Beyond Adult Congenital Heart Disease: Emily’s Story

In the story of Emily’s heart, there are three key chapters. The first began before she was born.

When she was still in utero, Connecticut Children’s Heart Center detected a congenital heart defect: coarctation of the aorta. In other words, her heart’s main artery was too narrow, which was getting in the way of normal blood flow. Over time, that would put dangerous pressure on her heart.

However, as the team explained to Emily’s parents, they knew how to fix it.

Many families go through this process, leaning on a raft of Connecticut Children’s specialists for guidance before a baby is born, and after. When baby Emily was 9 months old, the cardiac surgery team performed the procedure they’d long planned: They cut out the narrowed part of her aorta, and sewed the two wider ends back together in its place, allowing blood to flow freely.

The procedure went exactly as promised. Emily began to thrive. And she kept right on thriving.

She did so well after surgery, in fact, that all through childhood, Emily thought of her annual visits to Connecticut Children’s mostly as a chance to visit her extended family. She lost both her parents when she was young — her dad when she was 8 years old; her mom when she was in her 20s — and in their own way, the doctors and nurses at Connecticut Children’s helped fill that space. She remembers telling her first cardiologist, the since-retired Dr. Leopold, about getting the training wheels off her bike. He celebrated like a proud uncle.

“Connecticut Children’s feels like family,” Emily says. “These are the people who have cared for me since before birth. They’re a big part of who I am.”
 

Then, in her mid-20s, Emily’s health took a scary turn. For years, she’d been in New York City and loving her life there. She lived in Brooklyn, and had enjoyed many strolls across the Brooklyn Bridge.

But by age 26, that changed. She felt exhausted all the time. Breathing was painful. She couldn’t walk the couple blocks to the Brooklyn Bridge entrance, never mind make it across.

She needed Connecticut Children’s.

Emily made the annual trip from Brooklyn to Hartford, and met the then-newest member of the cardiology team — now division head — Shailendra Upadhyay, MD, aka Dr. Shai.

Dr. Shai is, among many other things, an expert in the latest and greatest treatments for people living with heart conditions. He started Emily on a beta blocker, a medication that transformed her quality of life.

“I will never forget the first time I took that beta blocker. I was in tears of joy because I felt so much better. Taking a deep breath was easier than it had ever been in my life,” Emily says. “I could do so much more — I came back to my life in New York in a bigger, better way.”
 

When someone is born with a heart defect, they can have complicated health challenges as they age — challenges that would be impossible to solve without first understanding the unique way their heart is put together. Connecticut Children’s Adult Congenital Heart Disease (ACHD) center, the state’s first ACHD Accredited Comprehensive Care Center, specializes in just that. The center sees patients who they’ve known since childhood, like Emily, as well as adults who’ve only recently learned they were born with a heart abnormality.

A couple years ago, at age 33, Emily decided to write the next chapter of her heart story: She scheduled open heart surgery for a second, less urgent, congenital heart defect she’d been living with, known as a parachute mitral valve. All her life, the Heart Center team had been watching and waiting for the right moment to intervene. Finally, Dr. Shai said it was time.

Emily took a deep breath and agreed.

“Having that surgery was the scariest thing of my life. But I never even considered getting a second opinion, because I know I’m in such good hands with Connecticut Children’s,” Emily says. “Being with this same team, having that consistency my entire life, makes such a big difference.”

During the procedure, division head of cardiac surgery Dennis Mello, MD, replaced Emily’s mitral valve with a mechanical substitute. For the next few nights, as Emily recovered in the hospital, she was aware of the nurses going out of their way to take care not only of her as the patient, but her sister and three aunts too.

“It really stands out to me, how well taken care of I was by the nurses — and that they took such good care of my family. It was nice to know that my caretakers were being treated so well,” Emily says. She also had a surprise visit from her go-to Heart Center nurse, Jamie Bopp, RN, who stopped by on break with a teddy bear and balloon.

And of course, on the final day in the hospital, Valentine the clown made a celebrity appearance. Emily popped on a red nose of her own, and helped spread the joy.
 

A few months after surgery, Emily and her sister went for a walk at one of their favorite spots near Rocky Hill, a scenic path around a pond and waterfall. It was a beautiful summer day, sunny but not too hot. Before, Emily would’ve had to stop after one loop. This time, they kept going. She felt strong, and powerful, and full of possibilities once beyond imagination.

She’d conquered open heart surgery. She’d reclaimed her inner clown. She had a sudden, inexplicable urge to ride a rollercoaster — and shortly after, that’s exactly what she did.

“Guess what? I discovered I love going on rollercoasters!” Emily says. “I used to be very fearful. I never wanted to do anything that was intentionally scary, even if I knew it would be fun, because I wanted to protect my heart. Now I’m doing it all. I wake up and I have all this energy. I feel better than I’ve ever felt before.”

“My life has changed — again,” she says. “And it’s thanks to Connecticut Children’s.”