By the time Celeste Richard learned she was carrying twins, she figured she knew a thing or two about childbirth from four uneventful pregnancies. Her fifth was a different story.
Never had she felt so tired, breathless or bloated. "I was swollen, more swollen than I had ever been, but I attributed it to carrying two babies," Richard says. "So did the doctors."
Richard, then 39, got worse, not better, after the twins were born. The day after she brought them home, the family was forced to leave their home in Welsh, La., because Hurricane Rita was barreling their way. Soon after the 10-hour evacuation to Hattiesburg, Miss., where they would be staying with relatives, Richard could hardly breathe.
Doctors found that her heart had all but quit. Richard had somehow developed a rare and mysterious form of heart failure called peripartum cardiomyopathy, which strikes pregnant women. When Hattiesburg cardiologist John Lovejoy made the diagnosis, Richard's heart was pumping out about 5% to 10% of its blood volume, far less than the 60% common to healthy hearts.
Shocking discovery
"I hate to tell you this," said Lovejoy, who admitted her to Wesley Medical Center, when he broke the news. He recommended that she be transferred to the Ochsner Clinic in New Orleans, where, if she failed to improve, she would be placed on a list for a heart transplant.
"That's the hardest thing," Lovejoy says. "Telling a woman who's just had two kids that she may need a transplant. It's a real shock at what should be a joyous time."
What he can't explain is why a 39-year-old "who has just delivered twins and was otherwise happy and healthy" would suffer a sudden, catastrophic breakdown of the heart muscle. Doctors say they are no nearer to understanding pregnancy-related heart failure than when it was first described in the 1840s.
That may change if Jordan Safirstein of St. Vincent's Catholic Medical Center Manhattan and his colleagues have their way. Working with the founders of an Internet-based support group called "A Mother's Heart" -- "The foundation for mothers with big hearts" -- Safirstein's team has launched the first Internet registry of patients with pregnancy-related heart failure.
Using the website, amothersheart.org/PRiCELESS.htm, they have begun recruiting patients for what they hope will be the first study of the disease involving hundreds, if not thousands, of patients.
"Numbers is power," Safirstein says.
Difficult to nail down
The numbers, or the lack of them, are a big reason why so little is known about pregnancy-related heart failure. The disease is so rare that it's all but impossible to study.
The best-known registry in the USA, managed by cardiologist Uri Elkayam of the University of Southern California, involves just 200 patients.
So far, Safirstein's Internet registry has enrolled about 100 women. Nearly 100 more are pulling together the medical information they'll need to join the group.
"I don't ever see a time when we should stop enrolling patients," he says, adding that he hopes that the registry ultimately will attract funding from the National Heart, Lung, and Blood Institute, or NHLBI.
In 2000, the NHLBI did its best to describe peripartum cardiomyopathy despite the information vacuum. All the agency could say with certainty is that the disease is marked by the development of heart failure in the last month of pregnancy without an apparent cause or any other pre-existing heart problem.
The agency called for the establishment of a national registry to learn more about the disease.
The cause remains a mystery. Doctors still don't know how often it occurs. In the USA, estimates veer wildly from one in every 3,000 to one in every 15,000 pregnancies. Death rates range from 18% to 56%. The disease sometimes goes undiagnosed until it's too late, because heart failure symptoms mimic those of a normal pregnancy.
Pregnant women with the condition "often go to the doctor saying 'I'm short of breath, my legs are swollen and I can only walk a block or two,'" and are ignored because that's the same litany of complaints doctors routinely hear from pregnant women, Safirstein says.
With so little information, the outcome is also difficult to predict. Most recent studies suggest that half of women recover some heart function, 30% remain in chronic heart failure and about 10% end up with severe heart failure or a heart transplant, or they die.
Those who recover are told that having another child could cost them their lives. "We're dealing with young women," Safirstein says. "At such a happy time in their lives, they're told they have heart failure and they can't have any more babies. It's devastating."
Much improved
Even Richard, now 41 with six children, found that hard to accept. But she recognizes the risks are too great. "My husband's thinking is, 'I don't think God gave us six children for you to die and leave someone else to raise them,'" she says. "I agree with that." Fortunately, her heart has regained much of its pumping power. "She's doing really well," Lovejoy says.
Richard says she'd like other women to have the same shot at recovery that she has had. That's why she has joined Safirstein's registry.
"There are so many unknowns," she says. "It's not easy to understand. Why did it affect me? I'm interested in finding out all we can about why this happens.
"Maybe we can help prevent it from happening to someone else."
The following fields overflowed:
OBJECT = bl_cover13_mothershearts13 D07_pregnancyheart_A_13.jpg13 D07_PregnancyHeart_B_13.jpg13
To see more of USAToday.com, or to subscribe, go to http://www.usatoday.com
??? Copyright 2007 USA TODAY, a division of Gannett Co. Inc.