Peripartum Cardiomyopathy
GENERAL PRINCIPLES
Definition
Peripartum cardiomyopathy (PPCM) is defined as LV systolic dysfunction diagnosed in the last month of pregnancy up to 5 months postpartum, with an incidence 1 in 1000-4000 pregnancies in the United
States.88
Etiology
The exact etiology of PPCM remains unclear.
There is evidence to support viral, nutritional, and autoimmune contributors. Animal model and epidemiologic data suggest that vascular dysfunction and toxicity incited by the peripartum hormonal environment plays a central role in PPCM.89-91Risk Factors
Risk factors that predispose a woman to PPCM include advanced maternal age, multiparity, multiple pregnancies, preeclampsia, and gestational hypertension. There is a higher risk in African-American women, but this may be confounded by the higher prevalence of hypertension in this population.
DIAGNOSIS
Clinical Presentation
• Women with PPCM typically present with NYHA III and IV HF, although mild cases and sudden cardiac arrest also occur.
• Because dyspnea on exertion and lower extremity edema are common in late pregnancy, PPCM may be difficult to recognize. Cough, orthopnea, and paroxysmal nocturnal dyspnea are warning signs that PPCM may be present, as is the presence of a displaced apical impulse and a new MR murmur on examination.
Diagnostic Testing
ELECTROCARDIOGRAPHY
On ECG, LV hypertrophy is often present, as are ST-T-wave abnormalities.
IMAGING
Diagnosis requires an echocardiogram with a newly depressed EF and/or LV dilatation.
TREATMENT
Medications
• Therapy in the postpartum patient mirrors GDMT for HFrEF. Most agents are safe in lactation. Data for sacubitril/valsartan and ivabradine are lacking.
• During pregnancy, ACE/ARB/ARNI, MRA, and ivabradine should be avoided. β-Blockers, loop diuretics, hydralazine/nitrates, and digoxin are safe.
• In patients with thromboembolism, low molecular weight heparin is required, followed by warfarin after delivery.
OutcomeZPrognosis
• The prognosis in PPCM is better than that seen in other forms of nonischemic cardiomyopathy.
• The extent of ventricular recovery at 6 months after delivery can predict overall recovery, although
continued improvement has been seen up to 2-3 years after diagnosis.
• Subsequent pregnancies in patients with PPCM may be associated with significant deterioration in LV function and can even result in death, particularly in women who do not recover normal LV function after the first insult. Women who do not recover LV function should be encouraged to consider foregoing future pregnancy.