When Jenny Rodgers saw her doctor for her 20-week prenatal visit, she expected normal results, as usual, but she and her husband received a shock— the ultrasound showed that the left side of their son’s heart was not developing properly.

Their unborn son, Aiden, was diagnosed with hypoplastic left heart syndrome (HLHS), a complicated condition that occurs in about one in 6,000 live births. HLHS causes the fetus’ left ventricle, mitral valve and aortic valve to severely under-develop, which in turn causes the left part of the heart to stop growing due to a lack of blood flow. Typically, the left side of the heart circulates oxygenated blood to the rest of the body, but HLHS patients’ hearts aren’t able to perform this function. Blood returning from the lungs to the left atrium must pass through an atrial septal defect to the right side of the heart, making the right ventricle work twice as hard to pump blood to the lungs by the pulmonary artery and out to the body.  

Rodgers, 33, called the diagnosis “earth-shattering,” especially because she was so far along in her pregnancy and now her baby had lowered chance of survival.

“When you’re talking about your baby, 85 percent is a pretty bad number,” she told FoxNews.com. “We’re lucky because a lot of these go undiagnosed. A lot of these babies, moms take them home and at two days old they start having issues, start turning blue, have difficulty feeding or go into cardiac arrest.”

Understanding the complications
Four weeks after the diagnosis, Rodgers had an appointment at Texas Children’s Fetal Center, and she left Benton, La., for Houston, eventually moving into an apartment at the medical center. Rodgers would only see her husband, Phillip, 37, and their son Noah, then about 19 months old, on the weekend or if there was a procedure. The family’s relatives offered support during the last stages of Rodgers’ pregnancy and after the birth.

Dr. Nancy Ayres, medical director of non-invasive imaging and director of the fetal cardiology program at Texas Children’s Hospital, first met Rodgers at her 24-week appointment and saw clearly that the left side of Aiden’s heart was completely underdeveloped: Both the left atrium and left ventricle were very small, as well as the mitral valve, which pumps oxygenated blood back out into the body.

“In the fetus, the right side of the heart does a little more work than the left, so these babies thrive in utero and do well,” Ayres told FoxNews.com.

A baby with severe congenital heart defects can have normal heart rhythm and no symptoms until after the mother delivers and the umbilical cord is cut. At that point, the mother is no longer breathing for the baby, and the baby’s lungs open to oxygenate blood. Ayres knew that when Aiden’s lungs were called into action, the oxygenated blood flow would not get into his body because of his underdeveloped left heart.

After delivery, the flap-like opening between the right and left upper receiving chambers of the patent foramen oval, which is open before birth, closes. When a baby has a severely underdeveloped heart, the oxygenated blood returning from the lungs is trapped in the left atrium due to the closure of the patent foramen oval. With a lack of oxygenated blood getting to the body, children born with the condition may experience brain damage along with multi-system failure.

Often, a mother may not notice her undiagnosed child isn’t eating well and that his color is ashen or bluish and by the time they go to the emergency room, the baby is already at a deficit, Ayres said. Even though a baby is days old, because of the damage, he would not be a good candidate for the cardiac surgery.

“The best outcome for delivery of a baby [with HLHS] would be at a major medical center that can actually take care and are available for emergency procedures after the baby goes from in utero to normal structures— those are the life connections for a baby with [HLHS],” said Ayres, who added that the national mortality benchmark for the stage 1 Norwood open-heart surgery is 15.7 percent, but at Texas Children’s it was 12.3 percent in 2014.

Two surgeries down, one to go
Aiden was born on May 19 and underwent his first Norwood surgery at four days old. During the complicated, risky procedure, doctors reconstructed his aorta and opened his atrial septum so all of the oxygenated blood was able to flow to his body. His right ventricle became the main ventricle pumping to his body, and a small shunt was put on his pulmonary artery to the lung vessels.

Fortunately, Aiden was able to be discharged at six weeks old after the major surgery— which not all babies are able to do.

“The fact that we were able to go to our apartment in Houston, just meant Aiden was doing really well,” Rodgers said.

In the time between surgeries, Rodgers and her husband keep a close watch on Aiden for any signs of heart failure, including writing down every ounce that went into his mouth, weighing him every morning to ensure he is not losing weight, and monitoring the oxygen level in his blood and heart rate.

“The more vigilant you are, as far as watching for signs, they found is the way to prevent these kids from just dying suddenly because you’re watching every single day what’s going on with them,” Rodgers said.

Some babies must remain in the hospital until their second surgery, which is done at around three to six months, when  they begin to outgrown the aortic pulmonary shunt surgeons created to allow pulmonary blood flow to the lungs. Aiden had his second surgery on Sept. 15, and he was released  to return home to Louisiana on Oct. 3.

Developmentally, Aiden looks to be normal and is about one month older than his age.

Aiden is due to have his third surgery between ages 2 and 3. During this operation, doctors will connect the inferior vena cava directly into the lung vessels so that blood from his lower and upper bodies will flow passively into his lungs, allowing his single right heart ventricle to pump oxygenated blood.

“Redirecting the flow allows all the blue blood to directly go to lungs and allows the right ventricle to pump only to the body,” Ayres said.

Aiden will be closely monitored as he ages, especially to ensure his right ventricle functions well. Complications may arise for patients if their heart begins to fail and doesn’t work well. Ayres noted that some patients need heart transplants in their early 20s because their right ventricle fails.

While Aiden is doing well now, there’s nothing to say he couldn’t have a turn in a week, a month, a year, Rodgers said, adding that the family hopes awareness of the condition will lead to research.

“These kids with heart defects, it’s not always genetic [and] can happen as a fluke,” she said. “I want everybody to know it’s out there and to fight for these kids.”