ANNE McILROY
From Friday's Globe and Mail Published on Friday, Mar. 14, 2008 9:00AM EDT Last updated on Monday, Mar. 30, 2009 3:14PM EDT
Deanna Dudley was a blue baby, born with two holes in her heart. Doctors told her parents she had only a 50-per-cent chance of living more than 24 hours.
She spent most of her early years in the hospital, but when she was 10, a surgeon repaired the defects. Ms. Dudley, now 36, was able to live a normal life. So normal, in fact, that last July she gave birth to a healthy baby boy.
Doctors have become so proficient at detecting and treating heart defects in children that the number of adults with congenital heart disease has soared by 85 per cent in a generation, an advance that has been heralded as one of the great success stories of 20th-century medicine.
Now, many of those babies - born with hearts that couldn't get enough red, oxygenated blood to their lips, noses and fingertips - are having children of their own. The special pregnancy program at Mount Sinai Hospital delivers about 55 babies a year from mothers with congenital heart disease, up from about 10 a year in 1987.
That is probably more than any other hospital in the world, says Mathew Sermer, head of the hospital's medical disorders of pregnancy program.
Most, like Ms. Dudley, have only a slightly increased chance of having an infant with heart problems - 2 to 5 per cent compared to 0.5 per cent for the general population, although with some defects the risk is significantly higher. Most can bear a child without bringing on heart failure, a stroke or other serious complications that can be triggered by the strain pregnancy puts on the heart.
"People who in the past wouldn't dare get pregnant do get pregnant and most of them do well," Dr. Sermer says.
Ideally, as was the case with Ms. Dudley, patients are screened before they conceive, both to assess their risk of having a baby with a malformed heart and to judge whether their own hearts can withstand the extra work of carrying and delivering a baby.
But sometimes women with congenital heart disease have unplanned pregnancies, or choose to have a baby against the advice of their medical team, says Ariane Marelli, an associate professor of medicine at McGill University in Montreal and director of the McGill Adult Unit for Congenital Heart Disease.
Karine Auclair, a chemist at McGill, was born with a narrow valve in her aorta, the largest artery in the heart, but didn't need surgery to fix it until she was an adult. There were complications, and she needed two other operations. Dr. Marelli advised her not to become pregnant, she says.
Two weeks later, Ms. Auclair started feeling nauseous.
"I was already pregnant," she says.
She and her husband decided to continue with the pregnancy despite the risks. Baby Lia was born on May 18, 2006, by cesarean section and weighed 6 pounds, 7 ounces.
"I was very lucky, everything was okay."
Dr. Sermer says he has had patients continue with pregnancies even though he has told them they have a 30 per cent chance of dying if they do.
Some have gone through many operations, and been told they would never survive, he says. Pregnancy is just another obstacle to overcome.
"Our role is to support the decision they make."
But these kinds of cases are rare, he says. He sees only a handful a year.
Pregnancy can be dangerous for women with heart problems because the volume of blood in the body increases by about 50 per cent during the second and third trimesters, says Erwin Oechslin, director of the Congenital Cardiac Centre for Adults at Toronto General Hospital. The centre collaborates with the Mount Sinai special pregnancy program. Many patients are seen by doctors at both hospitals.
"Pregnancy is actually a stress test, lasting about six months," he says. "The question is, can the heart tolerate this stress test?"
Ms. Dudley's heart is on the right side of her body, not the left. The team at Mount Sinai told her it could handle a
pregnancy.
With the help of fertility treatments, she and her husband conceived, and ultrasounds taken throughout the pregnancy showed normal fetal heart development.
"It was good to know. I wouldn't want to go through what my parents went through." Now that she is a parent, she says, she understands more about what it must have been like for them to have a seriously ill child whose second home was the hospital.
She went into labour five weeks early, but the medical team at Mount Sinai had already put in place a plan for the delivery. Some women with congenital heart disease give birth in the operating room in case emergency heart surgery is required. If prenatal screening shows the baby has heart defects, neonatal cardiologists and surgeons will also be on standby.
In Ms. Dudley's case, they had a defibrillator outside the door of her room. Doctors also decide ahead of the delivery if a patient should have a cesarean section. Ms. Dudley's heart was deemed strong enough to push the baby out.
She was given an epidural early on to spare her heart as much stress as possible. Different medications are used in women with congenital heart disease, Dr. Sermer says. Local anesthetics can cause a decrease in blood pressure, which could kill some patients, he says, so more narcotics are used instead.
Marcus Dudley was born after 12 hours of labour. He weighed 5 pounds, 3 ounces. Today he is starting to sit up on his own and eat solid food.
When Ms. Dudley was a child, her mother always told her she could do anything she wanted, but her father was a worrywart; he didn't want her to ride her bike, didn't want her siblings to make her cry.
"My dad would say, 'she is special.' Mom would say, 'she's not special, she's fine,' " says Ms. Dudley, who lived in Toronto when she had her baby, but has moved to Wallaceburg in southwestern Ontario to be closer to her family.
Her mom died of cancer a few years before Ms. Dudley became pregnant, but her father is a proud - and relieved - grandfather.
"He is ecstatic."
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