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Case Reports
. 2021 Mar 22;5(3):ytab088.
doi: 10.1093/ehjcr/ytab088. eCollection 2021 Mar.

Cardiomyopathy in the peripartum period due to left ventricular non-compaction and association with Ebstein's anomaly: a case report

Emily Evans  1 , Mandy McDonald  1 , Dawn L Adamson  1 , Jamal Nasir Khan  1
Affiliations
Case Reports

Cardiomyopathy in the peripartum period due to left ventricular non-compaction and association with Ebstein's anomaly: a case report

Emily Evans et al. Eur Heart J Case Rep. .

Abstract

Background: Left ventricular non-compaction (LVNC) cardiomyopathy is a persistence of abnormal foetal myocardium and is a rare cause of cardiomyopathy in the peripartum period. Unlike other causes of peripartum cardiomyopathy which typically improve, LVNC has significant long-term personal and family implications and needs lifelong follow-up.

Case summary: We describe a unique case of a 30-year-old woman who developed cardiomyopathy in the peripartum period which was revealed on cardiovascular magnetic resonance imaging to be due to occult LVNC. Our patient also had Ebstein's anomaly, which is a known LVNC association.

Discussion: Cardiomyopathy in the peripartum period can be a decompensation of previously asymptomatic subclinical cardiomyopathy. It is important to assess for LVNC in patients presenting with this. Cardiovascular magnetic resonance imaging is the gold-standard imaging modality and allows accurate diagnosis of LVNC, associated structural complications and rare associations such as Ebstein's anomaly. Left ventricular non-compaction is irreversible and has implications for patients and their family members.

Keywords: Cardiovascular magnetic resonance; Case report; Ebstein’s anomaly; Echocardiography; Left ventricular non-compaction; Peripartum cardiomyopathy.

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Figures

Figure 1
Figure 1
Electrocardiogram showing left ventricular hypertrophy.
Figure 2
Figure 2
Three-chamber echocardiographic view showing dilated left ventricle [LVID (end-diastolic dimension) 6.1 cm, normal reference 5.3< cm]. LVID, left ventricular internal dimension.
Figure 3
Figure 3
Four-chamber echocardiographic demonstrating prominent left ventricular trabeculations. Left: Dilated left ventricle with arrowed lines illustrating deep myocardial recesses (pathological trabeculations). Right: Corresponding image with colour Doppler overlay illustrating blood flow into deep recesses. LV, left ventricle; RV, right ventricle.
Figure 4
Figure 4
Cardiovascular magnetic resonance imaging: four-chamber cine still image demonstrating dilated left ventricle with (A) non-compacted myocardium [end-diastolic non-compacted to compacted ratio >2.3 (5), asterisk], (B) non-compacted and underdeveloped anterolateral papillary muscle (white arrow), and (C) increased tricuspid valve to mitral valve offset of 11 mm/m2 (normal range <8 mm/m2) due to apical displacement of septal tricuspid valve leaflet.
Figure 5
Figure 5
Four-chamber image from baseline echocardiogram demonstrating that due to LV dilatation the basal diameter of the ventricle was enlarged on four-chamber imaging reducing the end-diastolic offset between the tricuspid and mitral valves to 12.7 mm (6.9 mm/m2), making it extremely challenging to appreciate the increased offset and hence Ebstein’s anomaly.
Figure 6
Figure 6
Cardiovascular magnetic resonance imaging: Native T1 mapping showing mildly elevated global T1 values (1031 ms at 1.5T) globally in keeping with global myocardial fibrosis (this image shows single region of interest).
None
See this image and copyright information in PMC

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