Transposition of the Great Arteries: Ian's Story

Ian spent the first two weeks of his life surrounded by a team: his parents and the doctors and nurses of the Cardiac Center at The Children's Hospital of Philadelphia.

Born with a complex heart defect called transposition of the great arteries (TGA), Ian was taken by helicopter to CHOP a few hours after his birth.

ian There, Jonathan J. Rome, MD, director of the Cardiac Catheterization Lab, performed an emergency catheterization technique called balloon atrial septostomy, also known as the Rashkind procedure. The Rashkind procedure was first developed at The Children's Hospital of Philadelphia by William J. Rashkind, MD in the 1960s.

Ian's father, Ron, drove to CHOP to be with his baby. But Ian's mother, Christine, having just given birth, wasn't able to get to CHOP immediately. If Ian were born today, he may have been delivered in CHOP’s Garbose Family Special Delivery Unit (SDU), a state-of-the-art labor and delivery unit opened in 2008 for mothers carrying babies with known birth defects. The unit is on the same floor as the cardiac operating rooms, catheterization labs and patient units, so parents and baby stay close rather than being separated at birth.

Best clinical care, good communication

The night Ian arrived at CHOP, a CHOP cardiologist called Christine at the maternity ward and explained the procedure her baby would need. Once the catheterization procedure  was concluded, he called to tell her Ian was stable. Christine and Ron found this standard — the best clinical care, combined with thoughtfulness and good communication — throughout Ian's two-week stay in the Hospital and in the 15 years since as he has visited as an outpatient.

"I would never have gotten through this experience without the people at CHOP," Christine says.

They helped me in every aspect, from teaching me to take care of him to helping me learn how to nurse him. It's just an amazing, amazing group of people.

The Cardiac Center team, comprised of pediatric cardiac nurses, cardiologists, surgeons, social workers, therapists and much more, are recognized for setting the national standard for excellence in the specialized care of infants, children and adults with congenital heart disease. We understand that exceptional medical care is only one piece of the picture, and that truly comprehensive care for patients means also providing families with the resources and support they need, every step of the way.

Surgery to treat transposition of the great arteries

Ian spent several days in the Evelyn and Daniel M. Tabas Cardiac Intensive Care Unit (CICU).

Thomas Spray, MD, chief of the Division of Cardiothoracic Surgery at CHOP, performed open heart surgery to treat Ian's TGA. The wait during surgery "seemed like forever," Christine recalls. But their anxiety was alleviated by the cardiac team's efforts to explain the surgery and put them at ease, as much as possible.

The team explained the procedure numerous times in the days leading up to surgery.

"Our son's heart was the size of a plum and the vessels that were reversed were the size of lead in a pencil," Christine says. "Everything is extremely intricate and extremely tiny."

Christine and Ron met with Dr. Spray before the procedure. Anesthesiologist Susan Nicolson, MD, came to Ian's bed and accompanied him to the operating room.

"You know almost everyone who is with your child," Christine says. "He's not just going into unfamiliar territory. Everybody had a specialty but they all worked together."

Soon after the procedure, a cardiac nurse brought them wonderful news: "We got word that he was doing great. That's all we really needed to hear."

Difficult experience, happy ending

One of the most difficult experiences was to see Ian after the surgery. His incision was covered in bandages and ran nearly the length of his chest and he was heavily sedated.

"That was the scariest part," Christine recalls. "At first we couldn't even hold him. He was hooked up to so many things."

But he improved quickly.

"At some point we were able to hold him," she says. "We needed to hold him as much as he needed to be held."

Christine, who had met with one of the Hospital's lactation consultants and had been using a breast pump, was also able to breastfeed him for the first time.

Caring and skilled nurses

During this difficult time, Christine remembers the Cardiac Center nurses as incredibly helpful. Christine and Ron stayed with Ian as much as possible, but spent some nights at home. They would call Ian's nurse before they went to bed, to make sure he was OK.

The nurses played music for Ian on a radio next to his bed. One morning Christine and Ron walked in to find him wearing a soft new hat, a gift from one of the nurses.

"The nurses were spectacular," Christine says. "Over-the-top nursing. I've never met so many caring and skilled people in my life. That really helps to get you through."

The more than 250 specially trained pediatric cardiac nurses at CHOP are passionate about their work and are also highly invested in their role as patient and family educators.

Ian spent two weeks in the Hospital. Born Dec. 2, he finally came home Dec. 17, "making him the best Christmas gift that we will ever receive," his mom says.

Follow-up care for TGA

Ian's recovery continued and he had numerous checkups at CHOP in the first year.

Today, Ian is 15 and in ninth grade. He has a checkup with his cardiologist every two years, including an electrocardiogram and an echocardiogram.

Children born with TGA can experience leaky heart valves and other cardiac problems, so Ian will continue to see a cardiologist for many years. His primary care pediatrician is also well-versed in Ian's condition and knows what to look for during regular checkups.

A brighter future

So far, there have been no problems. Ian is an excellent student and hasn't experienced developmental delays, as some cardiac patients do. He is very athletic; his sports include baseball, swimming, soccer, tennis, basketball and golf.

Christine and Ron went on to have two more children, both born healthy. While Christine remembers the fear and stress of Ian's first days, she also remembers the good: the Cardiac Center nurses and doctors and the support and care they provided.

"We're so lucky that CHOP is basically around the corner," Christine says. "The care was just first-rate — extremely caring, sympathetic, skilled, intelligent and dedicated people. I couldn't have asked for anybody better to leave my baby with."

Originally posted: June 2009
Updated: January 2013

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