Baby’s rare heart defect surgery inspires Grey’s Anatomy

Nannette McDonagh loves to watch the medical television drama, Grey’s Anatomy, but she never imagined that her family would be the subject of one of the show’s recent episodes.

The producers of Grey’s Anatomy recently learned about Nannette and the miraculous recovery of her son, Ian, after finding an old magazine account on the Internet. They marveled at the unique, dramatic way Ian lived through a life-threatening birth defect, treated at Advocate Children’s Hospital—Oak Lawn.

Discovering congenital heart defects
When McDonagh was 20 weeks pregnant, her obstetrician noticed that the ultrasound showed Ian’s heart was not developing normally. A heart specialist diagnosed a rare combination of birth defects in Ian’s heart.

One was hypoplastic left heart syndrome (HLHS). In unborn babies with HLHS, the left side of the heart does not develop properly, which means it does not do a good job pumping oxygen-rich blood to the rest of the body. Even though only about one out of every 4,344 babies are born each year with HLHS, it is 100 percent fatal without treatment, says the U.S. Centers for Disease Control and Prevention.

The other condition Ian had was an intact atrial septum. In the womb, babies are supposed to have a small opening in the atrial septum, which is the wall that separates the heart’s left atrium and the right atrium.

This opening allows blood to travel from the lungs to the left atrium. Then, it mixes with blood in the right atrium before being pumped to the rest of the body. But Ian’s atrial septum was intact — it didn’t have an opening. So, blood coming from the lungs had nowhere to go, potentially causing pressure in Ian’s left atrium and pulmonary veins.

Infants with this type of birth defect in the womb have a grim chance of survival.

Going into labor in traffic
McDonagh’s prenatal cardiologist immediately referred her and her husband Pat to another hospital out of state for an experimental procedure to open the atrial septum while the baby was still in the womb.

“We were open to trying and doing anything,” Pat McDonagh said. “[The other hospital] started the procedure on my son’s ‘grape-sized’ heart. Unfortunately, they had to abandon the effort because my child was moving around too much in the womb.”

The McDonaghs returned to Chicago and were referred to Dr. Alexander Javois, a pediatric interventional cardiologist at Advocate Children’s Hospital—Oak Lawn.

At 24 months, Nannette’s water broke — while they were stuck in traffic on a highway.

“Here we were stuck in traffic on a huge busy highway — of all places to be,” Pat recalled. “I came face-to-face with my biggest fear — I may have to deliver our baby myself! A child who’s already been given very low changes of even surviving the delivery.”

Lifesaving surgery — in one hour
In November 2003, Nannette gave birth in Advocate Children’s Hospital’s heart catheterization laboratory. A team of physicians, nurses and technicians, led by Dr. Javois, were standing by ready to work immediately on reversing Ian’s heart condition.

Without it, Ian had 0 percent chance of survival, Dr. Javois said.

Dr. Javois and his team had to work fast to prevent the obstructed blood flow from causing life-threatening damage to Ian’s brain, lungs and heart. They performed an atrial septostomy. The procedure involved feeding a searing wire through Ian’s umbilical cord vein leading to his heart.

They then used the searing wire to burn a hole in the wall between his heart’s two upper chambers.  The hole allowed red blood to flow from the heart to the rest of Ian’s body.  At the time, the handful of previous attempts at other institutions in the country to perform a similar procedure had taken several hours to complete. Dr. Javois’s team performed it successfully in less than an hour.

Several weeks after this initial procedure, baby Ian underwent the first of eight surgeries to correct his HLHS. Internationally-noted pediatric heart surgeon, Dr. Michel Ilbawi, led the surgical team.

Baby Ian was allowed to go home less than two months later on Dec. 23, 2003 – a Christmas gift that his parents, Patrick and Nannette, called the “most precious and priceless ever.”

When Grey’s Anatomy called
“This was truly a miraculous outcome,” said Dr. Javois when baby Ian was discharged from the hospital. “We were amazed and pleased with how rapidly Ian responded. With so many odds working against him, little Ian never gave up. He is definitely a fighter.”

Dr. Javois and the McDonaghs were shocked when Grey’s Anatomy producers called to recap the procedure 10 years later. Few like it have been performed since, Dr. Javois wrote to the medical researcher for Grey’s Anatomy. The episode aired April 25.