Heart Healer

Family finds heart surgeon for rare defect in Memphis

It’s the news no expectant parent wants to hear during a routine ultrasound – news that their baby has a malformed heart.

Madison Cox was 20 weeks pregnant when she and her husband, Mason, learned of their baby boy’s serious condition. 

The Selmer, Tenn., couple was referred to the Fetal Center at Le Bonheur Children’s Hospital, where a pediatric cardiologist diagnosed the baby with Ebstein anomaly – a rare congenital defect that affects the heart’s tricuspid valve.

The couple started looking for options. Knowing he would eventually need surgery to correct his defect, the Cox family researched pediatric heart surgery programs across the country. Again and again, their search led them back to Le Bonheur.

“I joined online support groups for moms of Ebstein’s patients, and everyone told us Le Bonheur was the best. Other programs we looked into had seen Ebstein’s cases, but none had done as many as Dr. (Christopher) Knott-Craig had with the same outcomes,” said Madison. “We couldn’t believe the surgeon we needed was less than two hours from our driveway.”

Knott-Craig’s pioneering work with Ebstein and other congenital heart defects is a big reason Le Bonheur’s Heart Institute was named a Top 10 program by U.S. News & World Report in 2018, in large part because of strong surgical outcomes.

His Ebstein’s work was recently highlighted in The Annals of Thoracic Surgery, where Knott-Craig co-authored “Surgical Management and Outcomes of Ebstein Anomaly in Neonates and Infants: A Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis.” Knott-Craig is chief of Pediatric Cardiothoracic Surgery at Le Bonheur and executive co-director of the hospital’s Heart Institute.

He has surgically repaired 32 Ebstein cases in his career, one of the largest caseloads in U.S. history. Le Bonheur has performed four infant Ebstein repairs since 2017 with 0 percent mortality, compared to the national average of 9 percent.

The report looked at index operations reported in The Society of Thoracic Surgeons Congenital Heart Surgery Database: 255 neonates and 239 infants from 95 centers. The most common primary operation performed among neonates (younger than 30 days old) was Ebstein repair, followed by systemic-to-pulmonary shunt and tricuspid valve closure. The most common primary operation for infants (31 to 365 days old) was superior cavopulmonary anastomosis followed by Ebstein.

Researchers found that Ebstein anomaly in early infancy is very high risk and a variety of operative procedures can be performed. A dedicated prospective study is required, they wrote, to fully understand optimal selection of treatment pathways to guide a systemic approach to operative management.

“We want to continue to study this disease, as medicine advances and make sure we’re giving all neonates and infants the best shot at a typical childhood,” Knott-Craig said.