Peripartum Cardiomyopathy

pregnant woman in bed

What is peripartum cardiomyopathy?

Peripartum cardiomyopathy (PPCM), also known as postpartum cardiomyopathy, is an uncommon form of heart failure that happens during the last month of pregnancy or up to five months after giving birth. Cardiomyopathy literally means heart muscle disease.

PPCM is a dilated form of the condition when the heart chambers enlarge and the muscle weakens. This causes a decrease in the percentage of blood ejected from the left ventricle of the heart with each contraction. That leads to less blood flow. Then the heart can’t meet the demands of the body’s organs for oxygen, affecting the lungs, liver and other body systems.

PPCM is rare in the United States, Canada, and Europe. About 1,000 to 1,300 women develop the condition in the U.S. each year. PPCM is much more common in some countries and may be related to differences in diet, lifestyle, other medical conditions or genetics.

How is it diagnosed?

PPCM may be difficult to detect because symptoms of heart failure can mimic those of third trimester pregnancy, such as swelling in the feet and legs, and some shortness of breath. More extreme cases include severe shortness of breath and prolonged swelling after delivery.

During a physical exam, health care professionals will look for signs of fluid in the lungs. They will use a stethoscope to listen for lung crackles, a rapid heart rate or abnormal heart sounds. An echocardiogram can detect cardiomyopathy by showing the diminished functioning of the heart.

PPCM is diagnosed when the following three criteria are met:

  1. Heart failure develops in the last month of pregnancy or within five months of delivery.
  2. Heart pumping is reduced, with an ejection fraction (EF) less than 45% (typically measured by an echocardiogram). EF is how much blood the left ventricle pumps out with each contraction. A normal EF can be between 55 and 70.
  3. No other cause for heart failure with reduced EF can be found.

Laboratory blood tests are a standard part of the evaluation. This includes tests to assess kidney, liver and thyroid function; tests to assess electrolytes, including sodium and potassium; and a complete blood count to look for anemia or evidence of infection. Markers of cardiac injury and stress can also assess level of risk.

Symptoms of the condition include:

  • Fatigue
  • Feeling of heart racing or skipping beats (palpitations)
  • Increased nighttime urination
  • Shortness of breath with activity and when lying flat
  • Swollen ankles
  • Swollen neck veins
  • Low blood pressure, or it may drop when standing up.

The New York Heart Association system classifies the severity of symptoms in patients with PPCM:

  • Class I - Disease with no symptoms
  • Class II - Mild symptoms/effect on function or symptoms only with extreme exertion
  • Class III - Symptoms with minimal exertion
  • Class IV - Symptoms at rest

What are the causes?

The underlying cause is unclear. Heart biopsies in some cases show women have inflammation in the heart muscle. This may be because of prior viral illness or abnormal immune response. Other potential causes include poor nutrition, coronary artery spasm, small-vessel disease and defective antioxidant defenses. Genetics may also play a role.

Initially thought to be more common in women older than 30, PPCM has since been reported across a wide range of age groups. Several risk factors include:

  • Older maternal age
  • History of cardiac disorders, such as heart attack, heart valve dysfunction or myocarditis (inflammation of the heart muscle)
  • Exposure to toxins, such as alcohol or chemotherapy
  • High blood pressure
  • Multiple pregnancies
  • Multifetal pregnancy (i.e., twins)
  • Use of certain medications to prevent premature labor
  • African descent
  • Poor nutrition

How can PPCM be treated?

The objective of peripartum cardiomyopathy treatment is to keep extra fluid from collecting in the lungs and to help the heart recover as fully as possible. Many women recover normal heart function or stabilize on medicines. Some progress to severe heart failure requiring mechanical support or heart transplantation.

A physician can prescribe several classes of medications to treat symptoms, with variations that are safer for women who are breastfeeding.

  • Angiotensin converting enzyme (ACE) inhibitors – Help the heart work more efficiently
  • Beta blockers – Cause the heart to beat more slowly so it has recovery time
  • Diuretics – Reduce fluid retention
  • Digitalis – Derived from the foxglove plant, it has been used for more than 200 years to treat heart failure. Digitalis strengthens the pumping ability of the heart
  • Anticoagulants – To help thin the blood. Patients with PPCM are at increased risk of developing blood clots, especially if the EF is very low.

Health care professionals may recommend a low-sodium diet, fluid restrictions or daily weighing. A weight gain of 3 to 4 pounds or more over a day or two may signal a fluid buildup.

Women who smoke and drink alcohol will be advised to stop, because these habits may make the symptoms worse.

A heart biopsy may help determine if the underlying cause of cardiomyopathy is a heart muscle infection (myocarditis). But this procedure is uncommon.

How can women minimize their risk?

To develop and maintain a strong heart, women should avoid cigarettes and alcohol, eat a well-balanced diet and get regular exercise. Women who develop peripartum cardiomyopathy are at high risk of developing the same condition with future pregnancies.

What’s next?

Ongoing studies continue to help researchers better understand the cause of PPCM and develop new treatments. Health care professionals have tried treatments that alter the immune system, such as intravenous γ-globulin and immunoabsorption, but they’re not proven. Researchers also have focused on the role of prolactin in PPCM. Prolactin is a hormone released from the pituitary gland late in pregnancy and after delivery that stimulates breast milk production. But prolactin may have adverse effects on the heart muscle by limiting its blood supply and causing cell death. Bromocryptine is a medication that inhibits the pituitary secretion of prolactin. Early studies suggest it helps treat PPCM, but more research is needed.