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Peripartum cardiomyopathy
Srinivas Murali, MD; Marie R. Baldisseri, MD

Objective: To provide a review of the cardiac and obstetrical literature regarding the development of peripartum cardiomyopathy and, in particular, to examine risk factors, incidence, diagnosis, prognosis, and evidence-based treatment modalities. Design: An extensive review of the current literature. Results: Peripartumcardiomyopathy is a cardiomyopathy of unknown cause that occurs in pregnant females, most commonly in the early postpartum period. It shares many clinical characteristics with idiopathic dilated cardiomyopathy but occurs at a younger age and is associated with a better prognosis. Diagnosis is based upon the clinical presentation of congestive heart failure and objective evidence of left ventricularsystolic dysfunction. Conventional pharmacologic therapy for congestive heart failure, such as diuretics, digoxin, angiotensin-converting enzyme inhibitors, angiotensin-receptor blockers, and -adrenergic blockers, are routinely used and are quite effective. For those patients who remain refractory to conventional pharmacologic therapy, cardiac

transplantation and mechanical circulatory supportare viable options. Conclusion: Mortality rates in peripartum cardiomyopathy have decreased, and this is most likely related to advances over the past 5 yrs in medical therapy for heart failure. Aggressive use of implantable defibrillators has significantly reduced the risk of sudden death in these patients. For >50% of peripartum cardiomyopathy patients, left ventricular function normalizes withpharmacologic therapy. However, subsequent pregnancies almost always are associated with recurrence of left ventricular systolic dysfunction. (Crit Care Med 2005; 33[Suppl.]:S340 –S346) KEY WORDS: dilated cardiomyopathy; acute inflammatory myocarditis; endomyocardial biopsy; gestational hypertension; twin pregnancies; left ventricular systolic dysfunction; implantable defibrillators;angiotensin-converting enzyme inhibitors; mechanical circulatory support; cardiac transplantation

ardiomyopathy associated with pregnancy was first described in 1937 (1). Peripartum cardiomyopathy is now defined as cardiomyopathy that develops in the last gestational month of pregnancy or in the first 5 months after delivery, with no identifiable cause for heart failure in the absence of heart disease (2). Itoccurs in 1 in 3,000 to 1 in 15,000 live deliveries in the United States, but its prevalence is much higher in Africa (1 in 3,000) and Haiti (1 in 350) (3–5). The natural history of peripartum cardiomyopathy is variable and its clinical presentation quite heterogeneous (6). It occurs more frequently in older women, obese females, and multiparous mothers having twin pregnancies (7). Other risk factorsinclude preeclampsia and severe hypertension during pregnancy. Patients frequently present with systemic and pul-

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From the University of Pittsburgh School of Medicine, Clinical Services of the Heart Failure Network, and Pulmonary Hypertension Program (SM); and Department of Critical Care Medicine, University of Pittsburgh School of Medicine (MRB), Pittsburgh, PA. Copyright © 2005 by theSociety of Critical Care Medicine and Lippincott Williams & Wilkins DOI: 10.1097/01.CCM.0000183500.47273.8E

monary congestion, low cardiac output, cardiac arrhythmias, and systemic and pulmonary embolization (8). Less frequently, cardiomyopathy can present for the first time earlier in pregnancy. There are, however, a few distinct clinical differences between patients with peripartum cardiomyopathyand those with cardiomyopathy diagnosed earlier in pregnancy. This includes a higher frequency of twin pregnancies and a shorter duration of pregnancy in patients who develop cardiomyopathy early in pregnancy. Left ventricular ejection fraction, though, improves similarly in the two groups (mean SE, 23 28 months). In over half (54%) of these patients, normal left ventricular function (ejection...