Thanks to advanced surgical techniques, more babies born with congenital heart disease within the last few decades have survived to adulthood. Now, as they reach childbearing years, they’re planning to have babies of their own. They represent a new generation of individuals managing congenital heart disease and pregnancy, and many require specialized care.
“Typically, about 1 of 100 babies will have some form of congenital heart disease. In the past, only 10% to 20% would survive without surgery. Now 90% survive to adulthood, including a large group of women now in their childbearing years,” says Michael Earing, MD, medical director of the Chicago Adult Congenital Heart Disease Alliance with The University of Chicago Medicine.
To support and care for these individuals as they plan for and progress through their pregnancies, Dr. Earing started the ACHD in Pregnancy Program, a highly specialized, multidisciplinary care team at UChicago Medicine. Dr. Earing is one of just a handful of board-certified ACHD specialists in Chicago.
“For our patients, we provide an individualized approach to their care with specialists in genetics, maternal fetal medicine, cardiology, electrophysiology and anesthesiology for the best outcomes for mothers and babies,” he says. “A lot of women have been given misinformation about their ability to become pregnant and to deliver safely. This creates increased stress and often may not be warranted. Together we work with each individual to help make a detailed plan for how to have a safe mom and baby.”
Specialized care for congenital heart disease and pregnancy
The ACHD in Pregnancy Program supports individuals with a wide variety of conditions, including ventricular septal defects, atrial septal defects and peripartum cardiomyopathy and the heart problems that can accompany conditions such as Marfan syndrome and Ehlers-Danlos syndrome.
Some women even discover for the first time that they have ACHD when they become pregnant, says maternal fetal medicine (MFM) specialist Joana Lopes Perdigao, MD. Beyond the standard OB-GYN residency, MFM specialists attain three additional years of training in the unique physiology of women in pregnancy, which makes Dr. Perdigao’s expertise critical for the program.
Overwhelmingly, it’s ideal for those with ACHD to consult with the ACHD in Pregnancy Program specialists prior to conception. “For those who have been followed by a cardiologist and have had surgeries, we recommend meeting with MFM specialists and cardiologists who can see them through their pregnancy,” says Dr. Perdigao. “We can talk about what a pregnancy could look like and any associated risks.”
Each heart is different
An individualized approach helps each woman approach pregnancy for the best possible outcome. Patients who have had previous interventions as children are at increased risk for multiple different late complications.
“In general most children do well, but because of their unique surgeries, they often can have problems later in life such as leaky or tight heart valves, high blood pressure, high pressure in their lungs, abnormal heart rhythms, and even heart failure,” explains Dr. Earing.
“During pregnancy, there is 50% increase in the amount of work the heart has to do. These changes can stress the heart even further during pregnancy and can exacerbate many of these complications, increasing the risk for both the mother and the unborn baby. This is why it is crucial to always try and have these conversations prior to becoming pregnant,” Dr. Earing says
Getting ready for birth
As women contemplate their birth experiences, they’ll consult with the specialists to determine where they can most safely deliver their babies — and how.
“In some cases, it is appropriate for women to deliver with their own OB close to home. Not everyone will need to deliver at a tertiary care center like UChicago Medicine, says Dr. Perdigao. “We can give patients our recommendation as early as possible so they can plan accordingly.”
As they get closer to term, the ACHD in Pregnancy Program team monitors patients closely to make sure they are physically prepared for birth. “Many women can have a higher risk of arrhythmia as they near term and during their labor and postpartum course. At UChicago Medicine, we have the expertise to follow them closely in their labor and postpartum course and make sure we are all doing all we can to keep them healthy,” she explains.
Where women with ACHD have typically been encouraged to have a cesarean section, Dr. Perdigao says that’s not always necessary or advisable. “Women have been told they need a C-section based on the fact that they have a heart problem, and in some cases we do recommend that, but those cases are rare,” she says. “Most women can attempt a vaginal birth. We detail a plan to keep them healthy throughout their peripartum course.”
Healthy baby, too
Babies born to individuals with ACHD have an increased risk of having heart problems of their own, so the specialists provide extra attention to the growing baby. “The risk for baby is 5 to 7 in 100 births compared to the normal 1 in 100,” says Dr. Earing, adding that the dual expertise of a pediatric cardiologist who is board certified in ACHD brings specialist knowledge for best outcomes. “We take a look at 18 to 22 weeks to make sure baby is OK so that mom and dad have no surprises, and we make sure that when baby is born they get the right care.”
Throughout the experience, families are able to build a strong relationship with the specialists in the ACHD in Pregnancy Program — an aspect of individualized care that Dr. Perdigao says she enjoys most.
“We have had excellent results over the last year since we started, and we have seen quite a few women through deliveries that were safe and both moms and babies are healthy now,” she says. “But my favorite part is developing relationships with these mothers and ensuring they have a healthy delivery after 10 months of intense monitoring. It’s definitely one of my favorite parts of the job.”
One of just 50 programs nationally, the UChicago Medicine ACHD in Pregnancy Program is working toward becoming the only accredited program of its kind in the city of Chicago. By recognizing that each patient is unique — with a unique medical history and surgical background — the specialists on the team can provide individualized care during an important time in their patients’ lives.
“Most women will do great with pregnancy. But there are some at higher risk, so we counsel them before they are pregnant, but if they are already pregnant, we work to have a healthy mom and baby,” Dr. Earing says. “The best chance for a healthy outcome for mom and baby is in a specialty center like the UChicago Medicine ACHD in Pregnancy Program.”