Surgery helps young child escape heart problems that plagued generations of one family

Tara Sharp will never forget the moment she received the sonogram results.

“The doctor said, ‘There’s something wrong with the baby’s heart; we don’t know if she will survive,’” Sharp said. “I was devastated.”

Six months pregnant, Tara and her husband, Ben, were eagerly awaiting their second child. Now, instead of telling their older daughter to expect a sister, they wondered if the baby would live.

A genetic counselor soon asked about family history of heart problems. One aunt, Sharp learned from her mother, had a mild version of tetralogy of Fallot, the defect detected in a severe form on her own baby’s sonogram. But Sharp’s mom, Heather, revealed more: More than 50 babies on her family tree, including one of Heather's own siblings, had died in infancy due to severe versions of the same heart defect. It was the first time Sharp had heard this element of her family's past.

But Sharp’s pediatric cardiologist in Santa Rosa, Calif., offered hope. “She told us the best surgeon in the world for tetralogy of Fallot is right here at Packard Children’s,” she said.

That cardiovascular surgeon,Frank Hanley, MD, who directs the hospital's Children's Heart Center, invented a surgical repair called unifocalization that has helped hundreds of children survive with complex tetralogy of Fallot. The defect is comprised of several structural abnormalities in and around the heart, including a missing or malformed pulmonary artery to carry blood from the heart to the lungs. To compensate, the body develops small “collateral” arteries that travel from the aorta to the lungs. This abnormal anatomy increases lung blood pressure and causes lung damage. The defect also prevents the body from receiving fully oxygenated blood. Without surgical repair, most patients die in infancy or childhood.

Hanley advised Sharp to deliver the baby, who would be named Elena, at Packard Children’s.

“We wouldn’t know the details of Elena’s collateral blood vessels until she was born and could receive cardiac catheterization,” said Hanley, who is also a professor of pediatric cardiac surgery at the Stanford University School of Medicine. “Most infants with this condition do not require urgent newborn surgery, but about 10 percent do. So we wanted to assess Elena’s condition quickly.”

Sharp was referred to the Center for Fetal and Maternal Health at Packard Children’s, where patient care coordinator Meg Homeyer helped Sharp navigate her remaining prenatal care, including several diagnostic and consultative appointments with Packard Children's specialists. “We try to do everything we can to make the process less difficult,” Homeyer said, noting that some of Sharp’s monitoring was performed in Santa Rosa to spare her extra trips to Palo Alto. “We want the patient’s experience to be as simple, understandable and empathetic as possible.”

Sharp was scheduled to have a cesarean section shortly before her due date. But she went into labor three weeks early.

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