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Giving Heart Patients a New Lease on Life

Giving Heart Patients a New Lease on Life

Ellie Riggs

Ellie Riggs, 8, and her dog, Luca.

In 2012, Ellie Riggs, now 8, was spared a fourth open-heart surgery by having her procedure in the Helen B. Taussig Congenital Heart Center at the Johns Hopkins Children’s Center. Its state-of-the-art catheterization “lab” provides 3-D imaging to improve accuracy in procedures like stenting and dilation of narrowed pulmonary vessels, all while reducing radiation by a third from the previous generation of imaging. For the Riggs family, the lab offered their daughter a new lease on life and a swift return to home and the playground.

Ellie was only a few days-old when she came into the care of the Johns Hopkins Children’s Center. She was born blue, with congenital heart disease and pulmonary atresia. Known as tetralogy of Fallot, her disease – distinguished by heart abnormalities that prevent proper pumping and oxygenation of the blood – had not been diagnosed in utero. Immediately after birth in a Carroll County, Md., hospital, Ellie was transported to Johns Hopkins, where she had her first surgery at six-days-of age.

Pediatric surgeon Luca Vricella performed the open-heart surgery, using a technique known as the Blalock-Taussig shunt – developed at Johns Hopkins and first made famous in the 1944 “blue baby” operation – to keep blood circulating and to provide needed oxygen. When Ellie was 10-months old, Vricella opened her chest again, in a nine-hour surgery, to repair a congenital hole in her ventricle artery and to put in a cow’s jugular vein to connect the heart and lung to oxygenate the heart. When Ellie was 3, Vricella led her third open heart surgery, this one to replace the child’s smaller conduit with a larger one.

Recovery after all three surgeries, required lengthy stays in the hospital’s pediatric intensive care unit, followed by lengthy recuperation at home.

Around the age of 6, in the summer of 2012, Ellie was becoming easily fatigued and winded. Her Johns Hopkins pediatric cardiologist Joel Brenner, along with pediatric interventional cardiologist Richard Ringel, determined that one of her heart valves was failing, diminishing the critical oxygenation and pumping of her blood. Ellie needed a new valve, another surgery. But this time, she would bypass the operating room and have a far shorter, minimally invasive procedure in the cath lab, led by Ringel.

Ellie was already sitting up and watching a movie when her parents came to see her after surgery. Her heart repaired, she returned home the next day, with the promise to her doctor not to ride her bike for a month. Otherwise, it was all systems go; she was back in the business of playing with friends and her dolls.

Brenner guided the family through every aspect of their daughter’s care. “I can’t say enough wonderful things about Dr. Brenner,” says Molly Riggs. “He was responsive, attentive and very accessible to us, whether at Johns Hopkins or its pediatric cardiology practice in Westminster (Maryland), near where we live.”

Today, more than 250 children and adults with congenital heart disease are catheterized annually in the lab at the Johns Hopkins Children’s Center.

A husband with two children, Bryan Knepper’s experience mirrored Ellie’s. Born with tetralogy of Fallot, Knepper came to Johns Hopkins in 2007 for open heart surgery and repair of an aortic aneurysm. A few years later, in 2013, when a valve in his heart began to fail, his Johns Hopkins cardiac surgeon, Duke Cameron, suggested Knepper take advantage of a new minimally invasive approach being offered at the Johns Hopkins Children’s Center.

So, in lieu of open-chest surgery, with its risks of infection and long recovery, Bryan opted for a five-hour procedure in its cath lab. Guided by the catheterization machine, Ringel threaded a new valve up through the femoral vein in Knepper’s leg and into position in his heart.

“The difference in the two procedures was like day and night,” says Knepper. “Afterward, there was no pain, no six-week recovery. I felt great.” He flew back to his home and family in New York the next day.

“Our cath lab is pioneering a new era all together in American medicine,” says Johns Hopkins’ Pediatrician-in-Chief George Dover, director of the Johns Hopkins Children’s Center. “As the practice of pediatric cardiology has vastly improved, so have the outcomes for patients with congenital heart disease, who are now surviving and thriving.”

 

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