A Journey of Strength and Perseverance

Reign's Battle with a Rare Heart Condition

Healthy Baby ReignErika Piña's life took an unexpected but joyful turn when she discovered she was pregnant in the spring of 2022. Aside from experiencing morning sickness, everything was going smoothly, and she was excited about the new chapter ahead. But during a routine ultrasound, the excitement of finding out she was having a boy was overshadowed by news that there may be something wrong with his heart.

Erika was referred to Dr. Krittika Joshi, a pediatric cardiologist at the CHRISTUS Children’s Heart Center, as well as Dr. Lissa Melvin, a Maternal-Fetal Medicine specialist in the department of Obstetrics & Gynecology at CHRISTUS Children’s, for specialized care and a more advanced diagnosis. Drs. Joshi and Melvin would soon play crucial roles in monitoring Erika's pregnancy and ensuring the wellbeing of both mother and baby.

At 24 weeks gestation, an ultrasound revealed that Erika’s baby had tricuspid atresia with pulmonary stenosis (TA/PS), a rare congenital heart defect characterized by underdevelopment or malformation of the structures on the right side of the heart, especially the tricuspid valve, given the baby’s one single properly functioning ventricle.

“The right ventricle, pulmonary valve, and pulmonary arteries are abnormally small or poorly developed in individuals with TA/PS,” Dr. Melvin explained. “Consequently, the right ventricle is unable to pump blood to the lungs effectively, leading to restricted blood flow and reduced oxygenation.”

Without intervention after birth, TA/PS poses life-threatening risks, particularly in infancy.

The remainder of Erika's pregnancy journey was fraught with anxiety as she constantly worried about potential complications, but she was thankful for the care and guidance of Drs. Joshi and Melvin and the expertise offered at the CHRISTUS Children’s Fetal Care Center. At 40 weeks, she underwent an induced labor at CHRISTUS Children’s, which eventually resulted in an emergency C-section due to complications arising from a fever she developed, as well as her son’s heart condition.

Finally, on December 28, 2022, at 9:06 p.m., Reign Piña was born.

Erika was only able to catch a glimpse of her newborn son before he was taken to the Neonatal Intensive Care Unit (NICU) where he was hooked up to monitors and tubes for immediate and advanced medical care. He also continued to receive care from Dr. Joshi, who would continue to help guide Reign’s treatment plan for his heart condition.

“Dr. Joshi was amazing, and I am so thankful for her guidance throughout this time, but especially in those first few months,” Erika said.

Soon after Reign’s birth, another life-threatening medical condition emerged; Reign was diagnosed with biliary atresia (BA), a condition in which bile flow from the liver to the gallbladder is blocked, causing bile to be trapped inside the liver.

After a team discussion, a decision was made to treat the heart first and thus, at a mere 2 weeks old and weighing less than 7 lbs., Reign underwent his first open heart surgery: a Blalock-Taussig (BT) shunt surgery. This procedure, performed by Dr. Victor Bautista-Hernandez, a cardiothoracic surgeon at CHRISTUS Children’s Heart Center, aimed to redirect blood flow into the lungs and thus, improve oxygenation throughout the body.

“During a BT shunt surgery, a small tube or conduit is created to establish a connection between one of the main arteries that supplies blood to the body and one of the pulmonary arteries,” Dr. Bautista-Hernandez explained. “This allows some of the blood from the artery to flow directly into the lungs, bypassing the underdeveloped or obstructed structures on the right side of the heart. The BT shunt helps increase blood flow to the lungs, providing better oxygenation and relieving the cyanosis associated with conditions like TA/PS.”

This initial surgery was a success, and he arrived at the PICU with a closed chest and improved oxygenation and had a smooth recovery. This was the first step in a series of procedures Reign would need to address his heart condition.

On February 24, 2023, Reign underwent a surgical intervention known as the Kasai procedure to address his BA. Performed by Dr. Ann O’Connor, a pediatric surgeon at CHRISTUS Children's, it involved the removal of the problematic bile ducts outside the liver. In their place, Dr. O’Connor created a connection between the small intestine and the liver, enabling the drainage of bile. The success rate of this procedure varies, with a 30% chance of lifelong success, a 30% chance of complete failure, and a 30% chance of eventual failure. Additionally, a G-tube was inserted to ensure Reign received necessary nutrients for growth.

The following month was marked by a rollercoaster of emotions for Erika, as Reign’s liver function remained unstable. Despite initial positive signs, it was discovered that Reign had a bile duct leak, necessitating another procedure and the placement of a drain. After a brief period at home, Reign developed a fever, leading to multiple hospital visits due to various complications. His specialists at CHRISTUS Children’s worked closely with Erika to address every concern and complication, and finally, in April of this year, Reign’s health began to stabilize, and Erika brought him home. He began tolerating feeds, exhibited increased vitality, and showed enhanced levels of activity, a testament to his remarkable resilience.

Although Reign's journey isn’t over yet, each day brings more hope. He is now 8 months old, weights more than 7 lbs., and his body has normal oxygen levels. He is now ready to undergo his second single ventricle palliation (Glenn operation). In addition, his liver health remains a critical concern, and he is being monitored closely by Dr. Lucille McLoughlin, a pediatric gastroenterologist at CHRISTUS Children's, to ensure no additional intervention is needed.

Despite the whirlwind of her first few months of motherhood, Erika has remained steadfast in her love for her son and her hope for a bright future ahead. She has worked tirelessly to balance her role as a college student with her son’s complex medical needs, with sleepless nights and emotional exhaustion a part of her daily routine.

“I won’t sugarcoat it—none of this has been easy,” Erika said. “But I just have to keep going. I have to keep going for Reign.”

Smiling Baby ReignErika said that she is endlessly proud to be his mother, and she’s grateful she has the opportunity to share his story, adding that she is especially thankful for the exceptional care and dedication of the specialists at CHRISTUS Children’s.

“Everyone at CHRISTUS Children’s went to extraordinary lengths to ensure Reign's survival and quality of life,” Erika said. “Thanks to them and to Reign’s own incredible strength, I’m able to share my son’s story of perseverance.”

To learn more about pediatric cardiology care at CHRISTUS Children’s, visit https://www.christushealth.org/get-care/services-specialties/pediatric-care/cardiology.