Peripartum cardiomyopathy: disease or syndrome?

Abstract

Peripartum cardiomyopathy (PPCM) is a rare form of pregnancy-associated heart failure and is considered to be a diagnosis of exclusion. There are many hypotheses on the aetiology of PPCM; however, the exact pathophysiological mechanism remains unknown. It shows many resemblances to other conditions, such as familial dilated cardiomyopathy or myocarditis, and therefore it can be hard to make a definite diagnosis. We describe four cases of peripartum-onset heart failure in women who were suspected of having PPCM. We discuss the differential diagnosis, pathophysiological mechanisms and various diagnostic modalities.

Keywords: familial cardiomyopathies; heart failure; idiopathic dilated cardiomyopathy; pregnancy.

Figure 1

ECG of patient A at admission. There was a noted sinus tachycardia with low voltages in the extremity leads and a QS pattern in V1–V3. aVR, augmented vector right; aVL, augmented vector left; aVF, augmented vector foot.

Figure 2

ECG of patient B at diagnosis. Sinus rhythm with notched high-amplitude P-waves, QRS of 120 ms, negative repolarisation in the inferior and lateral leads, and QS complex in V1. aVR, augmented vector right; aVL, augmented vector left; aVF, augmented vector foot.

Figure 3

(A) Parasternal long axis on echocardiogram of patient B at diagnosis. The LVEDD was 76 mm. (B) Four-chamber apical view of patient B at diagnosis. Biventricular dilatation with severely impaired right and left ventricular function. Severe mitral regurgitation. BPM, beats per minute; LVEDD, left ventricular end diastolic diameter.

Figure 4

Four-chamber MRI view of patient C. Distinct tubular dilatation of the right ventricle (RV) with dyskinesia, impaired RV ejection fraction (34%) and elevated RV end-diastolic volume index (132.4 mL/min). Also oedema and late enhancement in the wall of the right ventricle.

Figure 5

Flow chart for management of peripartum onset heart failure. ARVC, arrhythmogenic right ventricular cardiomyopathy; DCM, dilated cardiomyopathy; PPCM, peripartum cardiomyopathy; HCM, hypertrophic cardiomyopathy; Hb, hemoglobin; Ht, hematocrit; NYHA, New York Heart Association.