Peripartum Cardiomyopathy

What is peripartum cardiomyopathy?

Peripartum cardiomyopathy is a rare condition that weakens your heart muscle. This lowers your heart’s ability to pump blood to the rest of your body, which can be life-threatening. This condition affects women in the last month of pregnancy or up to five months after delivery. For that reason, people also call it postpartum cardiomyopathy or pregnancy-associated cardiomyopathy. It can happen at any age but is most common in those older than 30.

Peripartum cardiomyopathy damages one of the chambers of your heart, the left ventricle, and makes it less able to do its job. Your left ventricle has the important job of pumping oxygen-rich blood throughout your body.

You might hear your healthcare provider use the term “left ventricular ejection fraction” (LVEF). They usually check this with an echocardiogram. LVEF refers to how well your left ventricle can pump blood out of your heart. Providers describe your LVEF as a percentage, and a higher number is better. Normally, your LVEF is 50% to 70%. Peripartum cardiomyopathy reduces your LVEF to less than 45%. The lower your LVEF, the more serious your condition.

An early diagnosis is key for getting proper treatment and greatly increases your chances of a smooth recovery.

How common is peripartum cardiomyopathy?

Peripartum cardiomyopathy isn’t common, but it’s hard to say exactly how rare it is. Estimates vary widely both in the U.S. and globally.

In the U.S., peripartum cardiomyopathy affects between 1 in 1,000 and 1 in 4,000 pregnancies. Estimates show it’s more common in the southern U.S. than in other regions.

What does peripartum cardiomyopathy feel like?

Peripartum cardiomyopathy symptoms are easy to miss because many of them are like what you feel during pregnancy. But because the condition is so serious, it’s important to watch for the following symptoms:

• Shortness of breath (dyspnea) — especially when you’re trying to sleep, lying down or doing physical activity.
• Fatigue.
• Swelling (edema) in your feet and ankles.
• Heart palpitations.
• Dry cough.
• Swollen neck veins.
• Lightheadedness.
• Low blood pressure (hypotension) or blood pressure that drops suddenly when you stand up.
• Chest pain.

Peripartum cardiomyopathy usually begins in the last month of pregnancy or later. But it can start sooner. It's also possible for pregnancy to put extra strain on your heart and make a previously undiagnosed heart condition worse. So, it’s important to track symptoms throughout your pregnancy, not just in the final weeks.

What causes cardiomyopathy in postpartum?

Researchers don’t know what causes peripartum cardiomyopathy. But they think possible causes include hormonal changes during pregnancy and other conditions like preeclampsia. Several factors may combine to cause this condition.

Peripartum cardiomyopathy may be hereditary (passed from parents to their biological children), but research hasn’t proven this link yet. About 15% to 20% of women with peripartum cardiomyopathy have genetic mutations that can cause cardiomyopathy. If you have this genetic mutation, the stress of pregnancy and delivery might trigger cardiomyopathy symptoms. It’s important for you and your cardiologist to know of any history of heart diseases, specifically cardiomyopathy or heart failure, in your biological family.

What are the risk factors for peripartum cardiomyopathy?

Peripartum cardiomyopathy has many risk factors. The more risk factors you have, the more likely you are to develop peripartum cardiomyopathy. The risk factors add up to pose an even greater threat than one would on its own.

Risk factors before pregnancy

• High blood pressure (hypertension).
• Diabetes.
• Obesity.
• Selenium and zinc deficiency.
• Substance use disorder (SUD).

Risk factors related to pregnancy

• Being pregnant for the first time.
• Being pregnant with twins or triplets.
• Having a prior diagnosis of peripartum cardiomyopathy.
• Using technology (like IVF) to help you get pregnant.

Complications during pregnancy

• Preeclampsia and eclampsia.
• Anemia.
• Thyroid disease.
• Asthma or autoimmune disease flare-up.
• Extended use of medicine to delay delivery if you have preterm contractions.

Special considerations for Black women

Research has shown that Black women:

• Have higher rates of a peripartum cardiomyopathy diagnosis.
• Have more severe heart damage and reduced heart pumping ability from the condition.
• Are more likely to have more serious complications.
• Get a diagnosis at a younger age (27 compared with 31).
• Get a diagnosis later in the postpartum period, when the problem has gotten more serious.
• Have a higher risk of dying from the condition.

What are the complications of peripartum cardiomyopathy?

As your left ventricle gets weak and tired, it can’t pump blood as efficiently to your lungs, liver and other organs that rely on it. This slowdown affects your whole body. It leads to heart failure and raises your risk of blood clots and thrombosis.

