Zach was born with a nearly completely obstructed pulmonary valve leaving his heart to his lungs. The pulmonary valve controls blood flow from the heart to the lungs so that it can refresh its oxygen supply before being pumped to the rest of the body.
At just two days old, Zach underwent emergency surgery to remove his pulmonary valve – and he lived without it for 15 years. Though many patients can survive for many years without this valve, it makes pumping blood to the lungs much more inefficient when over half the work comes rushing back into the heart. Eventually, they will need a replacement of the valve. When Zach was 15, his WVU Medicine Children’s pediatric cardiology team knew it was time for him to receive a replacement valve.
Zach’s Birth Story and Early Days
More than 16 years ago, when pregnant with Zach, his mom, Lisa, had a routine ultrasound. Her care team mentioned they may have seen a dark spot on his heart, but it may have just been a shadow. They repeated the ultrasound, and the dark spot didn’t show up. Lisa went into labor and delivered Zach, not expecting anything out of the ordinary.
After Lisa’s care team broke her water manually and Zach didn’t respond, they moved forward with an emergency Cesarean section.
The day after Zach was born, Lisa learned the team had detected a heart murmur. Because the Frostburg, Maryland area hospital where Lisa delivered didn’t have pediatric cardiology specialists, they airlifted Zach to WVU Medicine Children’s. Later that day, the team diagnosed Zach with critical pulmonary valve stenosis — a condition where the heart’s pulmonary valve doesn’t fully open during fetal development.
Zach’s First Heart Surgery
At two days old, Zach had open-heart surgery.
“They said his pulmonary valve looked like a head of cauliflower,” Lisa said. “It was just a mass, so they removed the entire valve.”
Lisa couldn’t be there for the surgery because she was in the hospital recovering from delivery complications. “A very sweet (WVU Medicine Children’s) nurse was by Zach’s side the whole time,” she recalled. “This is pre-FaceTime and pre-Zoom, so she told me how he was doing every minute, which gave me peace of mind.”
Zach spent about 18 days in the hospital following his surgery. When the team said Zach could go home to Frostburg, Lisa remembered feeling nervous because she also had three-year-old twins at home. But Zach’s care team assured the family he was doing well. The only special care he needed was to sleep in a little sling device that helped with acid reflux and healing. Zach didn’t need any medications, and his incision healed quickly.
On-going Monitoring Through the Years
After his original open-heart surgery, Zach and his parents made the hour-long drive to WVU Medicine Children’s once a year for follow-up with a pediatric cardiologist. He also saw his regular pediatrician closer to home for preventive care. The WVU Medicine Children’s Heart Center team informed the family that Zach would eventually need a replacement pulmonary valve, and timing would be important.
“They didn’t want to put a valve in too early because it could wear out more quickly,” Lisa said. “But we also didn’t want to wait too long and risk his heart enlarging on the opposite side due to the backflow of blood.”
Many children and teens living without a pulmonary valve don’t realize the symptoms they may be having are due to the need for a replacement valve. Most realize a few weeks after their replacement that what they thought was normal really wasn’t. Today, Zach has a much better exercise tolerance than before.
Time for a Replacement Pulmonary Valve
When Zach was 15, the WVU Medicine Children’s pediatric cardiology team noticed some heart enlargement on his imaging tests. They knew it was time for Zach to receive his replacement valve. Zach and his family met with his interventionalist, Alex Verhoeven, MD. They decided to schedule the valve replacement surgery for June 2023 using minimally invasive catheterization techniques to minimize his discomfort and down time.
Zach and his parents arrived early on the morning of his procedure, and the care team admitted Zach. After close to a six-hour surgery, Dr. Verhoeven informed the family they needed to stop. “We thought we could seat the new valve on a specific ridge in the heart,” Verhoeven said. “But it turned out not to be particularly rigid, so it wasn’t a stable landing zone for the valve. This was an unfortunate reality for over half of the patients in Zach’s position at the time.”
Preparing for the HarmonyTM Valve Procedure
Dr. Verhoeven knew about the latest valve technology – the HarmonyTM Transcatheter Pulmonary Valve. The technology could be an excellent option for Zach – but it wouldn’t be available for a few months. The care team sent Zach’s imaging to Medtronic, which developed the Harmony valve and the previous generation of Melody valves. Medtronic processed the imaging data and created highly detailed 3D models from all angles.
“This allowed us to better understand what it would look like when the Harmony™ Valve sat in Zach’s heart,” Verhoeven explained.
The medical team needed as much data as possible to decide if the Harmony™ Valve would be a good choice. With this data and their information on Zach and his condition, Dr. Verhoeven recommended the HarmonyTM Valve.
Zach’s Minimally Invasive Procedure in October 2023
Zach and his family agreed to move forward with the Harmony™ replacement valve. This meant that Zach needed to recover from the paused surgery and wait another four months to try the valve replacement again. During the procedure, Dr. Verhoeven used a catheter (long, thin, flexible tube) inserted through an incision in Zach’s groin to access his heart. He didn’t need a third open-heart procedure. The Harmony device is hourglass-shaped and the replacement valve (a pig valve, in Zach’s case) sits in the narrow center area of the device. The device, with slow and careful manipulation, came to sit exactly as predicted within the narrow 1mm range predicted by the prior data.
Zach recovered well from the procedure. He stayed in the hospital for one night.
“I highly recommend WVU Medicine Children’s care,” Lisa said. “They are outstanding, and I’m very pleased.”
Life with a Replacement Valve
Zach took a beta blocker medication for about four months after surgery. Today, he takes a daily baby aspirin – the only medication he needs.
Zach is a competitive year-round athlete and recently competed at the Maryland state indoor track championships. Though his doctors haven’t given him any restrictions, Lisa won’t let him play football to avoid injuries from the contact sport.
Three months after having the new valve placed, imaging showed the side of Zach’s heart with enlargement had shrunk. It was almost back to normal size.
Looking to the Future
Zach will likely need additional valve replacement procedures in the future. The current valve is estimated to last 10-to-15 years based on experience with previous valves. However, with the Harmony device, future procedures Zach will need can be performed through a minimally invasive, catheter-based approach instead of a traditional, open-heart surgery.
Dr. Verhoeven and his team are working to raise awareness about the Harmony device across the state and region. WVU Medicine Children’s is the only facility in West Virginia where eligible patients can receive the therapy.
“We suspect many patients could benefit from the Harmony device,” Verhoeven explained. “This is a brand-new option for a group of patients who previously didn’t have options for a catheter-based procedure.” Before the Harmony device, patients like Zach had to undergo an invasive, open-heart procedure to replace their defective valve.
