Journey of an Infant with Hypoplastic Left Heart Syndrome
It Takes Faith & Lots of Love
After Stephanie Rodriguez gave birth to Amara, she and her husband Juan didn’t know they would need to rely on prayer and an advanced pediatric cardiology team.
Shortly after birth, in December of 2021, Amara’s heart rate dropped and she had trouble breathing. Even though Stephanie had a smooth pregnancy with no complications, something was wrong.
“They rushed her to the NICU to stabilize her and to figure out why her oxygen levels were so low,” Stephanie said. “After a few days, they finally did an echocardiogram to see if something was wrong with her heart.”
Her doctors diagnosed Amara with hypoplastic left heart syndrome.
What is Hypoplastic Left Heart Syndrome?
HLHS is a serious congenital birth defect that affects how blood flows through the heart. In babies with this heart syndrome, also known as HLHS, the left side of the heart is underdeveloped.
Typically, the left side of the heart pumps oxygen-rich blood into the aorta, the large artery responsible for carrying blood into the rest of the body. Babies with HLHS do not pump enough oxygenated blood into the body due to the abnormalities present on the left side of the heart.
Amara would need highly specialized surgery for her condition, so she was transferred to The Children’s Hospital of San Antonio and placed under the care of Dr. Daniel Nento, a pediatric cardiothoracic surgeon.
“She underwent her first open-heart surgery at just 6 days old,” Stephanie said. “It was one of the scariest days of my life.”
Treatment for HLHS
Babies with Hypoplastic Left Heart Syndrome typically need three surgeries over several years to repair their heart so that it can fully function on its own:
- The Norwood
- Procedure Bi-directional Glenn Shunt Procedure
- The Fontan Procedure
During these procedures, surgeons reconstruct the heart and its circulatory system to function as it should.
The Norwood Procedure
The first surgery is called the Norwood procedure, which Dr. Nento performed on Amara within the first week of her life.
This surgery allows the right ventricle to do the left ventricle's job and pump blood into the body rather than just pump blood into the lungs.
During the Norwood procedure, surgeons build a new aorta from the bottom part of the pulmonary artery and direct blood flow from that artery into the body.
In addition, they create a new pathway, known as a shunt, to direct blood into the lungs.
This procedure is a temporary fix to keep blood flow sufficient while babies grow strong enough to survive the second procedure, which typically takes place at around six months old.
In Amara’s case, the Norwood procedure was a success at first. She had to have a gastrostomy tube (G-tube) inserted to help her get enough nutrients for her development, and she was finally able to go home on February 18, 2022.
Unfortunately, her pulmonary arteries didn’t grow as hoped, so she was readmitted to The Children’s Hospital of San Antonio, where Dr. Nento performed another surgery to enlarge them.
During this surgery, Dr. Nento enlarged the pulmonary arteries to help the blood flow. He also reconstructed the valve conduit into a larger one to provide more blood to Amara’s lungs.
“All in all, that surgery took 14 hours,” Stephanie said. “But it worked.”
Amara recovered in the NICU for another month, when she gained enough strength to come home finally.
Removal of the Shunt
She underwent the second surgery for HLHS in September, which involved the removal of the shunt placed during the Norwood procedure and creating a new pathway for blood from the upper body to travel directly to the lungs.
Typically, surgeons do this by disconnecting the superior vena cava from the heart and then connecting it directly to the pulmonary artery.
The superior vena cava is the large vein that returns blood from the upper body (the head and the arms) to the heart.
By connecting it directly to the pulmonary artery, blood from the upper body can now go directly from the body and into the lungs, thus bypassing the heart. This gives the heart a break, allowing it to pump blood into the body only and not the lungs.
The Fontan Procedure
Amara will get the final surgery in this series, the Fontan procedure when she is 18 and 36 months old. In this procedure, surgeons disconnect the inferior vena cava from the heart and connect it to the pulmonary artery.
The inferior vena cava is the large vein that returns blood from the lower body (the legs and the abdomen) to the heart.
Now, blood from the lower body can also go directly into the lungs and bypass the heart. Surgeons will also create a small hole to allow some blood to flow back to the heart, which helps manage the amount of blood flow into the lungs and gives the body some time to adjust to this new system.
Once this surgery is final, blood from both the upper and lower body will go directly to the lungs, and oxygenated blood will travel directly to the heart. The single functioning ventricle in the heart now only has to pump oxygenated blood into the body.
The Family Now
Although Amara still has this final surgery, her parents are thrilled to have her home. Soon she will have her first birthday, and they will celebrate her big milestone and how far she has come over the past 12 months.
“Amara has so much energy now and you can tell her little personality is coming out,” Stephanie said. “She has more energy for her therapies, whereas before, she would just get very tired very quickly. And we can play with her a bit longer too, which we love.”
Her brother has been happy to have his little sister home, too.
“You can tell he loves her; he’s been really sweet,” Stephanie said. “He’s a little scared because she’s so tiny, and he doesn’t want to hurt her, but he tries to be helpful. He likes to help me give her medication through her G-tube.”
Stephanie said that while the whole experience tested her faith, she relied on prayer to keep her strong, and she will be forever grateful to the team at The Children’s Hospital of San Antonio for saving her daughter’s life.
Ultimately, Amara answered a prayer for both parents who wanted a sibling for their 7-year-old son.
“I wouldn’t have been able to do this without Amara’s cardiology team,” Stephanie said. “They really prepared me for everything, every step of the way. When I walked into the room after that first surgery, they had already shown me pictures and told me that she wasn’t going to look like my baby, and things like that made the entire process easier. It was so tough, but they got me through it.”
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