Peripartum/postpartum cardiomyopathy is a dilated cardiomyopathy that may occur in the last trimester of pregnancy through the first several months postpartum.
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Epidemiology
The estimated incidence in the United States ranges from one in 900 to one in 4000 live births, with an increased incidence among mothers of advanced maternal age and in pregnancies with multiple gestations. A history of pre-eclampsia also appears to confer an increased risk, as do traditional cardiovascular risk factors.
Clinical presentation
Patients present with a clinical picture consistent with congestive hemodynamics, most commonly within a month after delivery:
- exertional dyspnea
- orthopnea
- jugular venous distension
- pulmonary rales
- displaced apical impulse
Pathology
The pathogenesis of post-partum cardiomyopathy is uncertain, with genetic factors, sympathetic tone, hormones, and malnutrition all suggested as contributing factors to its development.
Radiographic features
Plain radiograph
- chest radiograph demonstrates cardiac silhouette enlargement
- appearance is indistinguishable from other forms of dilated cardiomyopathy and the diagnosis is suggested by the patient history
- findings of pulmonary edema are dependent on the amount of systolic dysfunction
Echocardiography
Transthoracic echocardiography is often utilized as an initial screening test, which will frequently demonstrate the following:
-
left ventricular ejection fraction <45%
- alternatively, LV fractional shortening <25%
- non-regional (global) pattern of systolic dysfunction
- increased left ventricular volumes
- an elevated end-diastolic internal diameter often used as a surrogate measure (LVIDd >5.3 cm)
- mitral annular dilation may result in functional mitral regurgitation with a central regurgitant jet
- may also involve right ventricular hypokinesis and dilation
MRI
- cine SSFP sequences can demonstrate systolic dysfunction, similarly to echocardiography
- delayed enhancement in the left ventricular mid-myocardium has been described in the anterior and anterolateral wall 2
Treatment and prognosis
Most patients with treatment will recover, which typically occurs 3-6 months post-partum. Treatment is principally pharmacological (standard heart failure therapy, dopamine D2 receptor agonists and anticoagulation). The prognosis is better than in other forms of dilated cardiomyopathy, but progressive deterioration of systolic function may occur. The mortality rate is estimated at ~15% (range 4-28%) with significant regional variation. A heart transplant may become necessary 5,6.
Complications
- congestive heart failure
- ventricular dysrhythmias
- ischemic stroke
Differential diagnosis
-
congenital heart disease
- patients with previously asymptomatic lesions, particularly a patent foramen ovale or an atrial septal defect, are commonly diagnosed during pregnancy 4
-
dilated cardiomyopathy
- the hemodynamic milieu of pregnancy may result in decompensation of an already dysfunctional left ventricle
- results in elevated end-diastolic pressures and symptomatic heart failure
Practical points
Gadolinium contrast is contraindicated in pregnant patients and caution should be exercised in considering its use before delivery.
The necessary information for a presumptive diagnosis will often be possible without gadolinium contrast. Contrast may be useful for evaluation of therapeutic response postpartum, however.