A Mother's Love: Mia & Fable's Heartfelt Struggle

As a Certified Nurse Anesthetist at Oklahoma Children’s Hospital OU Health, 22-year-old Mia Woods knows all about seriously sick babies. But when Mia found out her baby had a heart condition during her pregnancy anatomy scan, she had no idea just how serious it was.

In the United States, about 40,000 babies are born with a heart defect each year, which accounts for about 1% of births. These defects range from mild to severe, but for Mia, the initial news about her unborn daughter’s heart defect was just the beginning of a series of life-threatening complications.

At 20 weeks pregnant Mia was told that her baby had a severe heart condition — a rare and highly complex form of dextrocardia with a single ventricle. The baby’s heart was twisted over to the wrong side and was missing one of the two squeezing chambers.

Dextrocardia is a rare congenital heart condition where the heart is positioned on the right side of the chest instead of the left. This anomaly occurs during fetal development, though the exact cause remains unknown. The heart’s anatomy can vary significantly in dextrocardia, sometimes involving the heart facing the opposite direction or having defects in its chambers.

The news was already serious but at Mia’s routine 36-week ultrasound, things grew even more grim — Mia’s baby had a dangerously high heart rate of 280 and had to be delivered by an emergency cesarean section.

A Dangerous Situation

Tiny Fable was placed in the neonatal intensive care unit (NICU) at Oklahoma Children’s Hospital after birth, and Mia didn’t see her until six hours later. Despite needing urgent surgery, Fable’s heart was in distress with supraventricular tachycardia (SVT), a heart rhythm disorder that causes a very fast or erratic heartbeat. The newborn was intubated and isolated to reduce all stimuli, and when she was 3.5 weeks old, Mia was able to hold her for the first time.

While some patients with heart disease this complex may be able to undergo multi-stage surgery and keep the heart they were born with, Fable had shown signs that she would not be able to go past the first stage surgery. For her heart condition it was decided that she would need a heart transplant to have any chance of survival, but would she make it to that stage?

Mia refused to give up.

“I know that Fable is a fighter, and I wanted to try everything to give her the chance to survive.”

Finding a Solution

Fable’s first surgery was the placement of a Blalock-Taussig (BT) shunt in the one ventricle of her heart. A shunt is a small tube used in babies with heart problems, like a single ventricle. It helps improve blood flow to the lungs so the blood can get more oxygen. A BT shunt is a temporary fix until the patient can have more surgeries to correct the underlying heart issue.

Fable’s SVT worsened and at 4 months old she experienced cardiac arrest and needed an immediate medical intervention to restart her heart. She had multiple arrhythmia and other complex medical conditions that were not responding effectively to a variety of other medical treatments. As Fable’s condition had reached a critical point, the pediatric cardiac team made the decision to perform a high-risk intracardiac electrophysiology (EP) study with radiofrequency ablation — a risky procedure for a 5-month-old baby.

“Fable’s SVT was so bad that she kept having it almost every day, for hours at a time despite her being on 5-6 very strong and potentially toxic medicines to try to control this,” said OU Health pediatric cardiac electrophysiologist, Dr. Shashank Behere, M.D., assistant professor at the University of Oklahoma College of Medicine. “Usually, having a few brief episodes of this problem would not have been a life-threatening concern, but in Fable’s case, the SVT was so terribly stubborn and dangerous that there was a real chance that it could kill her prematurely, either by worsening her heart failure or from the tremendous amount of medicine being used to try to stop the SVT, and failing to do so.”

A Lifeline

Intracardiac electrophysiology (EP) study with radiofrequency ablation is a specialized procedure used to diagnose and treat complex heart rhythm disorders, particularly in patients who may have additional health risks or complications. An EP study involves inserting thin steerable cables into the heart to check its electrical activity and to map out abnormal rhythms. Radiofrequency ablation uses heat to destroy the abnormal tissues and block the abnormal signals to fix the rhythm. During the procedure, cables are guided to the heart, and heat is applied to specific areas. While usually safe, it can have complications. This approach helps manage serious arrhythmias, especially in high-risk patients such as Fable.

“While ablation is standard therapy for older kids, it is rarely done in such small children,” said Dr. Behere. “It is exceptionally rare to consider in babies under the age of 1, and it is almost unheard of to do in someone under 1 year with such an extremely complex heart condition as she had.”

In June 2024, under the leadership of OU Health pediatric cardiac electrophysiologist Dr. Anjan Shah, M.D., associate professor at OU College of Medicine and head of pediatric electrophysiology and ACHD service; Dr. Behere; the Oklahoma Children’s Hospital electrophysiology and catheterization lab team, and cardiac anesthesiologist and associate professor at OU College of Medicine, Dr. Randall Schwartz, M.D., along with a host of other team members, successfully performed a high-risk intracardiac electrophysiology study with radiofrequency ablationon 5-month-old Fable.

The procedure was made even more challenging when the usual paths to Fable’s heart were found to be blocked off by clots in her major blood vessels. The team did not allow the difficulty to stop them — Dr. Shah fed the catheters to Fable’s heart through her right arm vessels rather than her neck or groin. Using the most state-of-the-art mapping system technology the team precisely homed in on the target area in Fable’s heart and ablated it effectively to resolve the problem.

Specialized Care for Complex Cases

Since her ablation, Fable has been completely free from SVT, allowing her to grow stronger as she awaits a new heart in the Cardiovascular Intensive Care Unit (CVICU), where she has lived since birth. Her mother, Mia, works nearby and cherishes every moment she can spend with Fable during breaks and after work. The next step for Fable is a heart transplant.

“Fable is waiting on a heart transplant,” said Mia. “And because of the successful ablation, the medication she is on, and the care she receives, she is ready when a heart becomes available.”

In the meantime, she is growing and is slowly meeting milestones. She loves music and is instantly calmed by her mother’s singing. She is naturally curious, and her favorite toys are vibrating teethers.

“Fable is the youngest patient to undergo the procedure at Oklahoma Children’s Hospital and possibly across the country,” said pediatric cardiologist Dr. Arshid Mir, M.D., chief of pediatric cardiology, head of cardiac imaging and associate professor at OU College of Medicine. “Very few pediatric heart centers could have done this procedure on a patient this age and weight with such a great outcome. This case highlights the exemplary skills our electrophysiology team has.”

Oklahoma Children’s Hospital provides comprehensive, multidisciplinary pediatric cardiac care unavailable anywhere else in the state.

“We were able to safely and effectively cure Fable’s electrical problem because we have an incredible team of cardiologists, cath lab nurses and cardiac anesthesia,” said Dr. Shah. “We also have access to state-of-the-art technology, and the requisite experience and technical proficiency for this type of complexity.”

Learn more about Oklahoma Children’s Hospital OU Health Heart Center or request an appointment by calling (405) 271-4411.