CHOC Patient Benefits from World’s Smallest Pacemaker

Sofia Rodriguez and CHOC Children’s cardiologist Dr. Anthony McCanta are making medical history. The petite 12-year-old recently became the smallest and youngest patient to receive the Micra® Transcatheter Pacing System (TPS). The innovative physician, who specializes in pediatric electrophysiology, joins an elite group of doctors in the world who have implanted the device, and is likely the first to do so in a child.

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The Micra® Transcatheter Pacing System is about the size of a vitamin.

About the size of a vitamin, Micra TPS, which was approved by the Food and Drug Administration last year, provides the most advanced pacing technology at one-tenth the size of a traditional pacemaker. And unlike traditional pacemakers, the device does not have cardiac leads, which are the wires that carry electricity from the pacemaker to the heart and the other way around. This activity occurs between 100,000 to 200,000 times in most children, eventually putting a strain on the leads. The leads can then break or stop working properly, requiring a new device to be implanted.

In addition to being leadless, the Micra TPS is small enough to be delivered through a catheter and implanted directly into the heart instead of a surgical “pocket” under the skin. This offers patients a safe alternative to conventional pacemakers without the complications associated with leads — all while being cosmetically invisible.

Sofia’s story

Diagnosed shortly after birth with a particularly complex form of tetralogy of fallot, a rare and serious heart condition, Sofia had her first open-heart surgery before turning 1 month old and a second, to implant a traditional pacemaker, when she was 6 months old. Abdominal surgery followed, as did a diagnosis of DiGeorge syndrome, a chromosomal disorder that affects the development of several body systems.

Sofia tackled each health challenge with a fierce determination to survive and, more than that, to enjoy her childhood. Her parents and the team at the CHOC Children’s Heart Institute supported her every step of the way, equally committed to her quality of life.

When the pre-teen needed a new pacing system (the cardiac leads from the first device had broken), Dr. McCanta advocated for the Micra TPS. He knew the benefits it would bring to Sofia, and other CHOC patients.

“The immediate benefit to Sofia is that she does not have another scar on her upper chest. The long-term benefit is she has no cardiac lead in her vein that could break, become infected or cause a blood clot,” explains Dr. McCanta. “When you consider the course of her entire life, the benefit of reducing complications of pacing is almost immeasurable.”

Sofia’s parents are proud of the part she played in the medical milestone. “She’s always been special, and here’s just one more thing that demonstrates how special she truly is,” says Sofia’s dad, Edgar. “We are thankful for people like Dr. McCanta and so impressed with the new technology. We feel truly blessed.”

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Sofia and her dog, Yoda

When she’s not on her computer, Sofia loves watching videos and listening to Latin music. She’s outgoing with lots of friends and a bright future ahead of her.

Learn more about the electrophysiology program at CHOC

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A Reunion of Heroes: Katie’s Story

After recently being discharged, Katie Flathom stopped by the CHOC Children’s cardiovascular intensive care unit (CVICU) again to make some introductions.

Suddenly, the 16-year-old has a group of heroes in her life: the coach who resuscitated her on school campus and the CHOC team that treated Katie for three weeks and will continue her care as she navigates life with a newly diagnosed heart condition.

During a recent athletic conditioning class in school, Katie collapsed and went into sudden cardiac arrest.

Her trained and quick-thinking conditioning coach snapped into action and resuscitated Katie with CPR until paramedics could arrive and transport her to CHOC.

“It was the longest 10 minutes of my life,” said Greg Vandermade, Katie’s coach at Mater Dei High School who also credits other students for alerting him to Katie’s condition and calling 911, as well as fellow staff who assisted by obtaining an automated external defibrillator (AED) to shock Katie’s heart into a normal rhythm.

At CHOC, Katie continued to have irregular heartbeats that required further defibrillation and cardioversion, procedures that help restore the heart’s natural heart rhythm, said Dr. Anthony McCanta, a CHOC cardiologist.

