When first-time parents Genell and Daniel Reynolds held their newborn little boy Grayson, they were overjoyed. Genell had a healthy, uneventful pregnancy and Grayson was delivered by a midwife in a St. Louis hospital in August 2018. Excited to be home with their new baby, the Reynolds took Grayson home after about 12 hours.
Over the next day, Genell began noticing that when Grayson cried, he turned a purplish color. Although she admits she didn’t have much experience with babies, her instincts told her something wasn’t right.
A few hours later, the midwife’s assistant visited the Reynold’s home to conduct a pulse oximetry screening to check Grayson’s pulse rate and blood oxygen level. In 2014, “Chloe’s Law” went into effect requiring all Missouri hospitals to perform this test within 24 hours after birth to detect newborn critical congenital heart disease.
This screening may have saved Grayson’s life. Grayson’s oxygen levels were dangerously low—under 63 percent—and his lips had a bluish tint. He was quickly taken to St. Louis Children’s Hospital.
One of the first physicians to see Grayson was Ginnie Abarbanell, MD, a Washington University pediatric cardiologist and co-director of the Echocardiography Lab at Children’s.
“This pulse ox screening is key to picking up congenital heart disease (CHD), because it’s not always obvious from the outside,” she says. “What Grayson had may not have been picked up in a fetal echocardiogram.”
Grayson’s echocardiogram at Children’s didn’t immediately show the problem. It took some detective work by Dr. Abarbanell and her team.
“At first, we had to scratch our heads to figure out why he was having symptoms because what we saw on the echo didn’t fit with why he was blue,” Dr. Abarbanell says.
Grayson was put on prostaglandin, which improved his cyanosis. Dr. Abarbanell pieced it together that Grayson had an extremely rare heart defect, cor triatriatum dexter. The defect usually occurs on the left side, which is called cor triatriatum sinister.
Cor triatriatum is a rare congenital cardiac defect in which the atrium is divided into two chambers by a membrane causing obstruction to the blood flow in either the left atrium (cor triatriatum sinister) or the right atrium (cor triatriatum dexter) eventually leading to cardiac failure. There are limited cases of cor triatriatum dexter described in the literature.
Grayson had come to the ER in the middle of the night. Within a few hours, he was diagnosed with cor triatriatum dexter by echocardiogram and was being scheduled for surgery that same day.
“He was getting sick quickly,” Dr. Abarbanell says. “When we first saw him, he was not only blue, but also turning grayish because he wasn’t getting enough oxygen. Once he was put on a medication called prostaglandin he stabilized.”
However, surgery was the only option to fix Grayson’s heart long term. This is when Dr. Abarbanell’s husband, Aaron Abarbanell, MD, a Washington University pediatric cardiothoracic surgeon at Children’s, met Grayson and his family. He performed surgery two days after Grayson was born.
“Grayson’s heart defect was tricky,” he says. “It was hard to pick up on the EKG. His defect is uncommon on the left but extremely rare on the right side. Grayson had a severe defect with quite a large flap that needed to be addressed.”
Because cor triatriatum dexter is so rare, Dr. Aaron Abarbanell had not performed corrective surgery on the right side before, although he has performed corrective surgery on the left side. “The concept is the same,” he explains. “We put Grayson on cardiopulmonary bypass, removed the membrane and he was good to go.”
“When we realized he needed surgery, I felt overwhelmed–it was the first moment as a parent when I felt helpless,” Genell says. “But I was grateful there was a solution for his heart problem and I trusted Dr. Abarbanell. He explained everything and kept me in the loop. He also was passionate about his work, which I think makes you better at your job. He told me he would treat Grayson like one of his kids until he could get him back to me. We had such a good experience with the hospital in less than ideal circumstances.”
While it takes about eight weeks for the breast bone to heal after this surgery, Grayson was home about a week after surgery.
At his follow-up appointment two weeks later, Grayson was gaining weight and was happy and healthy, Dr. Ginnie Abarbanell says.
“This is the best of all stories. Since Grayson’s heart was fixed through surgery, he shouldn’t have any cardiac issues for the rest of his life. Grayson’s good outcome illustrates the importance of pulse ox screenings.”
Dr. Aaron Abarbanell agrees. “This is proof positive the system works. When the pulse ox is different than it should be it means something is wrong and it’s worth investigating.”
Genell agrees. “I don’t know if I can do anything to repay what the hospital did for my son, but I want to raise awareness about the importance of this pulse ox test and to listen to your instincts as a mother.”