Women who were born with certain congenital heart defects, even if they have been repaired, have an increased risk of complications during pregnancy for both themselves and the baby. But with proper monitoring by both a cardiologist and a high-risk pregnancy specialist, many women who were once told they shouldn't have children do all right.
"Most women, with close management, can be successfully pregnant," says cardiologist Dr. Kevin Whitehead of University Hospital. "In general, the conditions that are highest risk are something that need to be evaluated before pregnancy, but it's not common to have to recommend not being pregnant."
The first part of pregnancy is not much different for women with a history of congenital heart problems. But by the third trimester, the body undergoes big changes and stresses, even in women with no such history. Most women who are pregnant find their heart rhythms change, they retain fluid and they feel short of breath. The volume of blood the heart must pump nearly doubles. And the risk for problems, some very dangerous, increases.
In fact, say Whitehead and colleague Dr. Mark Brann, medical director of the Adult Congenital Heart Clinic at the University of Utah, some of the women born with heart problems develop severe high blood pressure in the lungs, which can be associated with 50 percent mortality for the mother and 80 percent for the baby, for example. For other complications, risk may be only moderate but bears watching.
About 1 in 125 babies is born with a heart abnormality. Many of them have heart repairs, and as they age they figure they're just fine. They may not know that complications can rear years later during pregnancy, threatening mom, baby or both. That's why heart specialists recommend that those women see a heart specialist, preferably before becoming pregnant.
Alyssa (who asked that her last name not be used) was born 29 years ago with a transposition of the great vessels, meaning that her pulmonary arteries and her aorta were essentially switched. Her deoxygenated blood was returning from her body to her heart, then going back into her body again. She had a small hole in the lower part of her heart, so that blood with oxygen mixed with the deoxygenated blood, providing her blood with some oxygen, but not enough. When she was six months old, surgeons operated.
It wasn't until shortly after she married that her cardiologist told her any pregnancy might be high-risk for complications. He divided his patients into three groups: "Go ahead, you're fine." "If you want to have children, we'll have to watch you closely." And "No way." Alyssa fell into the middle group.
"I'd never thought about what my plans (to have children) were," she says. "I'd been raised with my mom's mind-set when she took me home that I was a child, not a china doll. I wasn't a sick kid, and I was raised like any other child, so I never thought about it, although I had more doctor appointments. I had no idea I'd have to be careful with pregnancy."
Children born to women who had congenital heart defects are also slightly more likely to have their own heart defects, although not necessarily the same ones. If the congenital heart defect is part of a chromosomal syndrome (and it's usually not), the chance a child will have a defect increases even more.
Melissa Spaulding, 26, was born with mitral valve regurgitation. She had her first open-heart surgery at age 5. As a teen, her mom told her if she got pregnant, she'd need extra monitoring — information that was crucial when she was carrying Devan, now 3.
She had an echocardiogram during the pregnancy, along with other tests. She regularly made the trip from Riverton, Wyo., to the U. to see her high-risk obstetrician and her cardiologist, and she didn't have any problems. But partway through her second pregnancy — Trevor is now 6 months old — regurgitation increased, enlarging her heart's upper-left chamber.
Still, she was able to work until she was 32 weeks along, and then she came to Salt Lake City for the rest of her pregnancy. When they reached the 36-week milestone, Trevor was born by emergency C-section after her blood pressure skyrocketed. She was in the hospital for a week, including a short stay in intensive care.
Two months later, she had another heart surgery. Although she's exhausted, she's recovering well.
Alyssa had no problems during her first pregnancy, either, although she was watched more closely. "I had more tests and more ultrasounds and in a way that was kind of fun for me. I got to see my baby more and hear the heartbeat more," she says of her daughter, who is now 4.
"I thought that went pretty smoothly," she told her doctors later, when she was considering a second pregnancy. "Can I do this again?" They assured her it would be fine, but midway through the pregnancy, she began to have arrhythmias — irregular heartbeats. It was not clear, though, whether pregnancy kicked it off since people born with transposition of the great vessels at some point do usually develop rhythm abnormalities. "They know all these things will happen, but not when," says Alyssa. "'She'll need a pacemaker.' Check. I've got that. 'She'll have arrhythmias.' Check again."
Echocardiograms showed in both her pregnancies that there are no problems with her babies' hearts.
Her youngest is now 2 and thriving. But Alyssa admits she was nervous her entire first pregnancy because she'd had miscarriages not related to her heart defect. "I was fearful it might happen again and just out of protectiveness did not let myself buy a single thing for the baby until 30 weeks. Even then, I was really scared until she was born." The extra monitoring actually reassured her somewhat, she says, about other issues besides those related to her heart.
The congenital heart problems the doctors see are nearly as varied as the women who come to them wanting to have children or already expecting, says Brann. They've seen patients who have holes in their hearts that were never fixed, as well as patients with heart-rhythm problems, the after-effects of rheumatic fever, or valve problems that require the use of blood thinners.
Some women have the wrong chamber pumping blood, or the heart pumps weakly. When a women has severe narrowing of one heart valve and is pregnant, it's "incredibly risky," Brann said.
"We love to be involved in the process before a woman gets pregnant," he says. "It's so much easier to go in knowing, and often we can fix it." A repaired valve problem, for instance, may remove the excessive risk. "We like to do what we can to get the heart ready for pregnancy."
Treatment may include bed rest and careful monitoring or early delivery. For some, it's more complex than that. Medications have to be carefully considered because some should not be used in pregnancy.
Brann notes that some congenital heart defects require lifelong monitoring, even after they're repaired. Tetralogy of Fallot, which involves four heart malformations, for instance, has been linked to heart-rhythm problems 30 years later.
E-mail: lois@desnews.com