PPCM Management: 5 Things to Know

The incidence of adverse pregnancy outcomes, particularly peripartum cardiomyopathy (PPCM), is on the rise in the United States. Cardiovascular disease is the leading cause of maternal mortality and accounts for 25%-30% of all maternal deaths. One half to two thirds of maternal cardiovascular death is due to cardiomyopathy. Maternal risk factors, including African American descent, advanced maternal age > 34 years, multiple-gestation pregnancies, preeclampsia, hypertension, diabetes, and obesity, are linked to the increase in adverse outcomes. Moreover, diagnosis of PPCM is often delayed because signs and symptoms can mimic those of normal pregnancy.

Here are five things to know about PPCM diagnosis and management.

1. Although PPCM is frequently associated with hypertensive disorders of pregnancy, hypertension is not a diagnostic criterion of the condition.

The Heart Failure Association of the European Society of Cardiology (ESC) Working Group defines PPCM as “an idiopathic cardiomyopathy presenting with heart failure (HF) secondary to left ventricular (LV) systolic dysfunction [ejection fraction (EF) < 45%] towards the end of pregnancy or in the months following delivery, where no other cause of HF is found.” In the United States, the reported incidence of PPCM ranges from 1 in 1000 to 1 in 4000 births, with > 40% of cases occurring in African American women. As our knowledge of the pathophysiology of PPCM continues to evolve, an imbalance in placental hormones leading to microvascular dysfunction and often hypertensive pregnancy disorders may be inferred from current data. The average worldwide prevalence of preeclampsia is 3%-5%; however, the prevalence of preeclampsia in PPCM is approximately 22%-29.3%. In addition, 37% of women with PPCM experience other hypertensive pregnancy disorders.

Most patients with PPCM have no history of familial cardiomyopathy. Furthermore, PPCM does not recur in all subsequent pregnancies, supporting the theory that the condition may have an incompletely penetrant genetic origin (ie, the condition doesn’t occur in all gene carriers) or is the result of a “double hit” phenomenon of genetic predisposition plus disease-provoking risk factors.

2. Signs of pulmonary congestion should prompt rapid assessment of LV function to accurately diagnose PPCM.

Figure. Steps in diagnosing peripartum cardiomyopathy (modified). BNP = B-type natriuretic peptide; LVEF = left ventricular ejection fraction.

The rate-limiting step in the diagnosis of PPCM is recognizing the signs and symptoms of HF on physical examination and imaging studies (eg, radiography, echocardiography) (Figure). Although dyspnea, lower-extremity edema, and decreased exercise tolerance can be associated with normal pregnancy physiology, elevated B-type natriuretic peptide levels and pulmonary edema are not.

The diagnosis of PPCM is further complicated by overlapping features of preeclampsia. Pregnancy-induced hypertension with or without preeclampsia may trigger pulmonary edema, which can coexist with PPCM, however more than 90% of women with preeclampsia do not develop PPCM. Conversely, at least 50% of women with PPCM do not have preeclampsia (the prevalence of preeclampsia may be underestimated as it is often underreported or misclassified). Therefore, the diagnosis of PPCM should only be made in the setting of reduced LVEF.

Early recognition and treatment of signs and symptoms of HF can help prevent PPCM-associated death. In an Ohio Department of Health report of 186 pregnancy-related deaths (defined as having occurred during pregnancy or within 1 year of the end of pregnancy from a cause related to or aggravated by pregnancy or its management) from 2008 to 2016, 35% occurred while pregnant, 46% within 42 days of delivery, and 19% within 1 year postpartum. PPCM deaths were much more likely to occur in the postpartum period. Moreover, this report found that of the 86 pregnancy-related deaths that occurred from 2012 to 2016, 56% were considered preventable (chance to alter outcome was not determined before 2012).

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