Life-threatening complications may include:

• Abnormal heart rhythms (arrhythmias).
• Cardiogenic shock.
• Severe heart failure.
• Issues from a blood clot.
• Injury to your brain.

How is peripartum cardiomyopathy diagnosed?

Your provider will diagnose you with peripartum cardiomyopathy if you meet all three of these criteria:

• You develop heart failure near the end of pregnancy or within five months of delivery.
• Your provider can’t find any other cause for your heart failure. (They have to rule out anything else that could cause your symptoms.)
• Your left ventricular ejection fraction (LVEF) is less than 45%.

To reach a diagnosis, your provider will talk with you about your health history and family history.

When making a diagnosis, your provider may refer to your condition as a Class I, II, III or IV. The higher the number, the more severe your condition. An early diagnosis reduces your risk of dying from peripartum cardiomyopathy and allows you to get treatment as soon as possible. If you get a diagnosis after the condition gets worse, you’re more likely to have serious complications like continued heart problems.

What tests will be done to diagnose peripartum cardiomyopathy?

There’s no specific test for diagnosing peripartum cardiomyopathy. Instead, your provider will use other tests along with the information you give them. They’ll perform a physical exam to look for signs of extra fluid in your body. Then, you’ll have tests that may include:

• Blood tests.
• Electrocardiogram (ECG/EKG).
• Chest X-ray.
• Echocardiogram (echo). This will show your left ventricular ejection fraction (LVEF), which is an essential part of your diagnosis.
• Heart MRI (magnetic resonance imaging).
• Heart catheterization.
• Myocardial biopsy (rare).

These tests will reveal any heart problems that you had before your pregnancy. Some people have heart problems for years and don’t know until they start having symptoms during pregnancy. Your test results will help your provider make an accurate diagnosis.

How is peripartum cardiomyopathy treated?

Peripartum cardiomyopathy treatment manages heart failure symptoms and helps your heart recover. The condition doesn’t go away on its own. If you get a diagnosis during pregnancy, your provider will make sure any medications are safe for the fetus. They’ll talk with you about treatment options and discuss possible side effects. You’ll also likely work with a team of specialists who can offer advice on high-risk pregnancy and the impact of heart disease on pregnancy.

You’ll probably need medications that also treat other types of heart failure, like:

• Angiotensin-converting enzyme (ACE) inhibitors.
• Angiotensin II receptor blockers (ARBs).
• Angiotensin receptor/neprilysin inhibitors (ARNIs).
• Beta-blockers.
• Diuretics.
• Hydralazine/nitrates.
• Mineralocorticoid receptor antagonists (MRAs).
• Sodium-glucose cotransporter-2 (SGLT2) inhibitors.
• Anticoagulants.

Depending on how well your heart is functioning, you may need other treatments, like:

• A cardiac device like a wearable or implantable cardiac defibrillator.
• A left ventricular assist device (LVAD).
• Heart transplant. This is usually for people with advanced disease who have a very low left ventricular ejection fraction (LVEF) and don’t respond to therapy.

Complications and side effects of treatment

Side effects vary based on the treatment and whether you’re pregnant at the time. Taking beta-blockers during pregnancy may cause your baby to have a smaller birth weight or conditions like hypoglycemia, bradycardia or heart block.

Some medicines — including ACE inhibitors, ARBs, ARNIs, MRAs and SGLT2 inhibitors — aren’t safe to use during pregnancy. But they’re generally safe to use while breastfeeding since only small amounts pass into your breast milk. It’s important to talk with your provider about the timing, risks and side effects of each medication.

How soon after treatment will I feel better?

More than 50% of people who have peripartum cardiomyopathy recover to have a typical heart function — often with less than six months of treatment. For some, it only takes a few days or weeks. Others may need years to recover.

But you’ll likely need to keep taking medicine for at least a year after your heart function recovers.

How can I lower my risk of peripartum cardiomyopathy?

The best way to prevent peripartum cardiomyopathy is to do whatever you can to keep your heart healthy. While you can’t avoid all of your risk factors, you can manage others by:

• Eating heart-healthy foods.
• Getting regular physical activity.
• Avoiding tobacco products and alcohol.
• Working with your healthcare provider to manage conditions like overweight or obesity.
• Checking your blood pressure regularly and treating it if needed.

If you had heart failure during a previous pregnancy, talk with your healthcare provider about your risk factors if you’re planning to get pregnant again.

What can I expect if I have peripartum cardiomyopathy?

If you have peripartum cardiomyopathy, you’ll work closely with your healthcare provider and a team of specialists to monitor your health. If you’re still pregnant, your health team will monitor the condition of your fetus, too.

