Johns Hopkins All Children's Foundation

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Mending Luca’s Heart


Posted on Feb 08, 2022

Mayuly and Daniel with their twins Luca and Sebastian at Johns Hopkins All Children's Hospital.
Mayuly and Daniel with their twins Luca and Sebastian at Johns Hopkins All Children's Hospital.

Sleep.

Shut eye.

There’s precious little of it when you’re parenting a newborn baby …

But twins? They have a way of raising your game.

Parents Mayuly and Daniel are all in — up every three hours for feedings, changing countless diapers and tending to all the needs of their beautiful boys, Luca and Sebastian. Doting big sister Bella helps too.

The family considers it all their very good fortune.

“When the doctor told us we were having twins, we were like, ‘What? That’s impossible!’” Mayuly says. “We are just blessed.”

But the boys’ entrance into the world would not come easily.

During Mayuly’s 20-week scan, she and Daniel learned something about one of the babies she was carrying that would change everything.

One twin, Luca, had life-threatening complex heart disease.

“We cried and cried,” Mayuly said. “In the moment, you just can’t process the news.”

Mayuly and her unborn babies required a high level of specialized cardiac care. Their Tampa doctor referred the family to Johns Hopkins All Children’s Heart Institute in St. Petersburg, Florida.

The hospital had rebuilt its Heart Institute over the past 18 months, acquiring talented pediatric heart surgeons and esteemed cardiologists, many with decades of experience.

Cardiologist and Heart Institute Co-director Michael Puchalski, M.D., met with the couple and confirmed Luca’s diagnosis: dextro-transposition of the great arteries (d-TGA) with ventricular septal defect (VSD).  

For a parent, it’s an intimidating diagnosis. But Puchalski wasted no time helping Mayuly and Daniel to understand their unborn baby’s condition and showing them a path forward to saving their child.

“Normally, the pulmonary artery comes off the right ventricle and the aorta off the left ventricle,” Puchalski says. “In transposition, the aorta comes off the right ventricle and the pulmonary comes off the left.”

When this occurs, the body has no efficient way to get oxygenated blood to the tissues. The result can be lethal.

VSD can complicate a situation further. With VSD, there is an abnormal opening in the wall between the main pumping chambers of the heart.

Luca had major heart surgery ahead of him.

The family would quickly learn they had come to the right place.

Team with Heart

Before the twins’ birth, the surgical team met with Mayuly and Daniel to give them a deep understanding what Luca would need and how things would unfold — before, during and after the surgery.

They even explained what each piece of medical equipment looked like and what it was for, so that the parents would not be frightened of the machinery that would help sustain their child.

“We asked lots of questions, and we had such a comfort level with the entire surgical team,” Daniel says.

On Dec. 28, Mayuly gave birth by way of C-section to Luca and Sebastian. 

She knew Luca would be whisked away for care in the cardiovascular intensive care unit (CVICU).

But first, a special moment to hold both of her newborn twins — even delicate Luca — in her arms.

For that moment, time stopped.

“Holding both of my babies — that was one of the best moments of my life,” Mayuly says.

The Mend

Babies with this diagnosis generally need to have a surgical repair within the first week of life.

On day two, Luca is transported into the operating room.

The cardiac surgery team is fully in motion — surgeons, anesthetists, perfusionists, scrub nurses and others working fluidly together for one goal — the best possible outcome for Luca.

“Communication and precision are essential,” primary surgeon Awais Ashfaq, MBBS, says. “We have a tiny patient who has a heart not much bigger than a walnut.”

To repair the switched arteries, Ashfaq works confidently with the team to first remove the coronary arteries and then transect the aorta and the pulmonary artery to make the arterial “switch.”

The team deftly executes the related reconstruction and repairs to baby Luca’s heart, including closing the VSD first identified by fetal echo.

Luca’s surgery goes smoothly — but what happens next is one of the things that may set this cardiac team apart from some others.

Breathing Room

With exceptional skill and monitoring, the team can often create conditions that allow a heart patient to come out of the operating room breathing on his or her own, without a ventilator.

“By doing this, we can really help expedite recovery in the CVICU,” Ashfaq says.

“This means we can minimize pain medications and get them moving more quickly and eating on their own.”

It’s not something every heart surgical team would have the expertise or the inclination to do. But it can make a positive difference in a child’s recovery.

That’s what the team was able to do for Luca.

 ***

With each passing day after Luca’s surgery, there was another little victory. 

Each day, another piece of medical machinery would be removed because he no longer needed it.

Each day, he ate a little more.

Luca’s dad looked forward to the evenings when Luca would be placed on the scale, and he could see how his boy was gaining weight and getting stronger.

Daniel had time during his child’s recovery to watch the CVICU staff closely, and he was gratified by what he saw and experienced.

“You never want your kids to have heart surgery, right?” Daniel says. “But we were so lucky to have Luca in the CVICU at Johns Hopkins All Children’s. They were wonderful. I don’t even have the words to thank them.”

Going Home

Ten days after his surgery, Luca is doing beautifully — and ready to be discharged.

He’ll get regular follow-up cardiac care in the years to come, and doctors will keep an eye on a small residual VSD to see if it closes on its own.

His outlook is excellent.

“Luca should do very well and have a long life, as strong and vibrant as his twin brother,” cardiologist Puchalski says.

His parents delight in the thought as they gather the twins in their arms to take them home.

“We’re from Cuba and family is everything in our culture,” Daniel says.

“I want Luca and Sebastian to grow to be great men — to love family and to help other people, just like so many people here have helped them.”