Young patient's 'special light' shines after surgery for rare heart defect

Sochi Nwoguh charms everyone she meets.

This gregarious four-year-old loves dinosaurs, hide and seek and “The Lion King.” Staff at the Kentucky Children’s Hospital (KCH) Congenital Heart Clinic excitedly anticipate her visits. Her cardiologist, Callie Rzasa, M.D., describes her as having a “special light” that shines bright –¬ even though the first three years of her life were a frenzy of appointments, scans, medications and ultimately a major heart surgery. 

Sochi spent the first month of her life in the neonatal intensive care unit at KCH with meconium aspiration. While she as there, scans revealed multiple defects in her heart. The official diagnosis was a partial anomalous pulmonary venous return (PAPVR) with inferior sinus venosus defect and partial cor triatriatum. In other words, all the veins from her right lung were draining into the right atrium of the heart instead of the left. 

“That type of atrial septal defect is very uncommon, said Carl Backer, M.D., chief of pediatric cardiothoracic surgery and Sochi’s surgeon. “I have probably only operated on four or five patients with this in my 30-year career.”

This combination of defects caused an excessive amount of blood to re-circulate through the lungs. As a result, Sochi’s heart was significantly enlarged. She was frequently out of breath and had a racing heartbeat. But the most telltale sign was what doctors call “failure to thrive”. Sochi wasn’t gaining weight; she was in the 15th percentile for weight gain.

“If left undiagnosed or treated, she could have potentially developed pulmonary hypertension which could lead to the need of a lung transplant,” said Backer. “She would have developed atrial arrhythmias, which would lead to congestive heart failure.”

“Her dad and I were terrified,” said Cynthia. “She is our first child, and we had no idea what to expect. There is no history of heart defects in our family, so it was confusing trying to navigate it all.”

For three years, Sochi had regular check-ups with Rzasa who watched her closely to make sure that she could grow to an optimal size for her complex surgery, as well as to manage Sochi’s pulmonary edema. Despite her health, Sochi was still a bundle of joy and happiness, which made treating her both a delight and a challenge.

“At times, she was too wiggly for excellent echo imaging, so our nurses, medical assistants and sonographers worked to make the echo feel like a game to avoid needing sedation,” said Rzasa. When the right side of Sochi’s heart enlarged and her growth plateaued, it was time for surgery.

“At first the family was understandably quite worried, said Rzasa. “We spent a lot of time describing management options and the expected surgery.  When it was time to have her surgery, everyone felt very ready and comfortable. Dr. Backer and the surgical team did an outstanding job with challenging anatomy in a small toddler.”

On July 13, 2022, Sochi was connected to a heart-lung bypass machine. Her heart was stopped while Backer and his team reset the cor triatriatum, the thin membrane that had divided the left side of Sochi’s heart into three chambers instead of two. To correct the errant pulmonary veins, Backer used Sochi’s own heart tissue to create a tunnel – called a baffle¬¬¬ – within her heart to direct the pulmonary veins through the atrial septal defect to the left atrium, where they’re supposed to go.

“I am forever grateful to Dr. Rzasa and the clinic staff who were with us every step of the way for the past three years from Sochi’s diagnosis till the surgery,” said Cynthia. “She explained all the medical terms in layman’s terms and with images and drawings. I am also thankful to Dr. Backer who performed the surgery for saving Sochi’s life. The nurses in the PCICU were amazing and knew all the right things to do to keep her comfortable.”

Following her surgery, Sochi spent two days recovering in the pediatric cardiac intensive care unit. Cynthia recalls Sochi being in a lot of pain for the first few days, but after a week, she was back to her happy, joyful self.

“By the end of the first week, she was asking to go to the playground,” Cynthia said.

Sochi’s recovery was free of complications. After following up with Backer, Sochi checks in regularly with Rzasa.

“One of the things that I treasure most about my role is getting to take care of these children longitudinally,” said Rzasa. “Sochi has always been a bright, curious and engaging little girl. This was evident as an infant and is now undeniable in this terrific little toddler.”

Sochi just turned four, and she is thriving. Her appetite has increased and she’s gained weight. She has a lot of energy, helpful in keeping up with her new baby sibling.

“We are thankful that Sochi’s heart defect was discovered early and closely monitored for three years before the surgery,” said Cynthia. “I don’t know what would have happened if we were back home in Nigeria where access to such specialized heart care is still inaccessible to most people.”