A tiny heart, a big battle

At three years old, Lydia Cuasito is an active, energetic, healthy toddler who keeps up with her older sisters. For a child who has undergone multiple complex heart procedures, her strength is nothing short of extraordinary.

"Whenever Lydia's older sister talks about her, she always tells everybody that her sister has a special heart and that she loves her baby sister so much because she had a broken heart when she was a baby," said Katrina Gonzalez, her mother.

At her most recent appointment, pediatric cardiologist Stephen Nageotte, MD, MBA, Director of the Pediatric Cardiac Catheterization Laboratory at Loma Linda University Children's Hospital, described her heart as looking "quite fantastic."

Her journey to this moment has been filled with many challenges and life-saving interventions.

When Lydia was born in October 2021, Gonzalez knew the road ahead would not be easy. Lydia was diagnosed with two life-threatening congenital conditions: Tetralogy of Fallot with pulmonary atresia, a rare and complex heart defect, and a congenital diaphragmatic hernia (CDH), a condition in which the diaphragm fails to form properly, allowing abdominal organs to press against the lungs.

In Lydia's case, the only way blood reached her lungs was through a vessel called the patent ductus arteriosus (PDA), a temporary artery that all babies are born with. The PDA usually closes after birth since it helps bypass the lungs while in the womb. However, keeping it open for Lydia was important, as it was her only source of blood flow to the lungs.

Before Lydia could undergo heart surgery, doctors had to first address the diaphragmatic hernia. At just three days old, she underwent surgery to close the hole in her diaphragm, allowing her lungs the space to grow.

A month later, she had a cardiac catheterization, a procedure in which doctors inserted a stent to keep her PDA open, ensuring blood could continue flowing to her lungs. The approach bought her time to grow before undergoing a complete heart repair.

"We were able to do that first procedure, normally a surgical procedure, without needing surgery. So we spared her to have one open heart surgery," Nageotte said.

At nine months, Lydia underwent open-heart surgery to correct her condition, closing the hole in her heart and reconstructing her pulmonary artery using her own tissue.

The procedure was a collaboration between cardiologists and cardiothoracic surgeons. Using soft catheters and wires, the team guided a metal stent into the PDA and inflated it, ensuring blood could continue flowing to her lungs.

"The multidisciplinary approach that we took for Lydia's care, involving various medical teams and Katrina's active participation, contributed to her successful outcome," Nageotte said.

Tetralogy of Fallot is a lifelong condition, meaning Lydia will need regular cardiology check-ups. Since she was born without a pulmonary valve, she will require additional surgeries to replace it as she grows, continuing into adulthood. However, many people with this condition live full, active lives.

"Olympic gold medalist snowboarder Shaun White also has Tetralogy of Fallot and became an elite athlete. I like sharing his story to reassure families that children with this condition can thrive," said Nageotte.

With technological advancements, Lydia's future procedures may not always require open-heart surgery. When she eventually needs a pulmonary valve, it might be possible to place it in the cath lab using a minimally invasive procedure instead. The field continues to evolve, offering hope that her journey will become easier with time.

Despite these medical hurdles, Lydia's future remains bright. Her family embraces every moment, from traveling to the Philippines and visiting the Grand Canyon to playing in the snow and enjoying the beach.

"Seeing Lydia healthy and happy is the most rewarding," Katrina said.