LAUSR

Peripartum cardiomyopathy (PPCM) contributes to maternal death during or after pregnancy. Previously healthy women present with often severe heart failure during the last month of pregnancy or the first months postdelivery.1 Peripartum cardiomyopathy is a global disease that occurs in about 1 in 1000 pregnancies, with a mortality rate ranging from 5% to 30%. Previous analyses have pointed to a worse outcome of patients with PPCM of African ancestry. In this issue of JAMA Cardiology, using a retrospective analysis of the University of Pennsylvania Health System, Irizarry et al2 present the largest cohort of patients with PPCM to date, including 121 African American women.2 The investigators used stringent definitions for PPCM and excluded all patients with a potential other explanation for heart failure, such as a history of congenital heart disease, valvular heart disease, or receiving radiation treatment or chemotherapy. African American patients with PPCM were younger, received their diagnoses later during the postpartum period, and presented with significantly lower left ventricular ejection fraction. These results are not completely matched by the baseline characteristics of the recently published first 400 patients in the worldwide PPCM registry of the EURObservational Research Programme.3 Among these patients, despite the huge differences in sociodemographic factors among regions of the world, the baseline characteristics of patients with PPCM, such as an obstetric history and clinical presentation, were remarkably similar. The mode of presentation and mean age were also independent of ethnic and socioeconomic backgrounds.3 However, a detailed analysis regarding racial/ ethnic groups has yet to be performed in this registry. Even more troublesome in comparison with non–African American patients in the Pennsylvanian cohort with PPCM, African American patients also had a markedly delayed recovery and a much higher proportion did not recover to an ejection fraction of more than 50%, despite receiving similar treatment with drugs often prescribed for heart failure such as angiotensin-converting enzyme inhibitors and β-blockers (although it is unclear whether drug prescription resulted in similar drug intake among African American and non–African American patients, and there are no data reported on drug dosage). However, compared with the Sliwa cohort of African American patients from South Africa4 that reported a 12% mortality rate, and a recovery of ejection fraction in only 21%, outcomes regarding mortality rates and recovery were clearly better in the current US cohort. In fact, mortality rates and cardiac transplantats, as well as arrhythmia, were not different between African American and non–African American patients. The findings of Irizarry et al2 point to either a more severe form of PPCM in African American patients or to socioeconomic factors that are associated with limited access to timely and advanced medical care. Racial/ethnic disparities in health care, socioeconomics, and the prevalence of risk factors are established. Indeed, African American patients with PPCM in the current study had a higher prevalence of hypertension before pregnancy than non–African American patients, but a similar incidence of pregnancy-induced hypertension was observed. The current study adds to our understanding of PPCM as a clinical syndrome, not a distinct entity: it is likely that several peripartal heart failure entities are summarized under this diagnosis of exclusion with different pathophysiologies and potentially different treatment approaches (eg, a shared genetic predisposition has been described in peripartum and dilated cardiomyopathy, and 10% of patients with PPCM have truncating genetic variants in the gene that encodes the sarcomeric protein titin that were found both in African American and non–African American patients).5 Clearly, more research is needed to discern the genetic basis and the pathophysiological mechanisms that explain different disease entities that are summarized currently under the PPCM syndrome. Larger patient cohorts need to be investigated in detail and followed up long term. What are the implications of the current analysis for the diagnosis and treatment of PPCM? A high index of suspicion is necessary, especially in cases that do not present with acute heart failure but rather develop subtly over weeks with unspecific symptoms of cardiac congestion.1 African American women in the peripartum period must be carefully evaluated, as well as all women with risk factors for PPCM, such as pregnancy-associated hypertension, twin pregnancy, and cesarean delivery. N-terminal pro B-type natriuretic peptide is the only widely available marker for the efficient screening of peripartum heart failure, but it is not specific for PPCM and may be elevated in other conditions (eg, pulmonary embolism). Echocardiography is the gold standard for establishing the diagnosis.6 Special attention has to be devoted to high-risk patients with an ejection fraction of less than 35%, especially less than 25%, and/or cardiogenic shock. Given the worse outcomes for African American patients, they should be viewed as highrisk patients. In critically ill patients, catecholamine treatment should be avoided whenever possible given the specific adverse effect in patients with PPCM; early use of transient mechanical support to unload the left ventricle appears to be helpful.6 Given the high tendency of improvement among many patients with PPCM, heart transplants or permanent ventricuRelated article Poor Outcomes in Poor Patients? Invited Commentary