Tetralogy of Fallot: Fiona's Story

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Today, 7-year-old Fiona is an agile climber who also loves to roller-skate and ride her bike. Her active lifestyle is possible thanks to surgeries she's received at Children's Hospital of Philadelphia (CHOP) to correct a serious heart problem.

Fiona Lianne was 20 weeks pregnant when she and her husband Chris found out there was a problem with their baby’s heart during a routine ultrasound. Their Lehigh Valley obstetrician wasn’t certain of the exact problem but told the couple it was serious enough that Lianne should be seen at Children’s Hospital of Philadelphia.

After tests at CHOP, a pediatric cardiologist told Lianne and Chris their unborn baby had a combination of heart defects known as tetralogy of Fallot (TOF). There was a hole between the lower chambers of the heart (ventricular septal defect); an overriding aorta, where the aorta is on top of both ventricles instead of just the left ventricle; a narrowing of the pulmonary valve (pulmonary stenosis), which affects blood flow from the heart to the lungs; and hypertrophy, where the right ventricle becomes thicker and more muscular than normal as a result of working harder to pump blood through the narrow pulmonary valve.

The couple was devastated by the diagnosis, but hopeful CHOP's cardiac and fetal experts could provide the care their baby needed.

Lianne would need to be monitored closely for the remainder of her pregnancy, and the baby would need heart surgery soon after birth. Plans were made for Lianne to deliver in CHOP's Garbose Family Special Delivery Unit (SDU), the world’s first birth facility in a pediatric hospital specifically designed for mothers carrying babies with known birth defects.

Heart surgery as a newborn

When Lianne was 37 weeks into her pregnancy, she delivered Fiona in the SDU. Mother and baby each had their own medical team attending to their needs before, during and after the birth.

Three days later, Fiona had heart surgery.

J. William Gaynor, MD, an attending surgeon in CHOP’s Cardiac Center, placed a patch in Fiona's heart to close the hole between the two ventricles (lower chambers of the heart) and added a conduit to allow blood to flow from the right ventricle to the pulmonary artery. He needed to wait until Fiona was older — and her heart was larger — to correct the problem with her pulmonary valve.

“Once she started to recover from the surgery, we could see the difference in her skin color,” Lianne says. “She had been dusky before. Now she was pinker.”

Challenges with reflux and aspiration

As Fiona recovered from heart surgery, she began to show signs of another problem: reflux.

She hadn't been able to hold down her milk and reflux medication wasn't helping as much as doctors had hoped. Doctors discovered Fiona was aspirating when she swallowed.

When Fiona was 2 weeks old, she underwent surgery again. Michael Nance, MD, reinforced Fiona's lower esophageal sphincter in a procedure called a Nissen fundoplication to reduce the reflux and placed a G-tube so she could be fed without aspirating.

When Lianne and Chris finally brought Fiona home after a month at CHOP, they made arrangements for follow-up cardiac care in Allentown, PA, near their home. They brought Fiona in for regular visits with Ruchi Gupta, MD, a pediatric cardiologist in a practice that coordinated closely with Fiona’s cardiology team at CHOP and is now a part of that team, as the Cardiac Center, Allentown.

Lianne and Chris brought Fiona back to CHOP in Philadelphia for her GI issues. As part of that care, another diagnosis was made. The cause of her aspiration problems was found to be a tracheoesophageal fistula, a condition in which a hole connects the airway to the esophagus, allowing food to pass into the airway when swallowing.

When Fiona was 4 months old, a third surgery closed the hole, eliminating her swallowing problems. The G-tube was removed soon after.

After her third surgery, Fiona had a long stretch of a normal, healthy life. She hit her developmental milestones on schedule, and she began walking early at 10 ½ months.

A second heart surgery

As Fiona approached her sixth birthday, she began showing signs she was outgrowing the heart conduit that was placed when she was 3 days old. That winter, she got very sick.

In January, Fiona developed respiratory syncytial virus (RSV) and ended up being hospitalized for nine days with pneumonia. A cardiac MRI and diagnostic catheterization procedure confirmed that the original conduit in Fiona’s heart was outgrown and needed to be replaced.

A second heart surgery was scheduled for the summer, again with Dr. Gaynor, to replace the conduit and pulmonary valve.

Two days after the procedure, Fiona was up and walking. A week later she was well enough to go home. She returned to school with her classmates in the fall.

'She can climb anything'

Fiona and Dr. Gupta Fiona with Ruchi Gupta, MD, at the Cardiac Center, Allentown Today, 7-year-old Fiona leads an active life. She enjoys roller-skating, biking and climbing. “She can climb anything,” Lianne says of her daughter.

Fiona also loves reading, drawing and music. She even writes her own songs.

Lianne and Chris say they appreciate the expertise and compassion their daughter and family have received from CHOP's medical teams in Philadelphia and Allentown. The family is thankful that they can see a local cardiologist in the Lehigh Valley while still having access to the cutting-edge resources of CHOP’s Cardiac Center — one of the world’s leading pediatric heart centers.

“Dr. Gupta is very approachable and very caring,” Lianne says. “She’s our trusted adviser. She’s so knowledgeable and so great with Fiona.

Everything about our experience with CHOP has been wonderful. The level of care is absolutely the best. They’ve made all this possible

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