Keeping the beat
By Loretta Coureas
Last fall, Shannon Stitz and her family were enjoying the heart-thumping excitement of the Neptune Festival air show at Oceana Naval Air Station when the 16-year-old suddenly became faint.
Her mother, Terri Taunton, says Shannon's lips were turning blue, her face pale. "We grabbed her arms to keep her from falling to the ground," Terri recalls.
Shannon doesn't remember the next few moments, but she says when she came to, she felt weak and confused. Terri says that as they waited anxiously for paramedics to arrive, she thought about the heart condition Shannon has had since birth. She sensed that Shannon may have reached a new stage of her cardiac care.
"She was born with transposition of the great vessels," her mother said. It meant that Shannon's pulmonary artery – which usually carries venous blood to the lungs to receive oxygen – and her aorta – which usually carries the oxygen-rich blood to her body – were switched. So, unoxygenated blood was being pumped throughout her body.
"Shannon went straight into the NICU at CHKD after she was born," Teri said. "And she had heart surgery when she was just 6 weeks old."
Reconstructive cardiac surgery created a channel in Shannon's heart between the left and right chambers to correct the effects of transposed blood vessels. For 16 years, she was under the care of cardiologists at CHKD who monitored her heart.
Last fall, paramedics at the scene said Shannon's blood pressure was extremely low, her heart rate very slow. Later, at the Naval Medical Center in Portsmouth, a cardiologist told the family Shannon needed a pacemaker and referred her back to CHKD's pediatric cardiology experts.
Cardiologist Bertrand Ross was very familiar with Shannon and with her condition, having been involved with her care for many years in CHKD's cardiology clinic. "We always knew Shannon would eventually need a pacemaker, but it's better to wait as long as you can for the patient to gain maturity," he said.
Terri said, "We were so glad to be at CHKD with all the familiar faces. But we weren't expecting Shannon would need the pacemaker until she was in her 20s."
Just weeks before Shannon's fainting spell, a revolutionary advance in pacemaker wire leads was approved by the FDA, opening the door for Shannon to be the first in Virginia to use the tiniest wire lead ever developed. "We were able to use this superior technology for Shannon's surgery in November," Dr. Ross said. "And she has done extremely well with her new pacemaker."
He said the advantage of the new technology is in its single thin flexible wire lead that connects the pacemaker to the heart. The wire is so tiny that it allows blood to flow efficiently around it as it threads through the blood vessel. "It is less likely to cause a problem in her vein," Dr. Ross said, explaining, "Previous leads consisted of twisted wires that took up more room in the blood vessel and were less flexible. The new leads are easier to manipulate and place exactly where they are needed within the heart."
Where they are needed is in the sinoatrial node of the heart, the place where electrical impulses normally fire naturally and cause heartbeats. But Shannon suffers from "sick sinus syndrome," meaning her natural heart beats are weak and irregular, causing slower-than-normal heart rate, occasional heart fluttering, dizziness and low blood pressure.
"Pacemakers are very efficient and necessary in cases like Shannon's," Dr. Ross said. "But they must be replaced in five to 10 years when the batteries wear out. These new leads, however, are less likely to need replacing, making replacement of pacemakers much less complicated."
Last November, after her spell at the air show, Dr. Ross installed Shannon's pacemaker while she was under sedation in CHKD's catheterization lab. "I threaded the single, tiny lead through the subclavian vein just under her collarbone to the space in her heart where the beats are controlled," he said. The pacemaker, which is about the size of a half-dollar coin and weighs just one or two ounces, was then inserted into a small incision in her chest and the lead wire attached.
Shannon says she didn't feel anything and could feel the pacemaker's position for only a brief period after her surgery when there was some swelling. "I can hardly tell it's there now," she said, lightly touching the small scar on her chest.
Dr. Ross says Shannon was the first patient at CHKD to have the new lead technology. "The timing of the FDA's approval was really to Shannon's advantage," he said. "We were able to use the latest technology to help this young lady get on with her life."
To Shannon, that meant finishing the 10th grade at Kellam High School with fewer missed days. And experiencing the level of health and energy a 16-year-old needs when heading to the beach with her friends, dancing at her ring dance, learning to surf, fishing with her family, bowling and whacking a baseball at the nearby batting cage.
"I don't have to worry anymore that my heart won't beat like it's supposed to," she says gratefully. Her mother and stepdad, Andy Taunton, are equally relieved. "I have been on pins and needles for her entire life," Terri said. "It's just really good to know she can do anything she wants to do now and not feel handicapped," Andy said.
Shannon says her heart's desire is to be a marine biologist. Terri and Andy are just glad Shannon has the heart to try.
Dr. Ross practices with Children's Specialty Group PLLC at CHKD.
This story was featured in the third quarter 2006 issue of KidStuff, a publication of Children's Hospital of The King's Daughters.