CHAPTER SUMMARY AND CENTRAL ILLUSTRATION
This chapter reviews the epidemiology, pathophysiology, and management of peripartum cardiomyopathy (PPCM), an idiopathic cardiomyopathy presenting with heart failure due to severe left ventricular systolic dysfunction. Presentation is usually during the first month after delivery but can be during the second or third trimester of pregnancy or a few months postpartum (see Fuster and Hurst’s Central Illustration). The incidence of PPCM in the United States is approximately 1 in 3000 live births and is significantly higher in Black women than in White women. The incidence worldwide varies and is higher in Africa and Haiti than in Europe and the United States. Risk factors for the condition include advanced age, preeclampsia, Black ethnicity, and multifetal pregnancy. Normalization of left ventricular function occurs in >50% of women in the United States, mostly within 2 to 6 months after diagnosis. Reported recovery rates in other countries vary. Although many patients do improve, PPCM can be associated with important and lasting complications. Moreover, subsequent pregnancy in women with a history of PPCM can be associated with relapse with reduced ejection fraction and worsening of symptoms; this is more likely in women with persistent left ventricular dysfunction prior to their subsequent pregnancy but occasionally occurs in women with recovered left ventricular function.
eFig 51-01 Chapter 51: Peripartum Cardiomyopathy
Peripartum cardiomyopathy (PPCM) is a pregnancy-associated myocardial disease, reported to occur in different parts of the world.1–3 PPCM is an idiopathic cardiomyopathy presenting with heart failure (HF) secondary to LV systolic dysfunction toward the end of pregnancy or in the months following delivery, where no other cause of HF is found.2–4 PPCM is therefore a diagnosis of exclusion, and other causes of cardiac dysfunction should be ruled out. At the same time, however, transient and unexpected depression of LV function typical to PPCM has been described in women with other forms of heart disease.5
INCIDENCE AND EPIDEMIOLOGY
The incidence of PPCM varies widely, between ~1:100 and 1:300 in Africa and Haiti, respectively, to an average of 1:20,000 live births in Japan.2 Incidence of PPCM in the United States has been reported to range from ~1 in 1000 to 1 in 4000 live births6,7 with a significantly higher incidence in Black women.8,9 Multiple studies from the United States also reported a more severe disease and worse outcomes in Black women with PPCM, which could be related to racial differences due to genetic predisposition and environmental difference.10,11 PPCM incidence in the United States is increasing most likely due to older maternal age, an increase in the rate of multifetal pregnancies, and an increased recognition of the disease.5
ETIOLOGY AND PATHOPHYSIOLOGY