Team provides care for pregnant patients with congenital heart disease

Katie Presler and her infant daughter, who was born healthy with the help of congenital heart disease specialists

Katie Presler, BSN, RN, wasn’t worried about her third pregnancy. The heart defect she was born with was managed with medication, and her two previous pregnancies had proceeded normally. On top of that, as a nurse at the University of Chicago Medicine, she figured she knew what she could handle.

But because of her heart condition, her obstetrician referred her to maternal-fetal medicine specialist Joana Lopes Perdigao, MD, who works with cardiologist Michael Earing, MD, in UChicago Medicine’s multidisciplinary Adult Congenital Heart Disease and Pregnancy program.

As an infant, Presler was diagnosed with Shone’s complex, a rare congenital heart condition that resulted in mitral valve stenosis, bicuspid disorder and aortic stenosis — all affecting blood flow within her heart. She’d had two heart surgeries as a child and began taking beta blockers as an adult, but otherwise lived most days without thinking about the disease.

The doctors said, Don’t panic. We have a plan in place. We’re going to get you through this.

So, when Earing and Lopes Perdigao created a care plan and warned her of potential complications, Presler shrugged. “I said, ‘This is my third pregnancy, I’ll be fine,’” she said. But only 12 weeks into her pregnancy, she began to feel short of breath just from walking. An echocardiogram showed that the pressure gradient in her heart had worsened, reducing blood flow.

“The doctors said, ‘Don’t panic. We have a plan in place. We’re going to get you through this,’” Presler said.

A Pump with Twice the Amount of Work to Do

One out of every 100 babies is born with a heart defect. In the past, only about 20% survived into adulthood. But thanks to medical advances over the past 60 years, now 90% percent of infants born with a heart defect survive into adulthood. This means more and more adults with heart defects are becoming pregnant, which presents unique challenges.

“The heart goes through a variety of changes in pregnancy,” Lopes Perdigao said. “But the biggest one is that you have about 50% more blood volume than you initially did at the beginning of pregnancy, by the beginning of your third trimester. It's like a pump that has twice the amount of work to do, and that can be pretty stressful on the heart. Most women do totally fine with it, but there are some women who need a little bit more help and a little bit more attention.”

When a pregnant patient with a heart condition is first evaluated, the multidisciplinary team creates a care plan based on the patient’s specific heart defect. The plan takes into account risks, patient history and the patient’s own wishes. Physicians discuss the plan with the patient, then schedule regular meetings with the team as the pregnancy progresses.

Constant Communication about the Plan of Care

Presler’s condition worsened as the pregnancy progressed. She was put on a diuretic to reduce swelling, and she tried to take it easy, but she felt out of breath most of the time. At five months pregnant, she had to take a leave of absence from her job. Eventually, Earing told her that her pressure gradient had gotten so bad that she would need heart surgery after she had the baby.

But every time there was a bump in the road, Dr. Earing and Dr. Lopes Perdigao had a plan. They were in constant communication about my plan of care.

“That really grounded me,” said the 36-year-old Presler. “My first two pregnancies were so normal, and now I realized that my heart problem was serious. But every time there was a bump in the road, Dr. Earing and Dr. Lopes Perdigao had a plan. They were in constant communication about my plan of care.”

Because her condition had worsened, the plan included delivering her baby early. The physicians asked Presler if she would consider delivering at 35 weeks — five weeks before her due date. “They are very knowledgeable, and I felt like I was in good hands, so I didn’t think twice about it,” she said. “This is why you come to University of Chicago. You know this isn’t the first time they are treating something like this, and you know you’re going to get really good care.”

Her daughter was born healthy in April 2021. Presler was monitored in the ICU afterward and was prepared to undergo surgery to repair her heart. But one month later, an echocardiogram revealed her pressure gradient had improved, and she likely would not need surgery right away.

Presler’s story shows that while becoming pregnant with a heart condition can cause anxiety, there are plenty of resources to ensure the pregnancy goes smoothly, Lopes Perdigao said.

“We fully understand how scary it can be,” Lopes Perdigao said. “It's an unknown, right? Especially if it's your first pregnancy. Come and see us, even when you're thinking about potentially getting pregnant. We can talk you through it. We can create a plan for you, and we go on from there.”

Michael Earing, MD

Michael Earing, MD

Learn more about Dr. Earing