When Damian was born, he was more than a one in a million; he was one in 5.5 million. Now, at seven months old, he’s even more unique.
“He’s a miracle,” says his mother, Ashley.
Damian was born with the extremely rare congenital heart condition of ectopia cordis – Latin for “out of place heart.”
Lacking the typical coverage of a breastbone, about 20 percent of Damian’s heart, covered only by a thin layer of a visceral membrane, protrudes outside his chest. The survival rate of ectopia cordis is around 10 percent, with most instances of the condition resulting in a stillbirth; most newborns who initially survive die within hours or days.
Thankfully, with care from a collaboration of experts at the CHOC Heart Institute, neonatal intensive care unit (NICU), and the Fetal Care Center of Southern California, Damian is not only surviving — but growing.
Soon, around his first birthday, Damian is scheduled to undergo the first of several surgeries to correct his life-threatening condition, whose causes are unknown.
Maternal-fetal care gives hope, preparedness
Damian is Ashley’s first child.
Ashley was 9 weeks pregnant when she experienced heavy bleeding. She was whisked to an ER and stayed overnight. Doctors first told her they could detect no heartbeat in her fetus. Hours later, they said they heard one.
“I pretty much lost it,” Ashley says of the emotional roller-coaster.
At 19-weeks pregnant, Ashley was referred to Dr. Jennifer Jolley, a maternal-fetal specialist at the Fetal Care Center of Southern California, a partnership between UCI Health and CHOC that opened in June 2021.
She diagnosed the fetus’ condition as Pentalogy of Cantrell, a version of ectopia cordis that is a collection of five congenital birth anomalies of the diaphragm, abdominal wall, pericardium, heart, and sternum.
“Dr. Jolley gave me the hope that I was looking for,” Ashley says. “She said, ‘Don’t worry, we’re going to take care of you, this baby is going to come out perfect.’”
With no in-utero therapy available for ectopia cordis, the team had to stand by and wait for the baby to arrive. A team of CHOC neonatologists that specializes in high-risk births was prepared when Damian was born at 36 weeks via Cesarean section.
“He didn’t require resuscitation or intubation or intensive care,” Dr. Jolley says. “He started crying and did what newborn babies do. And it happened to be that his heart was beating outside of his chest.”
Ectopia cordis causes severe complications
A newborn’s heart is typically the size of a large strawberry. Damian’s was positioned unusually low in the upper abdominal wall.
“It almost looked like it was beating inside the umbilical cord,” says Dr. Amir Ashrafi, anexpert in neonatology and neonatal-cardiac physiology who is part of the team treating Damian.
“The path ahead has only been accomplished a couple of times that I know,” says pediatric cardiologist Dr. Wyman Lai of Damian’s medical journey thus far.
Not only is Damian’s heart partially outside of the body, Dr. Lai says, but his blood vessels are abnormally positioned, and a part of his heart hasn’t been developed.
Innovative heart protection
Drs. Ashrafi and Lai joined other CHOC physicians in deciding how to treat Damian after he was born.
Because his heart had little protection from injury, it was imperative to move it into its proper place.
Pediatric Surgeons Dr. Mustafa Kabeer and Dr. Peter Yu encouraged the team that since a thin membrane was covering the exposed portion of Damian’s heart, waiting for him to grow strong enough for surgery was the best course of action.
“Sometimes, doing nothing is a hard thing to get [physicians] to agree to do,” says Dr. Lai.
To protect Damian’s exposed heart, the CHOC team looked for innovative solutions. They fashioned a 3D-printed shell attached to Velcro straps that Damian could wear at home. He stayed in the NICU for four weeks and did well and was sent home without a surgical procedure – just the chest covering.
Growing stronger, preparing for future surgeries
In the coming months, the CHOC team will perform Damian’s first surgery, which will involve structural cardiac repair in addition to putting the heart back inside his chest. Several more surgeries will follow as he ages.
“I think everyone is thrilled that he has a chance to do well,” Dr. Jolley says.
Says Dr. Ashrafi: “He’s not out of the woods, but we have every reason to be optimistic that he will do fine in the short and long term.”
Ashley appreciates every doctor and nurse who is taking care of Damian. “Without them,” she says, “I wouldn’t have the strength I do to this every day. They’re all amazing.”
For now, it’s time for Ashley to enjoy her son as he grows bigger and stronger for the surgeries ahead.
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As a partnership between UCI Health and CHOC, The Fetal Care Center of Southern California brings together experts in maternal-fetal medicine and pediatrics, so both mom and baby are cared for—no matter the diagnosis.