Challenges in the diagnosis of peripartum cardiomyopathy: a case series

Abstract

Background: Peripartum cardiomyopathy (PPCM) is usually characterized by overt heart failure, but other clinical scenarios are possible, sometimes making the diagnosis challenging.

Case summary: We report a case series of four patients with PPCM. The first patient presented with acute heart failure due to left ventricular (LV) systolic dysfunction. Following medical treatment, LV function recovered completely at 1 month. The second patient had systemic and pulmonary thromboembolism, secondary to severe biventricular dysfunction with biventricular thrombi. The third patient presented with myocardial infarction with non-obstructed coronary arteries and evidence of an aneurysm of the mid-anterolateral LV wall. The fourth patient, diagnosed with PPCM 11 years earlier, presented with sustained ventricular tachycardia. A repeat cardiac magnetic resonance, compared to the previous one performed 11 years earlier, showed an enlarged LV aneurysm in the mid-LV anterolateral wall with worsened global LV function.

Discussion: Peripartum cardiomyopathy may have different clinical presentations. Attentive clinical evaluation and multimodality imaging can provide precise diagnostic and prognostic information.

Keywords: Cardiac magnetic resonance imaging; Case series; Echocardiography; Peripartum cardiomyopathy.

Figure 1

Transthoracic echocardiography at admission showing signs of congestion such as right pleural effusion (A, solid arrow) and left pulmonary comet tails (B, dashed arrow), reduced left ventricular function (C, left ventricular parasternal short-axis view in diastole and systole), and severe mitral regurgitation (D, left ventricular apical long-axis view); following adequate therapy transthoracic echocardiography demonstrated normalized left ventricular systolic function (E), and mild residual mitral regurgitation (F).

Figure 2

Transthoracic echocardiography apical views showing (A) biventricular apical thrombi (right ventricle, red arrow, A; left ventricle, green arrow, A and B) and a vacuolated mid-septal left ventricular thrombus (blue arrow, A and B). Colour-Doppler (C) showing secondary severe mitral regurgitation.

Figure 3

Transthoracic echocardiography in the same patient as in Figure 2 following an embolic systemic episode while on heparin therapy. (A) The left apical ventricular thrombus is no more evident and the mid-septal thrombus (arrow) looks bilobulated but not vacuolated. Computed tomography short-axis views shows bilateral pulmonary (B, solid arrows) and splenic (C, dashed arrow) embolism.

Figure 4

Cardiac magnetic resonance steady state free precession cine sequences showing right apical ventricular mass (A, red arrow), and bilobulated left ventricular mass (B, blue arrow). After gadolinium injection both ventricular masses look not perfused (C, red and blue arrows, right and left ventricle, respectively). Short-axis T1 weighted phase sensitive inversion recovery sequence acquired 10 min after gadolinium administration excluded late gadolinium enhancement (D).

Figure 5

Electrocardiogram at presentation (A) displaying rightward axis deviation and diffuse ST-segment abnormalities. Transthoracic echocardiography (B) parasternal short-axis view showing a thickened left ventricular wall, especially in the anterior segment (23 mm, arrow) with a moderately reduced left ventricular function. Electrocardiogram showing diffuse ST-segment depression with ST-segment elevation in aVL (C). Urgent cardiac magnetic resonance (T2-weighted short inversion time inversion recovery black-blood sequence) showing an extensive area of transmural oedema of the basal-mid anterior (D) and antero-septal (E) wall (arrows).

Figure 6

(A) Electrocardiogram in the same patient as in Figure 5 following delivery showing sinus bradycardia and normal ST-T segment. Transthoracic echocardiography showing focal thinning of the anterior wall (B, arrow). Repeat cardiac magnetic resonance short-axis views showing a focal left ventricular aneurysm (arrows) (C, T1 weighted black blood sequence), with discrete oedema (D, T2-weighted short inversion time inversion recovery black-blood sequence), and late gadolinium enhancement (E, T1 weighted phase sensitive inversion recovery sequence acquired 10 min after gadolinium injection) of the basal anterior wall.

Figure 7

Electrocardiogram showing (A) ventricular tachycardia with right bundle branch block inferior axis and QS morphology in leads I-aVL; following DC shock electrocardiogram (B) showed incomplete right bundle branch block, q waves in leads I-aVL and negative inferior T waves. Cardiac magnetic resonance steady state free precession sequence performed 11 years before admission (C) showing discrete mid-cavity anterior aneurysm (white arrows). Phase sensitive inversion recovery sequence acquired 10 min after gadolinium administration demonstrated transmural late gadolinium enhancement of the antero-septal, and anterior walls (D,E, white arrows) and epicardial late gadolinium enhancement of the basal inferior wall (E, blue arrow).

Figure 8

Cardiac magnetic resonance following ventricular tachycardia shows a remodelled left ventricular with a large left ventricular aneurysm of the mid-cavity anterior wall (A, steady state free precession sequence, white arrows) with worsened left ventricular function, and transmural late gadolinium enhancement of the antero-septal, and anterior walls (B, C, T1 weighted phase sensitive inversion recovery sequence acquired 10 min after gadolinium injection, white arrows) and of the basal inferior wall (C, blue arrow).