Katharine Babst Ebstein’s Anomaly

The following message was posted by Katharine's mother, Amanda Babst.

Mother and Daughter Share Special Bond

Life experience has a way of shaping us all in unique ways. For me, one of those experiences involves my heart. I was diagnosed at about a year old with an atrial septal defect (ASD), a congenital heart defect. In layman’s terms, I was born with a hole in my heart. Doctors consistently monitored my heart when I was a kid with no big change, but with the knowledge (and a little anxiety) I knew I might need to have surgery to repair my heart. Over time, it looked like it was getting smaller and my doctors just wanted to monitor and wait to see how it was progressing. However, the ASD started to get larger during my teenage years. I had it repaired at age 18, two days after my high school graduation. Fortunately, I managed to recover successfully from the repair and have moved on to live a very active life. Beyond the repair of my heart defect, I’d like to think that the experience blessed me with an inner strength that I’d need to rely on very heavily in future years.

When my husband, Dave, and I found out we were pregnant for the first time, we were ecstatic, as most new parents tend to be. As a precautionary measure, I had to go through level two testing and receive a fetal echocardiogram. Throughout the pregnancy, we went to regular visits with the cardiologist and had a number of echocardiograms. At the end, we were having one or two a week. The diagnosis kept changing, but by the end right before our baby was born, we knew that it looked a lot like Ebstein’s anomaly, a congenital heart defect of one of the heart’s valves, and they had picked up some supraventricular tachycardia (SVT) as well, which is a faster than normal heartbeat. Our growing baby also had a moderate-sized ASD. We knew that we would need help when she was born. The doctors told us she likely will be sick, likely will not be able to breathe on her own, and likely will need to have heart surgery before she’s 10 days old.

Katharine Pauline Babst was born on July 9, 2010. She was delivered at a Chicago hospital, and was taken within a couple of hours to the children’s hospital, which was at a different campus at that point in time. She was a miracle because they got her off the ventilator, she was breathing on her own and she wasn’t blue. We were able to take her home after two weeks without having surgery, and she was monitored from there in Chicago.

We had learned that José da Silva, MD had developed a procedure for repair of Ebstein’s anomaly, which is now the standard of care around the world. This surgical technique, known as the cone procedure, reconstructs the tricuspid valve and the right ventricle. A doctor who had trained under Dr. da Silva was performing these procedures in Minnesota. Katharine’s file was sent to his team and our thinking was that, at the right time, we would look to have Katharine’s procedure performed there. The team in Minnesota reviewed her file and said she was absolutely a candidate for the cone procedure, but that we didn’t need to do it right now. They wanted to see how much bigger she could get because she was doing so well.

Then we moved to Pittsburgh. The first phone call I made was not to a realtor. It was to UPMC Children’s Hospital of Pittsburgh. I got three names from Katharine’s cardiologists, and one of them was the executive director of the Heart Institute at UPMC Children’s Hospital. I called him and he answered his own phone. My husband is a Pittsburgh native and constantly reminds me about how Pittsburgh is such a special place. But how many doctors answer their own phone? He talked to me for about 20 minutes about Katharine and her history. There were multiple doctors that could be a good fit for her complex case, but the executive director at Children's said he would personally love to take on her “case.”

He has been amazing. We were starting to talk about surgical repair, and Victor Morell, MD, chief, Division of Pediatric Cardiothoracic Surgery was actually reaching out to Dr. da Silva’s trainee in Minnesota to see if we could get him to come to UPMC Children’s to perform the procedure. In 2016, we went in for another checkup with our doctor, and he said let’s hold the presses because Dr. da Silva is coming to Pittsburgh in a few months to join the surgical team. From a life-long Pittsburgh sports fan’s view point, Dave said that we just found out that the Mario Lemieux of his field was being traded to our team. This just doesn’t happen!

Dr. da Silva repaired Katharine’s heart on June 13, 2017. He did a beautiful job, and we had a phenomenal experience at UPMC Children’s. The doctors, nurses, and hospital staff — not to mention the building itself — are such an incredible blessing for us all. This kind of experience also makes you appreciate your family, your friends, your employers and co-workers, and your community. So many people were incredibly supportive of us during this period of time. Dave and I also saw lots of children and families that were taking on incredible challenges and fighting against the odds with hope and amazing doctors.

As for Katharine, she’s progressing very well. Her recovery from the surgery was remarkable. I couldn’t get out of bed by myself for the first two weeks I was home from the hospital after my surgery. The recovery period was so much shorter for Katharine, and her surgery was so much more complex. One of the best moments for our family was when Katharine walked herself into our house a week after her surgery. Katharine’s younger siblings, Ryan and Maggie, welcomed her home and were so proud of their big sister and so genuinely happy to see her. Today, you can see such a difference in her already, in her energy and confidence level. She’ll continue to be monitored, but at this point she’s doing great.

Katharine and I share a very special bond. She looks a lot like me, and we’ve gone through this experience together. I know I can really relate to her. There’s a level of anxiety and fear that comes with having a sick child, and you worry about your child coping and about how scared they might be. It was helpful that I really knew what she was feeling because I’ve been through it — and we have matching scars to prove it.

Katharine’s most defining qualities are that she is an incredibly sweet-natured, caring, and brave girl. She has a BIG heart. Thank you, UPMC Children’s, for helping to ensure that we can see it support her throughout her life.

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