Cardiomyopathy - peripartum

Alternative names
Peripartum cardiomyopathy

Definition

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

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

Causes, incidence, and risk factors

Peripartum cardiomyopathy is a form of dilated cardiomyopathy without other identifiable causes of heart dysfunction (weakened heart).

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

Other risk factors include obesity, having a personal history of cardiac disorders (such as myocarditis), use of certain medications, smoking, alcoholism, multiple pregnancy, being African-American, and being malnourished.

Symptoms

     
  • Shortness of breath       o on exertion       o when lying flat (orthopnea)  
  • Fatigue  
  • Swelling of the ankles  
  • Increased frequency of night-time urination (nocturia)  
  • Feeling of racing or skipping of the heart (palpitations)

Signs and tests

During a physical examination, the physician will look for signs of fluid in the lungs by touch and tapping with the fingers. Listening to the chest with a stethoscope reveals 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 rising to a standing position.

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:

     
  • Echocardiogram  
  • Chest x-ray  
  • Chest CT scan  
  • Coronary angiography  
  • Nuclear heart scan (MUGA, RNV)

An ECG can show enlargement of the heart; problems with the electrical part of the heart, called conduction disturbances; and arrhythmias, such as atrial fibrillation, ventricular tachycardia, or an ectopic heartbeat (premature or abnormal beat). A heart biopsy may be helpful to determine an underlying cause of the cardiomyopathy.

Many cases of peripartum cardiomyopathy seem to be related to myocarditis, confirmed by the heart biopsy. Lab tests may vary.

Treatment

Hospitalization may be required until acute symptoms subside. Because the heart dysfunction is usually reversible, and because patients are usually young, all means necessary are applied to ensure survival.

This may include extreme measures such as using an aortic counterpulsation balloon, the use of immunosuppressive therapy (i.e., the type of therapy used to treat cancer or to prevent rejection of transplanted organs), or a heart transplant.

For most women, however, treatment focuses simply on relieving the symptoms. Some women’s symptoms resolve on their own (spontaneously).

Medications include diuretics (water pills) to remove excess fluid, vasodilators called ACE-inhibitors to improve cardiac outflow, digitalis to strengthen cardiac outflow, and low-dose beta-blockers. A low-salt diet may be recommended. Fluid may be restricted in some cases. Activity may be restricted when symptoms develop, including nursing the baby.

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

If you smoke or drink alcohol, stop. These habits may make the symptoms worse.

Expectations (prognosis)

There are several possibilities in peripartum cardiomyopathy. Some women remain stable for long periods, some deteriorate gradually, and some deteriorate rapidly and may be candidates for a heart transplant. The death rate may be as high as 25-50%.

In women whose heart returns to normal size after the postpartum period, the prognosis is good. In contrast, if the heart remains dilated (enlarged), future pregnancy may result in progression of the heart failure. It is not known how to predict who will recover and who will develop severe heart failure and need extreme measures.

Complications

Calling your health care provider

     
  • If you are currently or recently pregnant and symptoms indicate cardiomyopathy may be developing.  
  • Chest pain, palpitations, faintness, or other new or unexplained symptoms develop.

Prevention
Modify controllable risk factors. Eat a generally well-balanced, nutritious diet; exercise to increase cardiovascular fitness; and avoid smoking and consumption of alcohol.

Johns Hopkins patient information

Last revised: December 8, 2012
by Brenda A. Kuper, M.D.

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