Susie Devries rocked her 2-week-old daughter, Maggie, in the darkened nursery while the rest of the household — her husband, Geoff, and their two older daughters — slept.
18-month-old Maggie Devries (shown with mom, Susie, and her interventional cardiologist Dr. Troy Johnston) was treated with a combination of surgical therapy and cardiac catheterization — a hybrid technique that has less risk and better outcomes than open-heart surgery.
She puzzled over Maggie's extreme sleepiness and refusal to nurse that day, even after a well-child visit that very morning found the newborn to be in perfect health. Was Maggie getting the flu? Did she have a tummy ache? Devries held her daughter closer and reassured herself that her third child was fine.
At midnight, Devries rose from the rocking chair and flipped on the light. “Maggie’s face was this horrible shade of gray and her body felt cold and clammy,” she remembers. When a wet washcloth pressed to the baby’s forehead failed to produce a response, she woke her husband. Minutes later, an aunt arrived to stay with the older girls and the Mt. Vernon, Washington, couple rushed to the community hospital with their youngest in tow.
“All the way to the hospital, I kept thinking, ‘She just has the flu,’” recounts Devries. What happened next was too much for the couple to comprehend.
“A pediatrician in the emergency room listened to Maggie’s heart for, maybe, 30 seconds,” she explains. “He turned to us and said, ‘Your daughter is critically ill and I’m ordering a helicopter to take her to Children’s.’”
“We were dumbstruck,” says Devries. “The doctor was saying words about Maggie’s heart, but we were in such shock that we couldn’t make sense of them.”
In a matter of minutes, the helicopter landed and little Maggie began an amazing journey that led to her complete recovery — a seeming miracle built on more than two decades of scientific inquiry, innovation and patient safety led by Dr. Thomas Jones at Children’s Heart Center.
King of Hearts
Dr. Thomas Jones
As director of one of the busiest cardiac catheterization laboratories in the nation, Jones leads an interventional cardiology team that treats congenital heart defects without surgery. Using thin, flexible tubes known as cardiac catheters, the team guides tiny devices through arteries from the groin to the heart to fix structural abnormalities — all while the heart continues to pump normally.
“Avoiding open-heart surgery means avoiding heart stoppage and blood transfusions, and lessening the need for narcotics and other pain medications,” explains Dr. Harris Baden, director of Children’s Cardiac Intensive Care Unit . “The hospital stays are significantly shorter. Plus, no scars!”
For a quarter of a century, Jones’ pioneering work — from contributing to the development and testing of new catheterization devices to establishing national standards of care and teaching new techniques to other physicians — has helped interventional cardiology evolve from a diagnostic tool to a treatment-focused technology.
New catheterization procedures developed in part by Jones helped about 300 children avoid open-heart surgery at Children’s Heart Center last year.
Jones and Children’s chief of cardiac surgery Dr. Gordon Cohen, are also perfecting less invasive “hybrid” procedures , where Cohen’s surgical team provides open-chest access so the international cardiology team can enter the heart using catheters.
Buying Time
While Maggie fought for her life on the Children’s Neonatal Intensive Care Unit, the Devrieses met with interventional cardiologist Dr. Troy Johnston to hear the devastating news: Their brand-new girl was born with a hole deep in her ventricular wall, the divider that separates the two pumping chambers of the heart. This defect, called muscular ventricular septal defect or muscular VSD, would require a very successful, but still experimental, catheterization procedure when Maggie was closer to a year old.
More concerning at the moment was a narrowing of the aorta below the lower chambers of Maggie’s heart that was causing too little blood to flow to her brain, lower body and limbs.
Cardiac surgeon Dr. Lester Permut fixed the narrowed aorta, then put a Gore-Tex band around her pulmonary artery to limit blood flow to her lungs until Maggie’s heart could grow big enough for the hole to be permanently repaired by Jones and Johnston.
Improving Outcomes Through Research
In 2001, years before Maggie was born, Jones was asked to be part of an elite group of heart centers that would investigate a new device to close muscular VSDs. Jones has built a national reputation for Children’s Cardiac Catheterization Laboratory, and his participation in clinical trials to develop and test heart devices is highly sought after. Jones and Johnston are currently involved in seven clinical trials for cardiac devices that are pending FDA approval.
“It used to be that the only way to repair a hole deep inside the pumping part of the heart was to stop the heart, and cut through heart tissue,” explains Jones. “During the clinical trial, we found that this new device was much safer than open-heart surgery for the repair of muscular VSDs.”
At 7 months old, Maggie still weighed only 13 pounds, placing her below the fifth percentile for children of her age. Her body was using all of the calories it could get to keep her heart pumping. It was time to close the hole in her heart using the spool-shaped, nickel-titanium device that Jones and Johnston had been successfully trialing for the previous five years.
After Permut opened Maggie’s chest to provide the best access to the hole, Johnston ran a small catheter that would deliver the hole-closure device directly through the front wall of her heart. Permut removed the Gore-Tex band, and Johnston deployed the investigational device, called an Amplatzer Muscular VSD Occluder, which sealed the hole. The catheter was removed and Johnston sutured the tiny puncture in the delicate tissue where the catheter had entered the heart. Throughout the hour-and-a-half procedure, Maggie’s heart never skipped a beat, nor did she need a blood transfusion.
“We were discharged two days after the surgery,” recounts her mom. “Even in that short amount of time, I could tell Maggie had better color, more energy and was starting to gain weight.”
Today, Devries describes her healthy 18-month-old as the “rowdiest of her three girls.” Maggie is now at the 75th percentile for her weight and takes no medication.
Thanks in part to the leadership of Children’s cardiac catheterization team, the FDA approved the Amplatzer for widespread use in September 2007. Since only a handful of physicians in the nation are able to perform the challenging catheterization procedure that corrects muscular VSDs, Jones will soon be demonstrating the technique from one of the Children’s catheterization labs via live satellite broadcast to colleagues around the world.