In the latest Case Record of the Massachusetts General Hospital, a 40-year-old woman was admitted to this hospital 10 days post partum because of dyspnea and hypoxemia associated with leg edema and blood-tinged sputum. Diagnostic procedures were performed.
Causes of dyspnea and hypoxemia in the peripartum period are predominantly of pulmonary or cardiovascular origin, the latter generally associated with pulmonary edema. The peripartum period predisposes women to a number of pathologic conditions, including pulmonary embolism, amniotic-fluid embolism, infection, aspiration, preeclampsia, and peripartum cardiomyopathy.
• What are the clinical manifestations of an amniotic fluid embolism?
Amniotic-fluid embolism is a rare but catastrophic complication of pregnancy or labor. Patients with amniotic-fluid embolism usually present with cardiorespiratory collapse; this is typically accompanied by disseminated intravascular coagulation and systemic inflammatory responses. Delayed manifestation of amniotic-fluid embolism beyond 48 hours after delivery is extremely rare.
• What is the epidemiology and what are the clinical manifestations of peripartum cardiomyopathy?
Peripartum cardiomyopathy is characterized by congestive heart failure and left ventricular systolic dysfunction toward the end of pregnancy or in the months after delivery, in the absence of other identifiable causes of cardiac disease. The ejection fraction on echocardiography is nearly always less than 45%. More than 90% of cases present in the first weeks post partum. The incidence varies from 1 in 300 live births to 1 in 3000 live births; two “hot spots” are Haiti and Nigeria. Peripartum cardiomyopathy is most common in women of African descent but is seen throughout the world. The manifestation is similar to that of other cardiomyopathies, including such signs and symptoms of venous congestion as dyspnea, orthopnea, edema, and, in extreme cases, hypoxemia.
Morning Report Questions
Q: What are the principles of management of peripartum cardiomyopathy?
A: As with other cardiomyopathies, management of peripartum cardiomyopathy should focus on reducing preload and afterload and interrupting the maladaptive neurohormonal response to systolic heart failure. Diuretic agents and itrates are the treatments of choice for volume overload, although caution is required with the use of these agents before delivery. Angiotensin-converting-enzyme inhibitors and angiotensin II-receptor blockers could be administered but would be contraindicated if the patient were still pregnant. In the setting of a low ejection fraction and in light of the hypercoagulability of the puerperium, anticoagulation may be advised for the prevention of systemic embolism. Implantation of an automatic implantable cardioverter-defibrillator, to prevent death from arrhythmia, is relatively contraindicated, because systolic function frequently recovers. If the patient were pregnant, neither premature discontinuation of pregnancy nor delivery by cesarean section would be indicated.
Q: What is the prognosis of peripartum cardiomyopathy?
A: As many as 50% of women with peripartum cardiomyopathy eventually recover cardiac function, but 25% have progression to advanced heart failure, which often leads to cardiac transplantation or death. A lower ejection fraction at presentation predicts a worse outcome and delayed recovery. Peripartum cardiomyopathy usually recurs in subsequent pregnancies, so decisions about repeat pregnancies need to be considered on an individual basis.