Katie also went on extracorporeal life support, a treatment that takes over the heart’s pumping function and the lungs’ oxygen exchange until a patient can recover from injury. This allowed the CHOC Children’s Heart Institute team to continue to treat her life-threatening arrhythmias with medication, Dr. McCanta said.

Dr. McCanta performed an electrophysiology study procedure and implanted beneath Katie’s skin a subcutaneous implantable cardioverter defibrillator, a device that helps prevent sudden cardiac arrest in patients.

After Katie’s discharge and further testing, she was diagnosed with Arrhythmogenic Right Ventricular Dysplasia, or ARVD.  A rare type of cardiomyopathy  where the muscle tissue in the heart’s right ventricle is infiltrated and replaced by fatty tissue and scar tissue, ARVD weakens the heart’s ability to pump blood and makes the heart susceptible to life-threatening arrhythmias.

The diagnosis also means Katie, a cross country and track athlete, will need to give up running for good.

“It was hard at first,” she said.

But instead of sitting on the sidelines, Katie’s decided to pick up golf, a sport that’s compatible with ARVD.

Katie has even begun incorporating a golf swing into her physical therapy sessions at CHOC, and she had two clubs in tow as she, her family and coach Greg visited the CVICU recently.

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When Katie came back to visit the CVICU team and reunite her heroes, her CHOC care team presented her with a heart-shaped pillow, which they all signed with well wishes.

There, Dr. McCanta and the CHOC team presented Greg with a plaque recognizing him for his swift response and efforts that surely saved Katie’s life.

“Coach Greg responded to Katie with CPR on the spot and saved her life that day,” Dr. McCanta said. “His heroic actions, and those of Katie’s schoolmates and staff, including obtaining and appropriately using the AED, are the reason that Katie is alive today.”

Katie’s story underscores the importance of being trained in CPR and in the use of AEDs, Dr. McCanta said.

“Having AEDs in schools and training staff and students in CPR with an AED are some of the most important interventions that we have in saving lives of young people experiencing sudden cardiac arrest,” he said.

Getting AEDs installed in schools is among the goals of CHOC’s Life-Threatening Events Associated with Pediatric Sports – or LEAPS – program.

Coincidentally, Katie’s own grandmother, a nurse and health services coordinator in the Irvine Unified School District, has collaborated with LEAPS and helped get AEDs installed on her district’s campuses.

“Never did I think though that this would happen to one of my own family members,” said Marcia, Katie’s grandmother.

Learn more about pacemakers and cardioverter defibriilators

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Heart Month: Ryden’s Story

At 27 weeks pregnant, Kayleen Enoka discovered her baby boy, Ryden, had hypoplastic left heart syndrome (HLHS), a birth defect where the left side of the heart does not form correctly and affects normal blood flow through the heart. As a young, first-time mom, she was overwhelmed by the news.

“I felt incredibly helpless. I felt that I couldn’t do anything to help my baby and I wondered what would happen to him. I also felt that I must have done something wrong during the pregnancy to cause his heart defect. I was reassured by the perinatologist and the cardiologist that his defect wasn’t because of something I had done wrong. My mother sat with me through the diagnosis and held my hand and hugged me as I cried,” Kayleen vividly remembers.

After Ryden was born, he was immediately transferred to CHOC Children’s Hospital to be cared for by our CHOC Heart Institute. Kayleen was a partner in her son’s care from the beginning. He had to undergo a series of three surgeries, performed by Dr. Richard Gates, pediatric cardiothoracic surgeon at CHOC, with the first one, the Norwood Procedure, at just five days old. During the surgery, Dr. Gates made Ryden’s right ventricle the main pumping chamber for blood flow to his body.  A shunt was also placed as a pathway for blood to flow into his lungs to receive oxygen.

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Ryden was transferred to CHOC shortly after he was born for the first of three heart surgeries.

