Hypoplastic Left Heart Syndrome
Parents-to-be Matthew and Angel Garrett were informed at a 20-week anatomy ultrasound that their son, Gentry, had a rare congenital heart disease called hypoplastic left heart syndrome (HLHS). Though the road ahead was marked with uncertainty, the Garretts are grateful the path led them to Children’s of Alabama. “Nobody ever wants to be in a hospital,” Angel said. “It’s not a choice anyone would make, but we couldn’t be more thankful that we’ve had Children’s through it all.” Gentry was transferred to Children’s just two hours after he was born in preparation for his first of three open heart surgeries. Gentry’s first surgery involved building a new aorta, making the right ventricle pump blood to the body through the new aorta, then making the right ventricle pump blood to the lungs through a new path to the pulmonary artery. This is known as the Norwood procedure. “From the very start, the doctors were very open and honest with us about what Gentry needed to survive,” Angel said. “We knew this first surgery, while necessary, was going to be a hard one. But thankfully, Children’s survival rate for this surgery is incredibly high and he came through just fine.” Gentry had his first surgery at 5 days old with the hope that it would suffice enough for him to make it to 6 months old, giving him time to grow before his next surgery. But before he was even four months old, it became clear that the second surgery couldn’t wait. Gentry’s oxygen saturation levels were low and he was placed on oxygen. “When he made it to 3 months and 3 weeks old, they knew surgery couldn’t wait any longer,” Angel said. “The second surgery had to be done.” Gentry had his second surgery, known as the Glenn procedure, which finishes rerouting blood flow from the upper body to the lungs. As a result, the blood will go directly to the lungs without circulating through the heart at all, and the heart no longer has to work twice as hard. The surgery was successful though Gentry faced a hard recovery that kept him in the hospital for over a month. “It was tough,” Angel said. “His oxygen saturation got incredibly low, and they had to perform CPR on him four times. He also had a stroke on the left side of his brain, but he made it.” When Gentry turns 3, doctors plan to perform the third surgery, the Fontan procedure, redirecting blood flow from the lower body to the lungs. In babies with HLHS, low oxygen blood from the lower part of the body mixes with high-oxygen blood. After the Fontan procedure, low oxygen blood and high oxygen blood no longer mix. This lets the heart deliver only high oxygen blood to the body. Gentry is currently doing follow-up testing as part of Children’s collaboration with the Mayo Clinic’s Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome. The nationwide consortium works to accelerate innovation and discovery science by bringing clinical trials and expertise to patients across the country. Gentry is the first Children’s patient to participate in the consortium. In the meantime, medicine and frequent check-ins with his doctors allow Gentry to be home with his family and grow to be a happy and active toddler. “We don’t know what to expect over the next few months or years, but we’re thankful we have the doctors, nurses and staff of Children’s here with us,” Angel said. “They have taken such incredible care of Gentry and they take care of us, too. They have made an incredibly hard experience a little easier. I couldn’t imagine being anywhere else. No other hospital compares to Children’s.”
