only 10 days old, with a heart the size of a walnut, Evan had his first open-heart surgery with James Quintessenza, M.D., A conduit (a specialized tube) was placed to create effective circulations to both his lungs and body. 

“Dr. Q was incredible, and we could not have been in better hands,” says Patricia. “Nothing phases him. We felt confident that nothing could happen in that surgical suite that Dr. Q wouldn't be ready for.”

Evan’s surgery was a success, however, only a few days later, he had a setback in the form of a pulmonary hypertensive crisis, which required expert resuscitation.

“As horrifying as it was, we were also amazed at how quickly and calmly the team reacted to get Evan stabilized” Patricia recalls. 

“It was nothing like how it is on TV. There was no running and shouting ‘stat.’ They were the definition of professional,” Chris adds. 

Following a few additional weeks of recovery, Evan was able to go home. While he was on a feeding tube and still needing time to heal, Wein continued to make sure his heart was recovering well.

Later that summer, Wein noticed Evan was showing signs of respiratory distress. She recommended that they proceed with a cardiac catheterization to determine the next course of action. James Thompson, M.D., an interventional cardiologist, discovered Evan had two large aneurysms pushing on his heart and lungs. 

“We both cried a little, but we were relieved that we caught this and were eager to move forward,” Wein says, recalling how Evan would have to proceed with another surgery much sooner than anticipated. “He is a rockstar though, and he did great!”

The family left the hospital 10 days later with their little fighter.

Today, Evan is doing great and every visit with his heart team ends with a smile.

“Evan’s developing well and meeting his milestones,” Wein says. “Our little rockstar has been able to successfully wean off the feeding tube and is doing fantastically.” 

His parents have been thrilled with his progress as well, adding that he loves being outdoors and never sits still. He's extremely alert and curious — always on the move, trying to explore.

“He has recently discovered the Foo Fighters, and he's a big fan. He's just fascinated by their music. It's amazing watching him grow into his own person, and we're so thankful to Johns Hopkins for making it possible,” says the proud dad. They now consider the heart team part of their own family.

“His medical team took a vested interest in Evan. You can tell they cared about him as a whole person, beyond the label of his diagnosis. When we had to go back for his second surgery, it was hard, but it was also like coming home to a family,” Patricia says. “He had so many people taking such good care of him.”

Fetal Heart Program at Johns Hopkins All Children's Hospital

The fetal heart team at Johns Hopkins All Children's Hospital in St. Petersburg, Florida, provides you and your unborn child with expert, personalized care that addresses your child’s individual needs, from your first appointment with us through your child’s birth. Our team provides a full spectrum of services, including fetal diagnostic testing and clinical management before, during and after delivery.