Cardiac disease in pregnancy and peripartum cardiomyopathy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Anjan K. Chakrabarti, M.D. [2]

Synonyms and Keywords: PPCM; PPCMP

Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy that is defined as a deterioration in cardiac function presenting between the last month of gestation and up to six months post-partum. The etiology of postpartum cardiomyopathy is unknown. Reported prevalence of postpartum cardiomyopathy in United States is estimated to be 1 case per 1300-15,000 live births. Treatment for the disease is similar to treatment for congestive heart failure. Delivery is the recommeded overall treatment to decrease the volume load, improve ventricular function and simplify the medical management of these patients.

Peripartum cardiomyopathy is defined as:

• Heart failure within last month of pregnancy or five months postpartum
• Absence of prior heart disease
• No determinable cause
• Strict echocardiographic indication of left ventricular dysfunction:

• Ejection fraction <45% and/or
• Fractional shortening <30%
• End-diastolic dimension >2.7 cm/m2 BSA (body surface area)

The etiology of postpartum cardiomyopathy is largely unknown. It has been postulated that a defective antioxidant mechanism may contribute. There are elevated levels of cathepsin D and an increase in total prolactin and angiostatic 16kDa prolactin. These molecules promote:

• Apoptosis
• Inhibit endothelial cell proliferation
• Increase vasoconstriction
• Impair myocyte function

The potential role of prolactin forms the molecular basis of treatment with bromocriptine.

As with other forms of dilated cardiomyopathy, PPCM involves decrease of the left ventricular ejection fraction with associated congestive heart failure and increased risk of atrial and ventricular arrhythmias and even sudden cardiac death.

• Accelerated HTN
• Infection/sepsis
• Diastolic dysfunction
• High output state of pregnancy

• Estimates of incidence 1/1,300-15,000.

PPCM is more common among women with:

• Prior PPCM
• Multiple pregnancies
• African decent, Haitian descent
• History of toxemia
• Long-term tocolytic use
• Age >30
• Twin Pregnancy

• Some patients will have a relapse of PPCM after a partial or full recovery.
• The course of peripartum CMP is variable.
• PPCM is a leading cause of pregnancy related death in the United States.
• Mortality 25-50% (half deaths in first 3 months).
• 50% improve within 6 months.
• 20% to 40% of patients normalize their LVEF.
• If the LVEF remains poor at 6 months, then mortality is increased.
• PPCM is a risk factor for recurrence with subsequent pregnancies.
• Favorable outcomes with cardiac transplantation.

Signs and symptoms are similar to those of normal pregnancy

• Delivery is the recommeded overall treatment to decrease the volume load, improve ventricular function and simplify the medical management of these patients.

Pharmacotherapy should be discontinued or tapered gradually based upon repeated echocardiographic analyses. Some patients will have a relapse of PPCM after a partial or full recovery.

• Bromocriptine blocks release of prolactin (see pathophysiology regarding role of prolactin). This therapy has been shown to increase the left ventricular ejection fraction (LVEF) versus standard treatment. As a side effect, this drug will inhibit lactation.
• Digoxin and diuretics are pregnancy category Class C agents and can be used if the benefits outweigh the risks.
• ACE inhibitors absolutely contraindicated prepartum (hydralazine drug of choice).
• Anticoagulation recommended (heparin prepartum and coumadin postpartum) if the left ventricular ejection fraction (LVEF) is <35%.

• Ventricular assist device or cardiac transplantation can be performed in patients with severe refractory congestive heart failure.

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