Child Born with ALCAPA Thriving Today After Open-Heart Surgery
When Gracelin was just 2 months old, her mom, Candice, noticed how she was breathing while nursing. Gracelin turned slightly blue around the mouth, and to Candice, it just didn’t feel right. She called 911.
Candice’s maternal instinct was absolutely correct. As soon as the pediatric cardiology team saw the chest X-ray, they knew that Gracelin had a congenital heart defect. “It was the classic huge heart for a two-month-old,” James Gangemi, MD, says.
Gracelin had ALCAPA, a rare condition where the left coronary artery comes off the pulmonary artery rather than the aorta. If left untreated, it leads to heart failure.
Gracelin’s ALCAPA Story
Gracelin’s story, from newborn needing open-heart surgery to thriving 3 year old is possible thanks to quick medical care and her loving family.
CANDICE MARSHALL I'm Candice Marshall.
HUNTER MARSHALL I'm Hunter Marshall.
CANDICE MARSHALL And we are born and raised from Charlottesville, Virginia. We met each other in high school. We went to Monticello together and actually used to eat lunch together every day.
HUNTER MARSHALL Hard to believe it's been almost 15 years.
CANDICE MARSHALL Gracelin was born January 19th, 2022. I was actually going to my post- delivery appointment. I was like, well, you know, I’ll nurse her before I actually go to my appointment. And at that time, she had a slight breathing episode that just didn't feel right, but it may have lasted like 15 seconds.
And then she came right back. But I did notice that around her mouth, she was a little blue around her mouth and so I instantly called 911.
JAMES GANGEMI, MD They brought the baby in and upon chest x-ray, it was the classic, you know, huge heart on a chest x-ray for a two-month-old.
PETER DEAN, MD Now, ALCAPA is when the left coronary artery, instead of coming off the aorta, it comes off the pulmonary artery. So that causes the heart to be weak. If not diagnosed, the heart will continue to fail. It is an emergency.
JAMES GANGEMI, MD To repair this, this is a very technically precise operation.
CANDICE MARSHALL When Dr. Gangemi came to deliver us the news that she was going to have to have open heart surgery, I remember him particularly looking at us and saying, let her be my daughter tomorrow. I will take the utmost care of her. And I don’t think I'll ever forget a surgeon telling you that.
JAMES GANGEMI, MD Immediately after surgery, we go up to the dedicated pediatric cardiac intensive care unit. PETER DEAN, MD There was a kind of the immediate recovery of, she needed to get her heart working better. We needed to support her. We needed to heal from where the surgery was.
CANDICE MARSHALL The nurses in the PICU. Amazing.
HUNTER MARSHALL Yeah, the entire team was phenomenal. They didn't leave her side for 24 hours a day.
PETER DEAN, MD Yeah. We will always watch it. But her heart's really been working really well. And her heart muscle has normalized.
CANDICE MARSHALL We walk through the doors of cardiology, and I think they feel like family. Dr. Dean and Gracelin have a special relationship, and oftentimes it doesn't even feel like a doctor's appointment; it really feels more like a catch-up session. So we truly love Dr. Dean and the whole cardiology team.
PETER DEAN, MD She epitomizes the whole of why I love what I do. I love being able to care for these patients and their families. I feel very honored. I love being part of her team. HUNTER MARSHALL They've succeeded in the surgery. Everything that they've told us has been true to what they say. It was tough, but we've made it through there.
CANDICE MARSHALL I would just say trust the process and I trust UVA with everything.
Are Blue Lips Always an Emergency?
Blue lips can be an urgent sign; they can also be caused by gas, the effort of eating (called perioral cyanosis), or being a little cold. Because babies’ veins are close to the surface and quite fragile, they’re often visible and can create a blue hue.
Likewise, newborns often experience short periods of apnea, or ‘not breathing.’
Together, though, these symptoms can mean something much more serious. Candice’s quick reaction helped get Gracelin the care she needed quickly.
Are Many CHDs Not Caught Right Away?
Before the Newborn Screening guidelines, there were quite a few CHDs that weren’t caught until later. And still, there are many that can’t be caught until several days or weeks after birth. A baby’s heart changes over the first few weeks in ways that can expose an undetected CHD.
Attending your newborn’s well-child visits can help, and this is why there are so many when your child is a newborn. But parents, who spend the most time with their child, are often the first to know that something isn’t quite right.
Rarely, some congenital heart defects aren’t caught until adulthood. Anyone who has shortness of breath coupled with blue tinting to their skin or lips should talk about these symptoms with their doctor. Many congenital heart defects can be fixed with medicine or minimally invasive heart procedures.
Open-Heart Surgery
In Gracelin’s case, open-heart surgery was needed. At UVA Health Children’s, she was able to recover in a cardiac-specific PICU. Monitors, coupled with a dedicated crew of hospitalists and peds cardio-specific care team members, help to ensure that babies recovering from open-heart surgery have the support they need to recover.
Fortunately, Gracelin already had the best support: attentive parents who caught her symptoms early, got help, and who continue to facilitate her recovery. Gracelin continues to follow up with Peter Dean, MD, who enjoys getting to watch her grow up and catching up with her in visits.
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