Peripartum Cardiomyopathy: What to Know

Many women have never heard of it, but peripartum cardiomyopathy (PPCM) is the most common cardiovascular killer among new mothers.

PPCM symptoms are so similar to those of pregnancy — fatigue, shortness of breath and swelling in the feet and legs — that this cardiovascular emergency is often misdiagnosed or diagnosed late.

That can prove fatal.

Liviu Cojocaru, M.D., an obstetrician who specializes in maternal-fetal and critical care medicine at the University of Miami Health System, explains that PPCM, also known as pregnancy-associated cardiomyopathy, “is a rare form of heart failure occurring in women without prior heart disease, usually during the last month of pregnancy or up to five months postpartum.”

PPCM affects about 1 in every 1,000 to 4,000 live births.

Globally, prevalence varies significantly, ranging between 1 in 100 to 1 in 20,000 live births depending on geographic location.

PPCM is a systolic dysfunction characterized by the enlargement of the heart chambers and a weakening of the heart muscle. These conditions lead to a drop in the heart’s pumping ability. In other words, the amount of blood the heart pumps with each heartbeat decreases, sometimes precipitously.

The condition is treatable, and many women recover, but early diagnosis and treatment are crucial to limit the potential complications. “Delayed diagnosis of PPCM can result in persistent heart failure, abnormal heart rhythms (arrhythmias), or even death,” says Dr. Cojocaru.

The estimated mortality rate associated with PPCM ranges from as low as 2% to as high as 56% in some populations. In the more severe cases, survivors do not fully recover and may even require heart transplantation.

Studies have yet to uncover the underlying cause of the condition.

Most experts, such as Dr. Cojocaru, believe it is likely due to several factors. Recent research suggests an association between PPCM and hypertensive disorders of pregnancy, including preeclampsia.  In fact, one study found that severe preeclampsia significantly increased the risk of developing PPCM.

Another study found that high levels of prolactin (a hormone primarily responsible for stimulating milk production in mammals) and of soluble Fms-like tyrosine kinase 1 (a protein that inhibits the formation of new blood vessels) contribute to cardiac dysfunction.

Still other studies point the finger at nutritional deficiencies, abnormal immune response, prior viral illness and hemodynamic stress during pregnancy, according to the American Heart Association.

“No single, unified cause for peripartum cardiomyopathy has been identified,” says Dr. Cojocaru. “However, researchers have identified multiple factors that increase risk.”

These factors include: 

• advanced maternal age
• multiple pregnancies
• diabetes
• obesity
• pregnancy-induced hypertension
• certain genetic predispositions (About 10% to 20% of women with PPCM have genetic mutations associated with cardiomyopathies.)

Patients who identify as Black also face a higher risk, though it is not understood what role race plays in PPCM.

How is PPCM diagnosed?

Typically, doctors diagnose using an echocardiogram, a noninvasive test that assesses the heart’s pumping function. A physician will specifically look for an ejection fraction (EF) measure less than 45%. A normal measure is between 50% to 70%. Physicians use blood tests to assess various organ functions.

But even before a woman gets to this stage, she must be vigilant of her own symptoms. “Women should watch for unusual fatigue, prominent ankle swelling, rapid heartbeat or palpitations, persistent cough and shortness of breath, especially at rest or while lying flat,” Dr. Cojocaru says. “Prompt medical evaluation is critical if these symptoms develop.”

Once diagnosed, treatment for PPCM is the same as that for typical heart failure, with modifications for mothers who are breastfeeding.

With proper treatment, he adds, 50% to 80% of women show “significant improvement in heart function” within 6 to 12 months. Women with very low initial heart function or genetic risks have poorer recovery outcomes, however.

 “The primary goal of treatment is to relieve heart failure symptoms, improve cardiac function, and prevent serious complications,” he adds. “Treatment usually involves a comprehensive, multidisciplinary approach that includes medications, lifestyle modifications, and in some severe cases, advanced interventions.”

Written by Ana Veciana-Suárez, a regular contributor to the University of Miami Health System. She is an acclaimed author and journalist who has worked for The Miami Herald, The Miami News, and The Palm Beach Post.

Tags: Dr. Liviu Cojocaru