They’ll also create a plan for your type of delivery. If your heart failure is stable, your provider will likely prefer you have a vaginal delivery. But you may need an epidural, episiotomy or the use of forceps to make delivery easier.

If you have peripartum cardiomyopathy, you're more likely to have a Cesarean delivery (C-section) or preterm birth. It’s important to know your options and risks and talk about them with your provider.

After delivery, you’ll continue to work with your provider to manage your care and treatment. How you feel and how quickly you recover depend on many factors, but the most important factor is your left ventricular ejection fraction (LVEF). If your LVEF is less than 30% at diagnosis, you’re at a higher risk of serious complications.

Is peripartum cardiomyopathy permanent?

For some people, peripartum cardiomyopathy becomes permanent. For others, treatment can manage the symptoms and restore some or all heart function.

If your LVEF reaches at least 50% to 55%, a provider considers that a full recovery from peripartum cardiomyopathy. Most studies check this number six months after diagnosis. Recovery varies widely by region and ethnicity. Recovery rates in the U.S. range from 44% to 63% after six months, while recovery rates in some countries are just 21% to 36%.

One study found that 4 in 5 Black women don’t fully recover from peripartum cardiomyopathy, and 1 in 10 don’t survive.

You’ll have to monitor your health and work closely with your healthcare provider, even after your heart function returns to normal. That’s because 1 in 5 women who recover from peripartum cardiomyopathy have a heart failure relapse later. Also, even after your LVEF returns to a healthy level, you may still feel long-term effects like reduced exercise capacity.

What is the life expectancy of someone with peripartum cardiomyopathy?

Life expectancy with peripartum cardiomyopathy depends on your condition, how well the treatment works and many other factors related to your overall health. Your risk of dying is higher with a lower ejection fraction (LVEF). Also, the outlook isn’t as good for those who need medical devices to help their hearts function.

Researchers estimate the mortality rate is between 2.5% and 32% globally. The U.S. sits at the lower end with a mortality rate between 6% and 10%.

How do I take care of myself?

The most important thing you can do to take care of yourself is to visit your healthcare provider regularly. Follow your provider’s plan and call your provider to talk about any problems or changes you notice.

Some simple daily changes you can make may include:

• Lowering how much salt you eat. Find options to add flavor without salt.
• Weighing yourself to watch for fluid buildup. If you gain 3 to 4 pounds over a couple of days, call your provider.
• Quitting smoking. Ask your provider for helpful resources.
• Drinking less or avoiding alcohol completely.

If you’re breastfeeding, talk with your provider about any risks, like passing medicine to your baby through breast milk. Usually, the benefits of breastfeeding — like bonding with your baby — outweigh the risks. You may want to work with a post-pregnancy counselor to decide what’s best for you and your baby.

Peripartum cardiomyopathy and mental health

Peripartum cardiomyopathy can be a life-changing diagnosis. You may feel a range of emotions, all while you’re bonding with and caring for your baby. It’s a lot to handle at once.

Stress from peripartum cardiomyopathy raises your risk of developing mood disorders. More than 50% of people with peripartum cardiomyopathy develop generalized anxiety or anxiety over the condition of their heart.

While about 1 in 10 postpartum women in the U.S. have postpartum depression, about 1 in 3 women develop depression after a peripartum cardiomyopathy diagnosis.

If you feel upset, confused or overwhelmed, you’re not alone. If you aren’t already working with a counselor or therapist, ask your provider to recommend one.

When should I see my healthcare provider?

If you have any symptoms of peripartum cardiomyopathy, call your healthcare provider right away. You’ll have regular appointments during treatment. Once your heart is working well again (with a normal LVEF), you’ll need a provider to check your heart once a year or more often. This allows them to check to make sure the condition doesn’t return. If it does, they can catch it early with regular checkups.

When should I go to the emergency room?

If you have chest pain, heart palpitations, fainting or any new symptoms, call 911 (or your local emergency service number) or go to the ER.

What questions should I ask my doctor?

Questions to ask your healthcare provider may include:

• Can you treat me with medicine or do I need a medical device?
• Will I need a C-section?
• How often will I need follow-up appointments?

Can I get pregnant again if I have peripartum cardiomyopathy?

For many women, peripartum cardiomyopathy happens more than once. If you have it with one pregnancy, you’re more likely to have it again. About 1 in 3 women will develop it with a later pregnancy.

You can talk with your healthcare provider about whether it’s safe to have another pregnancy. If it’s too risky, you may want to explore options for birth control. You’ll need contraception that doesn’t contain estrogen because estrogen therapies raise your risk of blood clots and thrombosis.

If you do have another pregnancy, you’ll need close monitoring and regular checkups to catch any early signs of heart failure.