Peripartum cardiomyopathy

Definition

Peripartum cardiomyopathy is a rare disorder in which a weakened heart is diagnosed within the final month of pregnancy or within 5 months after delivery.

Alternative Names

Cardiomyopathy - peripartum

Causes, incidence, and risk factors

Cardiomyopathy occurs when there is damage to the heart. As a result, the heart muscle becomes weak and cannot pump blood efficiently. Decreased heart function affects the lungs, liver, and other body systems.

Peripartum cardiomyopathy is a form of dilated cardiomyopathy in which no other cause of heart dysfunction (weakened heart) can be identified.

In the United States, peripartum cardiomyopathy complicates 1 in every 1,300 - 4,000 deliveries. It may occur in childbearing women of any age, but it is most common after age 30.

Risk factors include obesity, having a personal history of cardiac disorders such as myocarditis, use of certain medications, smoking, alcoholism, multiple pregnancies, being African American, and being malnourished.

Symptoms

• Fatigue
• Feeling of racing heart or skipping beats (palpitations)
• Increased night-time urination (nocturia)
• Shortness of breath with activity and when laying flat
• Swelling of the ankles

Signs and tests

During a physical examination, the physician will look for signs of fluid in the lungs by touching and tapping with the fingers. Listening to the chest with a stethoscope will reveal lung crackles, a rapid heart rate, or abnormal heart sounds.

The liver may be enlarged and neck veins may be swollen. Blood pressure may be low or may drop when the patient stands up.

Heart enlargement, congestion of the lungs or the veins in the lungs, decreased cardiac output, decreased movement or functioning of the heart, or heart failure may show on:

• Chest x-ray
• Chest CT scan
• Coronary angiography
• Echocardiogram
• Nuclear heart scan

A heart biopsy may be helpful in determining an underlying cause of cardiomyopathy. Many cases of peripartum cardiomyopathy seem to be related to myocarditis, which can be confirmed by a heart biopsy.

Treatment

The woman may need to stay in the hospital until acute symptoms subside.

Because the heart dysfunction is usually reversible, and the women are usually young, everything possible will be done to ensure survival.

This may include taking extreme measures such as:

• Use of a balloon heart pump (aortic counterpulsation balloon)
• Immunosuppressive therapy (such as medicines used to treat cancer or prevent rejection of a transplanted organ)
• Heart transplant if severe congestive heart failure persists

For most women, however, treatment focuses simply on relieving the symptoms. Some symptoms resolve on their own without treatment.

Medications include:

• Digitalis to strengthen the heart's pumping ability
• Diuretics (water pills) to remove excess fluid
• Low-dose beta-blockers

A low-salt diet may be recommended. Fluid may be restricted in some cases. Activities, including nursing the baby, may be limited when symptoms develop.

Daily weighing may be recommended. A weight gain of 3 or 4 pounds or more over 1 or 2 days may be a sign of fluid buildup.

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

Support Groups

Expectations (prognosis)

There are several possible outcomes in peripartum cardiomyopathy. Some women remain stable for long periods, while others get worse slowly.

Others get worse very quickly and may be candidates for a heart transplant. The death rate may be as high as 25 - 50%.

The outlook is good for women whose hearts returns to normal size after the baby is born. If the heart remains enlarged, future pregnancies may result in heart failure. It is not known how to predict who will recover and who will develop severe heart failure.

Women who develop peripartum cardiomyopathy are at high risk of developing the same problem with future pregnancies and should discuss contraception with their physician.

Complications

• Cardiac arrhythmias (can be deadly)
• Congestive heart failure
• Pulmonary emboli (blood clots in the lungs)

Calling your health care provider

Call your health care provider if you are currently pregnant or have recently delivered a baby and think you may have signs of cardiomyopathy.

Also seek medical attention if you develop chest pain, palpitations, faintness, or other new or unexplained symptoms.

Prevention

Eat a well-balanced, nutritious diet, exercise to increase cardiovascular fitness, and avoid cigarettes and alcohol. Your doctor may advise you to avoid getting pregnant again if you have had heart failure during a previous pregnancy.

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References

McKenna W. Diseases of the myocardium and endocardium. In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011:chap 60.

Warnes CA. Pregnancy and heart disease. In: Bonow RO, MannDL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 82.