“My family and I all sat together waiting for news during the surgery. It was hard, but having so much support helped a lot. I remember when we walked into the room and everyone seemed to be moving so fast. When I asked how he was doing, I was told he was tenuous. That word has resonated with me over the years because I remember feeling that he wouldn’t survive the night. The doctors showed me where the bypass machine was and told me that it was there in case he needed it; again, I was frightened for my baby wondering if he would be strong enough to get through this. I believed in my heart that he was a fighter, but watching all the activity and how small he looked in his hospital bed, made it much harder to believe,” Kayleen says.

Ryden’s second surgery, the Glenn Shunt Procedure, performed when he was 6 months old, was just as scary because Ryden’s health was fragile, Kayleen recalls. The procedure created a direct connection between the pulmonary artery and the vessel returning oxygen-poor blood from the upper part of the body to the heart. After the surgery, Ryden had numerous complications and was hospitalized for 34 days.

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Ryden at about 6 months of age following his second heart surgery, the Glenn Shunt Procedure.

By the time of Ryden’s third surgery, the Fontan when he was 4 years old, Kayleen was ready but apprehensive. “Since Ryden was a little older, I could be honest with him. I told him what was going to happen, and even though he was scared, he was aware and was still able to smile,” Kayleen says.

Dr. Gates connected Ryden’s pulmonary artery and the vessel returning oxygen-poor blood from the lower part of the body to the heart, which allowed the rest of the blood coming back from the body to go to the lungs.  Ryden spent ten days in the hospital.

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After Ryden’s third heart surgery, his nurses gave him this heart pillow, signed by his care team.

Throughout the years, Ryden has experienced arrhythmias, is susceptible to colds, takes multiple medications, and was recently diagnosed with asthma. Kayleen has developed a close relationship with the CHOC Heart Institute team.

“I have always felt like I am a part of the team. In the beginning, I could never have too many questions; the doctors and nurses always took the time to make sure I understood what was happening. Now, when Ryden needs to be hospitalized, the care team always listens to my input. We work together because they understand that I know my son best,” she says.

Among the many experts involved in Ryden’s care, the Enokas have a special relationship with Dr. Anthony Chang, pediatric cardiologist at CHOC.

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Ryden and his cardiologist, Dr. Anthony Chang.

“Dr. Chang has been amazing. I wouldn’t have chosen another cardiologist because he takes the time to care for his patients. Ryden really admires him and often says when he grows up he wants to work on hearts like him,” Kayleen says.

“Ever since I took care of a baby with HLHS in 1983, my passion to help children with congenital heart disease has never subsided. HLHS is a heart defect that requires the supreme dedication of both doctors and nurses in cardiology and cardiac surgery as well as intensive care. It is, however, parents like Kayleen who continue to inspire all of us to help these children, and humbles us in all that they do when these children are not in the hospital or clinic,” Dr. Chang says.

Kayleen’s appreciation for CHOC and its mission inspired her to become an employee. She works as a department assistant in the clinical education and professional development department. She also volunteers her time as a member of the Family Advisory Council, an important group of patients’ family members who provide input on decisions, initiatives and discussions at CHOC. In addition, Kayleen participates in the CHOC Walk every year with “Team Ryden,” including friends, family and cardiovascular intensive care unit (CVICU) nurses.

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Ryden inspires a group of family and friends to participate in CHOC Walk every year in his honor.

Today, Ryden is a happy, fun-loving 7-year-old, who enjoys swimming and playing baseball. Throughout his journey, one thing that has remained unwavering, is Kayleen and Ryden’s close relationship. When Ryden has questions about his heart, Kayleen is always happy to talk openly and lovingly with her son, and reminds him that he has a “special heart.” His middle name — Pu’uwaikila — means “heart of steel,” and Kayleen’s little fighter is surely living up to the name.

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Kayleen Enoka and her son Ryden.

As American Heart Month comes to a close, Kayleen offers parents of heart patients the following tried and true tips that have helped her along the way:

1. Trust your child to know his limits. I’ve always let Ryden push himself, while still keeping a close eye on him of course.

