The heart of a warrior

Baby Aria’s journey raises awareness of congenital heart disease

By Audrey Posten, North Iowa Times

Looking at her infectious smile and shock of fuzzy, blonde hair, there’s no question Aria Koenig is an adorable baby. But for the Monona 1 year old, what’s most remarkable is what lies within—the heart of a warrior.

You see, Aria was one of the one in 100 babies born with congenital heart disease. She had five defects in all, including hypoplastic left heart syndrome (HLHS), where her heart’s left side had not developed as strongly as the right.

At just four days old, Aria had her first surgery. It was one of four surgeries she’d have by the mere age of six months.

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Aria’s story began Feb. 15, 2017, when she was born at Winneshiek Medical Center, in Decorah, via C-section to Randee and Eric Koenig.

“The whole pregnancy was good,” Randee recalled. “She was over 8 pounds, a big, healthy baby—bigger than we thought she would be.”

The first day was fine, Randee said, as Aria proceeded to eat well. It was when she went to the nursery that an obstetric nurse noticed Aria’s grayish coloring.

Staff immediately alerted Randee and Eric that Aria’s oxygen stats were very low and that she’d developed a murmur.

Plans were made to send Aria to La Crosse, but when they didn’t have an available flight team, the newborn went to Mayo, in Rochester, instead.

“She was in the NICU that whole day, getting echoes and oxygen and that good stuff,” Randee said. “On day four is when she had her first surgery. That’s the only way she would have lived.” 

Since Aria’s left ventricle was so small, the initial surgery made her heart work as one ventricle. The surgeon, said Randee, hoped that left ventricle would grow enough that he could eventually repair it.

Exhausted from her recent C-section, and in shock over the situation, Randee credited her husband, Eric, with knowing which questions to ask during this crucial period.

“The surgeon put it into terms you could understand,” Eric said.

“They drew a lot for us, in order for us to understand it,” Randee noted. “They’re not only heart surgeons, they’re artists, because they drew it out so you could see exactly what she’s supposed to look like and what she looks like.”

Aria remained in Rochester for 18 days. Her parents were impressed with the quick turn-around, in addition to how well she ate.

“Usually, that’s one of the hardest things for babies after surgery,” Randee said.

Not long after, though, as Randee headed back to work, there was a wrench in the plan. At an appointment, a chest X-ray revealed that the shape of Aria’s heart had changed a bit, so an echocardiogram was performed.

“Her function was down a little bit based on that,” Randee said. “They basically just gave us some medicine to help get her heart beating a little stronger and sent us home.”

Two days later, Randee was home feeding Aria when Mayo called. They wanted her in Rochester immediately.

Aria’s heart function was down to around 20 percent, significantly lower than the 60 or 65 percent of blood the heart should release in each pump. 

“She had calcification and obstructions that developed very quickly,” Randee explained. “They’re not sure why they happened so fast.”

“That calcification, or scarring,” said Eric, “was decreasing the flow of blood.”

During this surgery, a new shunt was put in Aria’s heart, and some things were changed out, hopefully to let her grow a little longer before the next surgery, Randee said.

The surgery went well, but the next day, Aria went into cardiac arrest. Her chest was still open, as a way to relieve any swelling from the previous day’s procedure.

“She ended up with chest compressions for about an hour and they ended up putting her on ECMO (extracorporeal membrane oxygenation), which is the lung bypass machine, and it pumps the blood for her,” Randee described. “She was only on ECMO for two days, which is long enough.”

Randee said this stay was Aria’s rockiest, and prompted discussions about a possible heart transplant.

“When we asked the surgeon, he was very optimistic, but he still wanted us to have that talk just in case,” Eric said. “But you could tell, from him, he wasn’t looking at that as an option. He’s the one who actually goes in and sees [her heart] and looks at it. I wasn’t as nervous going into that because of that.” 

“But we were on some pretty heavy heart failure medications when we left, about 10 meds,” Randee said.

After struggles eating, Aria also had to get a G-tube.

“That’s not fun, but, to me, it was worth it,” Randee said.

During this time, a CT scan found that her arch, which was critically narrow to start and had been fixed during the first surgery, had narrowed again. Randee said a balloon catheter was used to help with the issue.

A chest washout also prompted some repair of the pulmonary artery.

“Then she seemed to do OK,” Randee said. “Feeds took some time, but otherwise she seemed to be doing really well.”

That was May. By an appointment in early August, Aria’s function had decreased again.

“We didn’t even get to leave,” Randee shared. “They admitted her to the ICU right away. They put her on an IV drip of a med to help increase that function because it had decreased so much there were concerns about her going into surgery with how low it was functioning at that point.” 

During this surgery, Aria’s heart became biventricular, with both the right and left ventricles functioning.

“The surgeon’s goal the whole time was to get her to an anatomically normal heart, which she now has,” Eric explained. “It’s just the plumbing around it is a little bit different.”

Because of that plumbing, he said Aria had a heart catheterization procedure at the end of October.

“The plumbing just kind of got in the way of things,” he said. “Otherwise, her heart functions normally, like anybody else’s. I would say she’s a normal baby.”

Aria is still on a few heart medications, but only has check-ups every two months now. Doctors are keeping a close eye on her pulmonary valve, and, at some point, another CT scan will be performed to check out her arch.

“The probability is high that she’ll need surgery again down the road just because she is so complex, but we’re hoping a long time,” Randee noted.

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Randee said Aria’s surgeon admitted she was one of the trickiest cases he’s seen. The cardiologists often talk about her anatomy.

“Every heart kid is different,” Randee commented. “Aria had five different defects. I became close with a couple heart moms there, but their kids’ defects have been very, very different.

“Even if they are the same type of defect, the severity of it is sometimes different,” Eric added. “Whereas Aria can recover from hers, someone else might not.”

Some children with congenital heart disease don’t even have to have their chests opened for surgery, said Randee. They’re in and out in a couple days.

Although ultrasounds can sometimes detect a heart defect, Randee said that’s not always the case, especially with Aria.

“They looked at it, and her heart must have looked OK,” she said, adding, “I don’t necessarily trust Aria, though. I don’t know that she’d show us.”

What’s unique about Aria’s case, said Randee and Eric, is that she rarely displayed symptoms of heart trouble.

“Through all of [the surgeries], you wouldn’t know it by looking at her, how she acted, that something was going on,” Eric said. 

“A baby who’s heart function was that low would probably be blue, probably be short of breath, sleeping all the time,” Randee remarked. “We checked her oxygen levels every day and they were always where they were supposed to be, and her heart rate always looked good.”

That, said Randee, just shows Aria’s strength, her heart warrior powers.

“She’s just so resilient. I think all heart babies are,” Randee reflected. “She smiles through some of the worst stuff.” 

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The Koenigs have been open about Aria’s journey, sharing updates on the Facebook page “Baby Aria - Our Heart Warrior.” Randee said it’s been a helpful asset to disseminate information to a lot of people. She and Eric are amazed at the amount of responses they’ve seen online and in person.

“The community—every day Eric would call me and say ‘so and so dropped something off.’ It’s just been amazing. It still is. People are still incredibly generous,” Randee said. “I think it’s brought a lot of awareness to something I knew nothing about. It’s allowed people to be aware of what congenital heart disease is.”

That’s especially poignant this month, which is congenital heart disease awareness month. It’s ironic, noted Randee, that Aria’s birth fell in the month, just a day after Valentine’s Day and a day after congenital heart disease awareness week.

So what theme did the family choose when they celebrated Aria’s first birthday two weeks ago? Hearts, of course. Nothing less for their strong heart warrior.