Heart Center
At Children's Medical CenterFamily-centered Care
Ryan Valek, 14, is no stranger to tying up his parents' phone lines. But while most teens are busy talking with friends, Ryan uses the phone to send his heart rhythm signals to Children's.
Ryan uses a special telephone transmitter to make over-the-phone pacemaker checkups every three months. The transmitter works by combining the pacemaker signals and his heart rhythm into one signal that can be sent over the telephone lines. A receiver in the Cardiology department at Children's then changes the tones into the electrocardiogram tracing that cardiologists on the hospital's medical/dental staff use to check his heart rhythm and the pacemaker's functioning.
What seems like a burden for a teen on the cusp of high school is a minor nuisance for Ryan, who since birth has coped with the effects of a combination of severe congenital heart defects – tricuspid atresia and pulmonary atresia.
Tricuspid atresia is a type of congenital heart disease in which the valve between the right atrium and right ventricle fails to develop. Blood that returns from the body to the right atrium cannot directly enter the right ventricle and must pass through a hole in the atrial septum into the left atrium and then the left ventricle, causing a low oxygen level.
Children like Ryan born with pulmonary atresia have no pulmonary valve; therefore, blood is unable to flow normally from the right ventricle into the pulmonary artery and on to the lungs, which also causes less oxygen to circulate in the blood.
Early intervention
Ryan had his first surgery at Children's on his second day of life when a central shunt was made and alleviate his cyanosis (a condition caused by lack of oxygen in the blood). "During Ryan's delivery, he experienced an erratic heart beat, and an emergency C-section was performed," said Ryan's father Kevin. "When he came out looking very blue due to the lack of oxygen, a cardiologist, Dr. Edgar Newfeld, was called and determined it was most likely heart related. We knew Children's was the best place for him, and Ryan was transferred within a few hours of being born.
A month after Ryan's first birthday, he was back at Children's to undergo a major cardiac operation called a bilateral bidirectional Glenn procedure. With this operation, the superior vena cava (the large vein that brings oxygen-poor blood from the head and arms back to the heart) is connected to the right pulmonary artery. This allows blood from the head and arms to passively flow into the pulmonary artery and proceed to the lungs to receive oxygen. The operation helps create some of the connections necessary for the final operation in the series for patients like Ryan, the Fontan procedure. "We knew this wasn't the end of Ryan's treatments, but it was a necessary step in the journey to recovery," said Ryan's mother Debbie.
The Fontan procedure
Three years after the Glenn procedure, Dr. Hisashi Nikaidoh, a cardiothoracic surgeon on the medical/dental staff at Children's, performed the Fontan procedure, which allows all the oxygen-poor (blue) blood returning to the heart to flow into the pulmonary artery, greatly improving the oxygenation of Ryan's blood.
"At that time, the Fontan was a good operation for tricuspid atresia patients, and the immediate result for Ryan was wonderful," Dr. Nikaidoh said. "The downside is that over time, this type of Fontan connection often forces the right atrium to enlarge, leading to atrial arrhythmia and the need for a revision of the Fontan operation to correct the problem."
A new repair
Ryan spent a decade following his initial Fontan procedure symptom-free, requiring only daily medications and annual check ups. Then things changed.
"The day it all started, he came in and said he could feel his heart beating, which he'd done before, so I figured it was nothing," said Ryan's mother Debbie, a pediatric nurse. "So I went ahead and listened to his heart, and it was all over the place. There was no mistaking it, something was wrong."
Hidden inside, Ryan's right atrium had enlarged to the point it had to be removed and a pacemaker installed to control the heart's beating function.
Dr. Joseph Forbess, chief of Cardiothoracic Surgery at Children's, performed the more than 10-hour Fontan repair surgery on Ryan in August.
"The Fontan conversion with Cryo-Maze procedure appears to add significantly to the durability of the patient's own cardiovascular system," Dr. Forbess said. "Otherwise, many more Fontan patients like Ryan would be faced with cardiac transplantation as their only surgical option."
Children's has provided medical care for three members of the Valek family. From left, Children's cardiac patient Ryan Valek is pictured with his mother, Debbie, a childhood cancer survivor treated at Children's; and brother, Bobby, a cardiac patient at Children's.
Mom, sons bond through patient experiences
Ryan Valek isn't the only member of his family to have relied on Children's for care.
Ryan's mother Debbie was diagnosed with Stage 3A Hodgkins lymphoma when she was 14. Surgery at Children's removed her spleen and various lymph nodes, and she received three months of radiation and nine months of chemotherapy. She has been in remission for more than two decades.
Ryan's brother, Bobby, also was born with a congenital heart defect. When Bobby was a newborn, Debbie pulled out her stethoscope on a whim one day and listened to his heart. She thought that she heard a murmur, so the next time the Valeks brought Ryan for a check-up at Children's, they brought Bobby along, too.
The doctor confirmed the presence of the murmur, and an echocardiogram revealed several atrial septal defects. Dr. Thomas Zellers, chief medical officer at Children's and a cardiologist on the hospital's medical/dental staff, closed the holes when Bobby was 8, using cardiac catheterization. For the past three years, he has experienced no side effects and requires no special medication.
"For me, Children's has always been a place of hope and healing," Debbie Valek said. "But I must admit that when I left Children's all those years ago following my cancer treatment, my full intention was to never have to step back in there again. But when Ryan was born and Bobby was diagnosed, we were very glad Children's was nearby."
Kevin Valek, Ryan and Bobby's father, said that Children's has had an invaluable impact on the family.
"We're so fortunate to have a first-class pediatric hospital like Children's available," he said.
Ryan Valek, 14, uses a special telephone transmitter to send his heart rhythm signals to Children’s for regular pacemaker checkups.
Articles, News & Events
|
|
|
|
|
-c-t.jpg){kind=small}
|