  1. When your child is developmetally ready, be open and honest about his condition. You might be worried you’ll scare him/her, but I’ve always felt that Ryden has the right to know what’s happening to him.
  2. Children with congenital heart diseases may have self-esteem issues (i.e. scars, lack of ability to keep up with other children.) Remember to let your child know that he/she is special and what makes them different is also what makes them amazing. I always tell Ryden that his scar on his chest is what shows his strength. And, that chicks dig scars – it’s an inside joke (he’s never allowed to date).



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Electrophysiology Advances Restore Patient’s Quality of Life

A teenaged patient’s longtime arrhythmia has been repaired and her quality of life dramatically improved thanks to emerging technology and the skill of a CHOC Children’s cardiologist.

Lauren Flotman, 15, had experienced irregular heartbeats for years before Dr. Francesca Byrne, a pediatric cardiology specialist, diagnosed her with supraventricular tachycardia, or SVT, and Dr. Tony McCanta, a pediatric heart rhythm specialist, repaired the condition through radiofrequency ablation.

The episodes first surfaced when Lauren was about 8 years old and they began increasing in frequency as she aged. They’d occur without warning or pattern.

For Lauren and her family, the sudden attacks caused great concern. Not only was she drained and tired after an episode, but Lauren dreaded them happening, especially during a pep squad routine when her teammates were depending on her.

Lauren was elated to finally have a name for her condition.

“It was a huge relief for sure to have a diagnosis,” she says. “I always had to just describe the feeling because I didn’t have a name. Now I can say I have SVT.”

Lauren’s diagnosis was reached after a Holter monitor captured her heart racing at 220 beats per minute. Dr. Byrne referred Lauren to Dr. McCanta to discuss treatment options, which included anti-arrhythmic medications or an ablation procedure.  After reviewing their options carefully, the Flotmans decided to pursue ablation.

For Lauren’s ablation, Dr. McCanta used a new technology called an intracardiac echocardiogram, or ICE, to create a three-dimensional map of the inside of her heart without using fluoroscopy (X-Ray radiation), enabling a catheter to apply radiofrequency energy to the precise location in her heart causing her SVT.

ICE technology involves a tiny ultrasound probe imbedded into a catheter that is advanced through the vein directly into the heart, allowing for very clear, accurate image quality. These ultrasound images then integrate with a three-dimensional electroanatomical mapping system, which acts like a GPS (global positioning system) for the catheters within patients’ hearts, to provide an accurate real-time shell of the inside of the patient’s heart. This allows the doctor to safely move catheters inside the beating heart without using radiation.

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Dr. McCanta and the electrophysiology team at CHOC were among the first in the world to routinely utilize intracardiac echocardiography in pediatric and adolescent patients.

While radiofrequency ablation has become a safe and common treatment for SVT in children and adolescents since the mid-2000s, intracardiac echocardiography (ICE) has not traditionally been used in pediatrics due to the large-sized catheters. But when a smaller catheter was created, which was more suitable for the size of young patients, Dr. McCanta and the electrophysiology team from the CHOC Children’s Heart Institute were among the first in the world to routinely utilize the new technology in pediatric and adolescent patients.

“For a young, healthy patient like Lauren, increasing safety and minimizing the use of radiation are extremely important, while still being able to provide a cure for her arrhythmia with ablation” says Dr. McCanta.

After a few days of taking it easy following the procedure, Lauren felt back to her usual self – only without the constant fear her heart would suddenly begin racing.

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Lauren’s longtime arrhythmia has been repaired and her quality of life has dramatically improved, thanks to the electrophysiology team at CHOC.

“Our team loves utilizing advanced technologies like ICE and three-dimensional mapping to help children, adolescents, and young adults with heart rhythm problems,” says Dr. McCanta, “Seeing patients like Lauren get back to all of the things they love doing is why we do this!”

Since the procedure, Lauren has been vocal at church to educate her peers about being conscious and vocal about their health.


Get the facts about CHOC's advanced electrophysiology program



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