Salt Lake City, UT -- (ReleaseWire) -- 02/28/2022 --Many people may not be aware of a rare form of heart disease that can affect some pregnant and postpartum women called peripartum cardiomyopathy.

"Peripartum cardiomyopathy or PPCM is a heart muscle disease that affects women late in pregnancy, just after delivery, or several months afterward. This condition occurs in women with no history of heart disease," said Kismet Rasmusson, NP, a cardiovascular nurse practitioner and heart failure specialist at the Intermountain Healthcare Heart Institute in Salt Lake City.

"These women present with 'heart failure' (a syndrome that occurs when the heart's structure or function becomes abnormal, combined with the presence of fluid retention) and symptoms as a result," said Rasmusson.

PPCM is a rare condition. It occurs in approximately one in every 1,000-4,000 live births in the United States. PPCM is more common in countries such as Haiti and Nigeria, occurring in up to 1 in 100 to 300 births.

The rates of PPCM seem to be rising due to a variety of reasons such as improved detection of PPCM, increases in maternal age and the presence of other associated risk factors for cardiovascular disease like obesity, high blood pressure (including pregnancy-related high blood pressure), and diabetes.

PPCM is more commonly diagnosed in women while they are in their early 30s, but can occur during any child-bearing age.

National data shows the incidence of PPCM is higher in women with African American ancestry, (it's been reported in over 40% of Black women). From state-level data, Black women have 3-16 times the rate of developing PPCM compared to white women.

"The good news is that more than half of women with PPCM (50-80%) have their heart function return to normal within six months of the diagnosis. We learned this from a national study that Intermountain participated in that included centers across North America (including Canada)," said Rasmusson. "Black women, with a very weak and enlarged heart at the time of diagnosis and delay in diagnosis and treatment, have been found to be associated with less recovery of heart function."

Symptoms of PPCM include shortness of breath, especially with lying down or during sleep; swollen legs or bloated abdomen; fatigue (unexplained and more than normal); and significant cough.

Women should talk to their doctor or midwife if they are experiencing any of these symptoms while pregnant or in the months postpartum.

"Diagnosing PPCM is often tricky, since the symptoms are often experienced in normal pregnancy, but in PPCM are more severe," she added.

PPCM is diagnosed with an ultrasound of the heart called an echocardiogram, chest Xray, an ECG and blood tests.

Risk factors for PPCM include being a Black woman, women who have had multiple pregnancies; multiple gestations (like twins, triplets, etc.); older maternal age, non-Caucasian ethnicity; use of tocolytic therapy (like terbutaline) to stop labor; high blood pressure, before or during pregnancy; obesity and diabetes.

Across the world, investigators have been investigating the cause of PPCM, but many factors may have a role, including abnormal stress or stress on the heart, viral infection, abnormal autoimmune response, the hemodynamic stress of the pregnancy, high blood pressure, nutritional deficits, hormonal abnormalities, and genetic factors.

Treatment during pregnancy includes taking safe heart failure medications that won't affect the baby and then after delivery taking medications that are safe during breastfeeding.

Given the complexity of the care while pregnant with PPCM, it is important for high-risk obstetrics teams to partner with heart failure specialists to provide team-based care. This may include discussing timing for a safe delivery and creating a contingency plan if the patient becomes very sick.

Treatment after pregnancy may include heart failure medications including diuretics, if needed (to get rid of excess fluid), monitoring heart function with office visits, and heart tests such as an echocardiogram of the heart. A pacemaker or defibrillator therapy may be needed in some women with PPCM if the heart doesn't recover. In women with severe forms of PPCM, a heart transplant or mechanical heart pumps may be needed.

Having a discussion about family planning and contraceptive options is important, since PPCM can occur in subsequent pregnancies, especially if the heart doesn't return to normal after PPCM is diagnosed.

Kismet Rasmusson, NP, a maternal fetal medicine surgeon with University of Utah Health and Intermountain Primary Children's Hospital, where she is co-director of fetal intervention at the Utah Fetal Center.

About Intermountain Healthcare
Located in Utah, Idaho, and Nevada, Intermountain Healthcare is a nonprofit system of 25 hospitals, 225 clinics, the Intermountain Medical Group with some 2,700 employed physicians and advanced care practitioners, a health plans division called SelectHealth, Homecare, and other health services. Helping people live the healthiest lives possible, Intermountain is committed to improving community health and is widely recognized as a leader in transforming healthcare by using evidence-based best practices to consistently deliver high-quality outcomes at sustainable costs. For updates, see https://intermountainhealthcare.org/news.