LJ’s Story: Transposition of the Great Arteries

When their unborn baby was diagnosed with a congenital heart defect called transposition of the great arteries, Kristin and Joe turned to Jack Rychik, MD, director of CHOP’s Fetal Heart Program. Dr. Rychik and the program’s multidisciplinary team of experts gave them hope – and a treatment plan that would save their baby’s life. Now 6 months old, LJ is “the happiest baby I’ve ever met,” says his dad.


Transposition of the Great Arteries: LJ's Story

Kristin: The first time we heard that LJ had congenital heart defects, we were absolutely devastated. And the ride home, I don’t think Joe and I spoke one word to each other, we just wept.

Joe: We found out the news about our baby, it was the most difficult time for us.

Hello Dr. Rychik

Jack Rychik, MD: Hey Joe, hey Kristin, how are you?

Kristin: How are you Dr. Rychik? Great to see you.

Jack Rychik, MD: Hey LJ. How are you doing?

The first time I met the Spitales, Christine was, I think, about 21 weeks pregnant. After a fetal echocardiogram, we were able to identify that in fact LJ had something called transposition of the great arteries. Normally the right side of the heart, which receives the low-oxygenated blood, gives rise to a vessel called the pulmonary artery, that then delivers blood to the lung. Blood picks up oxygen from the lung, returns to the left side of the heart and then is ejected into a vessel called the aorta that delivers that oxygenated blood to the body.

In transposition of the great arteries, those two vessels are switched. Such babies can get very, very sick very quickly.

Kristin: As you can imagine, it was extremely difficult for us to digest this news.

Jack Rychik, MD: With the arterial switch operation, we can now essentially turn the heart back into something that’s almost identical to a normal heart.

Kristin: When we visited Children’s Hospital and we first met Dr. Rychik, it was the first bit of comforting news that we had received. Learning that we were candidates for the SDU, we relocated to the area.

Jack Rychik, MD: The Special Delivery Unit is a critical aspect of the overall management for our patients who have important congenital heart disease and need to have management as soon as they’re born.

Kristin: I had gone into labor sooner than expected, and we delivered at the SDU. They stabilized him with the breathing apparatus, and once I was in recovery, they let me know that he would in fact need a balloon procedure.

They scheduled his surgery for two days after he was born. That was a tough day, knowing that not only couldn’t we see him for five hours, but just the idea of such a small, little being being opened up like that was very difficult.

Joe: I’ve been in a lot of difficult situations with my job as a detective, but this was the most difficult time ever.

Kristin: It’s one of the most memorable days because after nine months of carrying a baby that I knew would have some challenges, it was so emotional.

The surgeon came into the room and told us that the surgery went very well.

Joe: Dr. Mascio, when he told me that it was a perfect surgery, it was just so much relief.

Kristin: We follow up with our local cardiologist. She’s extremely pleased with everything that she’s seen and she does not see any issues for the future.

Jack Rychik, MD: Typically we see kids once a year, young adults once a year perhaps, or something like that, for general checkups. But when you’re not at your doctor's office, these kids are out doing normal stuff, leading completely normal lives.

Joe: This is Louis James. He was born at The Children's Hospital of Philadelphia on August 17, 2014.

Kristin:  And he’s very, very happy.

Jack Rychik, MD: And currently hungry.

Kristin: Very hungry.

Jack Rychik, MD: Every heart has a story, and this is LJ’s story.

Topics Covered: Transposition of the Great Arteries

Related Centers and Programs: Cardiac Center, Fetal Heart Program