What Is Peripartum Cardiomyopathy?

Peripartum cardiomyopathy (PPCM), also known as postpartum cardiomyopathy, is a rare heart condition that can occur during the later stages of pregnancy or in the weeks to months following pregnancy. "Cardiomyopathy" is a term for a heart muscle weakness. A weak heart muscle impairs the heart's function and often leads to heart failure.

Peripartum cardiomyopathy occurs in about 1 of every 1,000 to 4,000 deliveries. Some symptoms are similar to symptoms you may notice in pregnancy, like shortness of breath, fatigue, and leg swelling.

Read on to learn more about peripartum cardiomyopathy, its symptoms, management, and outlook.

What Is Peripartum Cardiomyopathy?

Peripartum cardiomyopathy is an otherwise unexplained heart failure that occurs near the end of pregnancy or in the year following pregnancy. In peripartum cardiomyopathy, the heart is often enlarged, and the pumping function of the heart's main chamber (the left ventricle) is impaired. This leads to heart failure symptoms like shortness of breath and swelling.

Usually, the left ventricle pumps out over half of the blood inside it with each heartbeat. This is a measure known as the ejection fraction (EF), and normal EF is around 55% or higher. However, in PPCM, there is a reduced EF (less than 45%) as the left ventricle is weakened. This is also known as systolic heart failure or heart failure with reduced ejection fraction (HFrEF).

Peripartum cardiomyopathy usually occurs near the end of pregnancy or shortly after but can be diagnosed anytime during the year after pregnancy. In some cases of peripartum cardiomyopathy, there can be improvement and a full recovery in heart muscle function with time and medications.

What Causes Peripartum Cardiomyopathy?

The exact cause of peripartum cardiomyopathy is still under investigation. Studies have suggested a role of genetics, inflammation, and abnormal immune response during pregnancy. One theory involves the breakdown of the prolactin hormone (that stimulates breast milk production during and after pregnancy) into a toxic fragment to the heart.

This may explain how treatments that use bromocriptine, a medication that reduces prolactin levels, is beneficial in peripartum cardiomyopathy.

Who's at Risk for Peripartum Cardiomyopathy?

The following factors increase the risk of developing peripartum cardiomyopathy:

• Being over age 30
• History of preeclampsia, a blood pressure disorder during pregnancy
• History of high blood pressure before pregnancy
• Smoking
• Family history of peripartum cardiomyopathy
• Diabetes

Having had peripartum cardiomyopathy with a previous pregnancy greatly increases the risk, especially if the left ventricular function has not completely recovered.

What Are the Symptoms of Peripartum Cardiomyopathy?

Some of the symptoms of PPCM can mimic symptoms that are commonly seen toward the end of pregnancy. It's important to discuss any new or changing symptoms with your healthcare provider.

Symptoms of peripartum cardiomyopathy are similar to symptoms in other types of heart failure, and include:

• Shortness of breath
  
• Difficulty breathing when lying flat, needing to prop your head on pillows to breathe comfortably, and waking up at night short of breath
• Swelling in the feet, legs, abdomen
• Sudden weight gain
• Fatigue
• Palpitations

How Is Peripartum Cardiomyopathy Diagnosed?

Diagnosis of peripartum cardiomyopathy is often delayed since some of the symptoms are similar to symptoms that are frequently seen in pregnancy, like shortness of breath and leg swelling. When peripartum cardiomyopathy is suspected, a heart ultrasound, known as an echocardiogram, provides information about the heart's structure and function.

An echocardiogram can reveal a dilated heart and calculate the ejection fraction. It can also provide clues as to the cause of the cardiomyopathy. When no other cause is identified, and the condition is diagnosed in the right time frame relating to pregnancy, peripartum cardiomyopathy is diagnosed.

Other tests may include:

• Laboratory tests for kidney function, electrolytes, and heart proteins (cardiac biomarkers)
• Electrocardiogram (ECG or EKG)
• Chest X-ray in some cases to evaluate fluid in the lungs
• Other heart imaging tests to look for other causes of heart failure

What Are the Treatment Options for Peripartum Cardiomyopathy?

Treatment of peripartum cardiomyopathy includes medications, and in some cases special medical devices to support your heart. If you experience fluid retention, you may be advised to limit salt intake.

When peripartum cardiomyopathy occurs during pregnancy, early delivery may be necessary. A cardiologist (heart specialist) and obstetrician-gynecologist (ob-gyn; a specialist who manages many aspects of women’s reproductive health, including pregnancy and childbirth) will work together to determine risks and benefits to you and your baby and make recommendations on an individual basis.

It's essential to follow closely with a cardiologist who can prescribe medications for your heart and monitor your heart's function over time.

Medications

Medications used in peripartum cardiomyopathy and other types of heart failure may include:

• Diuretics
• Beta-blockers
• Angiotensin-converting enzyme (ACE) inhibitors or angiotensin receptor blockers (ARBs) (may be given after pregnancy but not during pregnancy due to risk of harm to fetus)
• Aldosterone antagonists
• Anticoagulants (blood thinners)

In some studies, bromocriptine has been shown to improve outcomes in peripartum cardiomyopathy. However, the 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure, a report by the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines, states that evidence is uncertain.

Cardiac Devices

In severe cases of peripartum cardiomyopathy involving cardiogenic shock, temporary circulatory support devices may be needed. These devices assist a weakened heart in pumping blood to your body. When there is an increased risk of arrhythmia (abnormal heart rhythm or heartbeat), such as in those whose heart remains weak for some time despite using medications, an implantable cardioverter defibrillator (ICD) may be recommended.

Peripartum Cardiomyopathy Complications

Peripartum cardiomyopathy can range in severity, with little impact on heart function and mild symptoms, to severe, with cardiogenic shock (life-threatening condition in which the heart can’t pump enough blood and oxygen to the brain and other organs) and death. Complications can include:

• Cardiogenic shock
• Blood clots
• Arrhythmias
• Stroke
• Death

Long-Term Outlook

The prognosis of peripartum cardiomyopathy varies widely. Studies have shown recovery of heart function in up to 75% of cases. Those diagnosed late in the time frame and with more severe peripartum cardiomyopathy are less likely to experience full recovery of heart function.

Importantly, peripartum cardiomyopathy can recur or worsen with subsequent pregnancies. If you have had peripartum cardiomyopathies, ask your cardilogist about the risks and benefits of getting pregnant again. if you do become pregnant, it is recommended to have careful monitoring during and after pregnancy.

How to Prevent Peripartum Cardiomyopathy

It may not be possible to prevent PPCM, however, there are ways to help keep a strong heart in general. These include:

• Quit smoking and avoid secondhand smoke.
• Avoid alcohol (especially during pregnancy) outside of pregnancy limit alcohol and drink only in moderation.
• Get regular physical exercise.
• Eat a heart-healthy diet.

For those who have had peripartum cardiomyopathy previously, especially if heart function has not recovered, making a plan to avoid unexpected pregnancy is recommended.

Summary

Peripartum cardiomyopathy is a rare but potentially severe form of heart failure that can affect women late in pregnancy or the year after delivery. Symptoms may mimic some of those experienced in pregnancy, such as shortness of breath and ankle swelling. In severe cases, cardiogenic shock may occur.

Treatment includes medications, and sometimes cardiac devices are needed. A team of cardiologists and obstetricians can make an individualized treatment plan and discuss risks for future pregnancies.