Pulmonary Atresia: Justin's Story

For a few minutes after Justin was born, on a rainy October evening in 1995, everything seemed fine. His parents were congratulated and a nurse carried the newborn away to bathe and swaddle him.

"Then one of the doctors came back and informed me there was a problem," Justin's father, Scott, recalls. "That's when everything became a blur."

Justin had begun to turn blue.

The diagnosis: Pulmonary atresia

Gerald Vekteris, DO, a pediatrician from The Children's Hospital of Philadelphia, was on-call at the hospital where Justin was born. He quickly recognized the baby had a heart problem and ordered medication to stabilize his condition. Justin was taken by ambulance to Children's Hospital.

justin"Our baby was whisked away in the middle of the night and taken to a hospital in Philadelphia that we had never been to," Scott recalls. "It was terrible."

The next morning, Scott and his wife, Val, met the group of people that would become an enormous part of their lives: CHOP's Cardiac Center team.

Doctors performed a cardiac catheterization, a procedure that shows the heart's structure and function. The test confirmed Justin had a congenital heart defect called pulmonary atresia. His pulmonary valve hadn't formed correctly and was blocked, preventing an adequate flow of blood from heart to lungs.

Justin's medical team sat down with his family to explain the treatment for pulmonary atresia and answer their many questions.

"It was all overwhelming, but we were able to understand what was going on," Val recalls. "The doctors and nurses were very informative."

Surgery to treat pulmonary atresia

At 2 days of age, Justin had open heart surgery to treat his pulmonary atresia. The cardiothoracic surgeon placed a tiny patch across the valve to open it. After two weeks at CHOP, Justin came home. 

He returned to the Hospital two months later when doctors felt he was strong enough for the surgery needed to place a more permanent patch to help ensure there was enough blood flow to the lungs. Thomas Spray, MD, chief of Cardiothoracic Surgery at CHOP, performed the second surgery.

Before and after the surgeries, Justin's parents remained at their baby's bedside. They were able to hold Justin as he slept and healed, with Cardiac Center nurses positioning the infant in their arms, "with all the wires attached," Val remembers.

Justin recovered quickly after surgery to treat his pulmonary atresia. The nurses taught Scott and Val how to care for the scar on Justin's chest as it healed.

"He healed really well and the scar today looks fine. He's not embarrassed about it," Val says.

Atrial septal defect (ASD) surgery

As he grew, Justin had frequent checkups at the Cardiac Center. When he was 5, cardiologist Jonathan Rome, MD, performed a catheterization to patch a tiny hole between the upper chambers of his heart, called an atrial septal defect (ASD).

Before his ASD procedure, Justin's oxygen-saturation levels (the amount of oxygen in the blood) were in the upper 80s. It was adequate, but not ideal for a child his age. After the catheterization, his levels went to 100 percent.

A brighter future

Today, Justin is 14 and completed 8th grade with straight A's. He received five scholarships (Physical Education, Spanish, Language Arts, Science and Outstanding Character) during 8th Grade Recognition Night.

He has just started his freshman year in high school and is taking all honors classes. Justin has also joined both the Surf and Boy’s Crew Team in Ocean City, N.J., where he lives with his family.

“Justin has gone beyond our expectations,” Scott proudly and happily reports.

Follow-up with the medical team at CHOP

Every August Justin visits cardiologist Paul Stephens Jr., MD, at the CHOP Specialty Care and Surgery Center in Voorhees, NJ. This annual check-up includes echocardiography and electrocardiography. Justin has a stress test (on a treadmill) and cardiac MRI approximately every two years.

In the future, depending on how his heart develops as he reaches maturity, Justin might need additional medical procedures or a new heart valve.

Justin will continue to see a cardiologist all of his life, eventually transferring to an adult program, such as the Philadelphia Adult Congenital Heart Center. This program unites the breadth of resources from two premier institutions, CHOP and Penn Medicine. Recently he wrote an essay for school that began "Thump, thump, thump" and went on to describe his wish to become a heart surgeon.

Of the days immediately after Justin's birth, Scott says: "Those are some of the worst memories we have. But once you meet people at the Cardiac Center, you end up trusting them. We owe a lot to them."

"We felt comfortable there," Val recalls. "We knew that we were in the right place, and there is comfort in that."

Originally posted: June 2009