Summary: A 38-year-old postpartum female presented with fatigue, shortness of
breath, and dyspnea on exertion, 3 months following an uneventful
spontaneous vaginal delivery. Prior medical and family histories were
unremarkable. The patient had normal blood pressure, electrocardiogram,
cardiac enzymes, C-reactive protein, lipid profile, and viral titers.
Echocardiography demonstrated moderately depressed global
Postpartum cardiomyopathy (PPCM)
Cardiac magnetic resonance imaging (MRI) was subsequently performed.
Short-axis CINE steady-state free-precession (SSFP)imaging, acquired
following perfusion imaging, confirmed moderately depressed
left-ventricular function, with an ejection fraction of40%. As shown
with select short-axis diastolic (Figure 1) and systolic (Figure 1)
images, the shortening fraction was 15%.
Short-axis T1 dark-blood
imaging (Figure 2) through the basilar left ventricle (LV) revealed
mildly heterogeneous myocardial signal intensity. Corresponding
short-axis T1-weighted inversion recovery (IR) imaging (Figure 2)
acquired 10 min after perfusion imaging demonstrated abnormal
subepicardial delayed hyperenhancement (DHE) involving the
anterior-lateral, inferior-lateral, inferior, and septal myocardium.
Two-, 3-, and 4-chamber (Figure 3) delayed IR sequences confirmed
regions of abnormal myocardial DHE involving the subepicardial mid and
basilar inferior wall (A), anterior septum (B, long arrows), basilar
inferior-lateral wall (B, short arrow), mid and basilar inferior septum
(C, long arrows), and basilar anterior-lateral wall (C, short arrow).
retrospective analysis, note early myocardial retained Gadolinium in
the short axis CINE SSFP acquisition (Figure 1), corresponding to
regions of abnormal DHE.
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Postpartum cardiomyopathy (PPCM) is a rare idiopathic cardiomyopathy
resulting in left-ventricular (LV) dysfunction and heart failure,
presenting in late pregnancy or in the first few months following
delivery. LV ejection fraction is typically reduced to below 45%.
the United States, the incidence ranges between 1 in 2500 to 4000. The
majority of patients (approximately 75%) present within the first 4
postpartum months. PPCM is postulated to arise from oxidative stress and
elevated levels of the prolactin-cleaving protease cathepsin D in
cardiomyocytes, in the setting of elevated prolactin (eg, nursing),
resulting in a subprolactin fragment. This fragment promotes
vasoconstriction and induces endothelial apoptosis, leading to impaired
cardiomyocyte function. Contributing causes may include an inflammatory
process, an abnormal autoimmune process, viral infections,
cardiovascular disease risk factors (eg, hypertension, diabetes, tobacco
use), and pregnancy risk factors (eg, age, number of pregnancies).
with the patient in this case description, clinical presentation is one
of heart failure. Symptoms may include shortness of breath, persistent
cough, dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea,
chest pain, palpitations, dizziness, and abdominal discomfort(eg,
hepatic congestion). Physical examination may reveal peripheral edema,
elevated jugular venous pressure, rales, tachycardia, s3 or s4gallop, a
mitral regurgitant murmur, or a pericardial friction rub.
standard initial evaluation of clinically suspected PPCM includes chest
radiography, electrocardiography (ECG), laboratory analysis, and
echocardiography. Detailed diagnostic evaluation is essential to assess
for alternative cardiac (eg, hypertensive cardiomyopathy,valvular heart
disease) and noncardiac (eg, pulmonary embolism) etiologies, as PPCM is a
diagnosis of exclusion. Cardiac MRI with a perfusion and viability
protocol is an invaluable tool to characterize the myocardium, to
quantify cardiac chamber volumes and ventricular function, and thereby,
to more definitively diagnosis PPCM.
Key MRI findings for PPCM include global depressed function and a
nonvascular territory pattern of DHE (as seen with other inflammatory
cardiomyopathies). DHE will be found in the subepicardial myocardium,
corresponding to regions of myocardium infiltrated with cellular
lymphocytes and containing a variable extent of myocyte edema, necrosis,
fibrosis, or a combination thereof, depending upon the time course of
- Sliwa K, Hilfiker-Kleiner D, Petrie MC, et al. Current
state of knowledge on etiology, diagnosis, management, and therapy of
peripartum cardiomyopathy: A position statement from the Heart Failure
Association of the European Society of Cardiology Working Group on
peripartum Cardiomyopathy European Journal of Heart Failure. Eur J Heart Fail. 2010;12:767–778.
- Kawano H, Tsuneto A, Koide Y, et al. Magnetic resonance imaging in a patient with peripartum cardiomyopathy. Intern Med. 2008;47:97-102.