Rare disease can affect women late in pregnancy
Brittney Morton, 24, left, plays with her 1-year-old daughter, Maliyiah Meadows, on the porch of their Homewood home. Ms. Morton has the heart ailment peripartum cardiomyopathy, which attacks women during pregnancy or in the first five months after childbirth.
Molly Kimberling, 20, of Mount Washington and her 18-month-old daughter, Ava. Ms. Kimberling had a heart transplant in 2010 after developing peripartum cardiomyopathy.
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Molly Kimberling was too sick after delivering to enjoy her new daughter, Ava.
"I couldn't breathe. I couldn't walk. I was just really sick," said Ms. Kimberling, now 20, of Mount Washington, of the period following Ava's birth on Jan. 22, 2010. "I had no energy. Nothing."
Brittney Morton, of Homewood, started feeling sick when her daughter, Maliyiah Meadows, born Jan. 18, 2010, was several months old.
"I had weight gain. I was still at my birth weight and [had] some swelling." She would lose her breath just walking from one room to another and had chest pain.
Although the degrees of their illnesses differed, the two young women were diagnosed with the same condition: A rare heart disease called peripartum cardiomyopathy.
The disease, cause of which is unknown, apparently strikes women late in pregnancy or in the first few months after delivery. In the United States, it occurs in one of every 3,000 to 4,000 live births -- much more often in other parts of the world, especially Africa and Haiti, according to Dennis McNamara, director of the Heart Failure/Transplantation Program at UPMC.
The disease can be life-threatening, just like other forms of dilated cardiomyopathy, or weakened heart. But about 50 percent of those stricken recover over the first six months after diagnosis and the heart goes back "almost completely to normal," Dr. McNamara said.
That was not the case for Ms. Kimberling or Ms. Morton, though Dr. McNamara said "Brittney was not as sick as Molly when she presented."
Ms. Kimberling eventually needed a heart transplant, which took place July 18, 2010. She said she now feels "better than ever before," and Dr. McNamara said she has "done as well as anyone possibly can."
But Ms. Morton's heart has not recovered and as a preventive measure she had a defibrillator implanted to shock her heart should she go into cardiac arrest. She takes medications typical to a heart failure patient: two diuretics, a beta blocker and an ACE inhibitor.
"I'm not like my usual self, but I'm getting better with time," she said.
Dr. McNamara said Ms. Morton was being evaluated for gall bladder problems rather than heart failure when he first saw her, a "classic, or common, story."
Peripartum cardiomyopathy patients are often misdiagnosed before their real problem is pinpointed.
These patients can have "shortness of breath, especially on exertion; fluid or swelling in the feet and easy fatigue," said Indu Poor- nima, director of the Women's Heart Center of Allegheny General Hospital. The same symptoms can accompany a normal pregnancy, she added. "Weight gain can cause shortness of breath; swelling in the feet [can occur] because of pressure on the uterus, so it can sometimes be hard to differentiate unless the patient comes in to the hospital with heart failure itself."
Presented with these symptoms when the woman is postpartum, doctors respond "you just had a new baby" or "you're out of shape from the pregnancy," Dr. McNamara said. "You don't expect a 24- or 25-year-old woman to have heart failure, especially one that's just given birth."
Other symptoms, which are common to heart failure, include swelling of the belly, nausea and abdominal discomfort caused by fluid backing up into the liver.
There are identifiable risk factors, said Ronald Thomas, director, Division of Maternal-Fetal Medicine for the West Penn Allegheny Health System: It tends to happen more often to older mothers, women having multiple births, women of African-American heritage and women with a history of high blood pressure.
"We know African-Americans are at the highest risk," Dr. Poornima said.
It also happens more commonly in women "who've had many pregnancies," Dr. McNamara said, "but it can happen to any woman, and I see many women in their first pregnancy who present with this disorder."
He said some doctors also think there is a "subset of women who may have a genetic predisposition to cardiomyopathy that is brought out by the stress of pregnancy."
Whatever the cause, a patient with symptoms is diagnosable by a combination of medical history, physical exam, and, most important, an echocardiogram, doctors said.
"In terms of treatment, it's pretty much the same as treatment for cardiac failure or cardiomyopathy in general," Dr. Thomas said. Patients should be given blood-thinners, medications like beta blockers slow the heart rate to improve the heart's squeeze and to decrease the workload and diuretics to decrease fluid overload, he added. They also may need to be treated for any irregular heartbeat.
If the woman has not yet delivered, some heart medications should not be given because of danger to the baby, Dr. Poornima said. For example, she said ACE inhibitors and ARB could affect the fetus' kidneys.
Any danger to the mother is danger to the baby, Dr. Thomas said.
"[The fetus] is absolutely dependent on maternal health and her ability to circulate blood," he said. "One of the toughest decisions is whether the baby is better inside or outside and then deciding how to get the baby outside, whether by vaginal delivery or cesarean section."
More times than not, though -- "roughly 90 percent of the time," Dr. McNamara said -- the mother already has delivered when the condition is diagnosed.
The classic definition of peripartum cardiomyopathy is for women to show the symptoms either in the last month of pregnancy or five months after childbirth, but that definition is being questioned, he added. "Most modern investigators feel that timeline is arbitrary and women can actually present earlier in pregnancy or later than five months [peripartum]."
Whether a woman who has had peripartum cardiomyopathy can have another child depends on whether her heart has recovered completely.
Generally, if the patient's heart fully recovers, she may be able to consider another pregnancy, Dr. McNamara said. However, in that next pregnancy, there's approximately a 20 percent chance the peripartum cardiomyopathy will recur.
If the patient does not recover, it would be strongly recommended she not consider pregnancy again as the risk to the mother is too great, he said.
Ms. Morton, whose heart has not recovered, has been advised that "now is not a good time [for pregnancy] ... it's hazardous to me and my well-being," she said, adding she has not discussed long-term prospects with her doctors.
But Dr. McNamara said "we don't recommend in general pregnancy for women with cardiomyopathy and a weakened heart."
Even if the patient has recovered there is risk, he repeated, "so we counsel them to appreciate the risk and work with them. ... We have followed many women through a second pregnancy [but] they have to be watched fairly closely."
The issue of another pregnancy is different if the patient has gone on to have a heart transplant, like Ms. Kimberling has. "Certainly we have had transplant patients have a child," Dr. McNamara said. "It's not common, but we've had women who've done that."
Ms. Kimberling said she will be cautious: "Before I ever got pregnant I'd make sure it's OK."
First Published September 5, 2011 12:00 am