Cardiac disease in pregnancy and peripartum cardiomyopathy



Synonyms and Keywords: PPCM;

Overview
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 five 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.

Definition
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)

Common Mimickers

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

Demographics

 * Estimates of incidence 1/1300-15000. Previous studies likely overestimated
 * More common in women with:
 * Multiple pregnancies,
 * African decent,
 * h/o toxemia,
 * Long-term tocolytic use,
 * Age >30,
 * Twin Pregnancy.

Cause
The etiology of postpartum cardiomyopathy is unknown. 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.

History and Symptoms
Signs and symptoms are similar to those of normal pregnancy

Treatment

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

Pharmacotherapy:

 * Digoxin and diuretics are Class C recommendation.
 * ACE inhibitors absolutely contraindicated prepartum (hydralazine drug of choice).
 * Anticoagulation recommended (heparin prepartum and coumadin postpartum).

Prognosis

 * Mortality 25-50% (half deaths in first 3 months).
 * Remainder stable/recover within 6 months.
 * Can recur with subsequent pregnancies.
 * Favorable outcomes with cardiac transplantation.