Liam Drury – Aortic Stenosis

Learn more about Liam's experience with Aortic Stenosis at Children's Hospital.

A Christmas Miracle

It may have been a coincidence of timing that Liam Drury’s young life was saved through a heart transplant on the 24th of December, but it seemed like a Christmas miracle to all those who played a role in his recovery.

Barely 7 months old at the time of his transplant, little Liam of Martinsburg, W. Va., had already experienced more than his share of critical health issues. Even before he was born, local doctors detected a cardiac complication and advised his mother, Stacy Staubs, to have her delivery at a Washington, D.C. hospital that would be equipped to deal with potential problems.

Nine weeks premature and weighing less than four pounds, Liam started his life in May of 2012 with aortic stenosis, a congenital heart defect in which the aortic valve cannot open completely. On the second day of his life, he had balloon valvuloplasty to widen the valve in his tiny heart. Ms. Staubs recalled, “That procedure was successful, but because Liam was so small he had to stay at the hospital.” Ms. Staubs and Liam’s father, Jim Drury, spent the summer watching over their son in the hospital’s neonatal intensive care unit.

Rhythm and Valve Problems

Discharged and back at home by the end of August, Liam did well for several weeks, but then his behavior turned fussy – he just wasn’t acting like himself. He seemed tired, but couldn’t rest. When his mother noticed a little blood in the urine in his diaper, she called Liam’s cardiologist. On the doctor’s advice, Liam was taken to a Virginia hospital where he was determined to be experiencing supraventricular tachycardia, a type of arrhythmia in which the heart rate rapidly accelerates, resulting in weakness and fatigue. He was flown to a Washington, D.C., pediatric hospital for care. There, doctors stabilized his arrhythmia with medications, and after another short stay Liam was discharged.

In late October, Liam started having episodes where he would scream, cry, and hold his breath until he nearly passed out. Another life flight to the D.C. hospital ensued. Ms. Staubs said doctors diagnosed his condition as acid reflux, and he was discharged after a couple of days.

“The night we got home, Liam started to scream again, held his breath, and this time he turned blue,” Ms. Staubs recalled. His father provided resuscitations until paramedics arrived.

The baby was life-flighted back to the hospital, where he experienced three cardiac arrests. “They determined that Liam had hypertension of the lungs,” Ms. Staubs said. “They put him on medication for that. The muscles in his heart had gotten much worse, and so had his aortic valve.”

Surgeons replaced the aortic valve, but Liam’s pulmonary hypertension led to a new heart rhythm issue. He would cry, then hold his breath, and his heart rate would plummet, a condition called bradyarrhythmia. His mother recalls that he was on an “insane” amount of medications, including sedatives and painkillers.

Increasing Pressures

Liam’s condition worsening, the D.C. doctors advised that a heart-lung transplant was needed; however the hospital was without a pediatric heart or lung transplant program. Its cardiology team contacted other hospitals in the East and as far west as Texas. All were concerned that Liam might be too weak to survive transport, let alone another surgery. The Heart Institute at Children’s Hospital of Pittsburgh of UPMC, with its Heart Failure and Recovery Program, accepted the challenge.

At the end of November, after Liam had two stable nights on a ventilator at the D.C. hospital, Children’s Specialized Transport Team flew the critically ill child to Pittsburgh, while his parents made the 250-mile drive by car.

Surgical Director Peter Wearden, MD, PhD, examined Liam and determined that he was in no condition for a transplant. “He had very high heart pressures due to a small pumping chamber and a severely leaking aortic valve,” Dr. Wearden said.

“High pressures on the left side of the heart were causing oxygenated blood to back up into the lungs – injuring them – rather than out to the body. We didn’t know if his lungs were so badly injured that he would be able to tolerate a heart transplant.”

The team’s first goal was to lower the pressures within Liam’s heart, eliminating backflow so the lungs could recover.

A Strategy for Healing

In a novel approach, Dr. Wearden used a PediMag blood pump as a ventricular assist device (VAD) along with an oxygenator to provide some relief for both Liam’s heart and lungs. Dr. Wearden’s team placed tubes, called “cannula,” from both the right and left atria, drawing both oxygenated and deoxygenated blood through the PediMag pump to relieve pressure on the heart and reduce the flow through the faulty aortic valve. The mixed blood was then channeled through the mechanical oxygenator so that oxygen-saturated blood could be returned to Liam by way of his aorta.

With the high pressures alleviated, Liam’s lungs recovered sufficiently in four days, allowing the team to remove the oxygenator and the cannula serving the right side of the heart and take Liam off the ventilator. Although the PediMag pump was still needed to support the left side of the heart with the faulty aortic valve, the parents could hold their son again. With this treatment and medical therapy, a follow up cardiac catheterization showed that Liam’s pulmonary vascular resistance had returned to acceptable levels, and he was listed for a heart transplant only.

In three weeks a donor heart became available, and Children’s surgeons conducted the transplant on Christmas Eve.

“On Christmas Day, his donor heart was working magnificently, he was removed from ECMO, a heart and lung by-pass machine, and his chest was closed,” Dr. Wearden said.

During a four-month recovery following surgery, Liam and his family stayed at the Ronald McDonald House adjacent to Children’s Hospital while his condition was monitored by specialized clinical support teams. Surgeons provided a gastronomy-tube (G-tube) as a means to supplement his nutrition and diet and to help with growth, and he was also provided with a pacemaker to help with his arrhythmia.

In the spring of 2014, as Liam approached his second birthday, Ms. Staubs reflected on the experience, saying, “I’m very thankful that we got to go to Children’s and have everything done. I don’t know that everything would have worked out as well somewhere else. Having the Ronald McDonald House connected to the hospital was one of the ‘plusses’ in Pittsburgh.”

“Dr. Wearden is a wonderful doctor. He was willing to try whatever he could to help Liam, and he did an amazing job.”

Still small for his age at just over 18 pounds, Liam is attempting to walk and babbles a few words. “You would never think a little child could go through as much as he did and still smile,” Ms. Staubs said. “He bounced back so quickly, and smiles at you even though he has his ups and downs. He’s definitely a little miracle.”