European Journal of Echocardiography 2008 9(1):156-157; doi:10.1016/j.euje.2007.05.005
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2007. For permissions please email: journals.permissions@oxfordjournals.org
Comprehensive contrast and 3-dimensional echocardiographic imaging of left ventricular noncompaction cardiomyopathy
Jose Baez-Escudero,
Manu Pillai,
Vijay Nambi and
Hisham Dokainish*
Department of Medicine, Section of Cardiology, Baylor College of Medicine, 6620 Main Street, 11A.08, Houston, TX 77030, USA
Received 18 April 2007; accepted after revision 10 May 2007; online publish-ahead-of-print 9 July 2007.
* Corresponding author. Tel: +713 798 2608; fax: +713 798 2751. E-mail address: hishamd{at}bcm.tmc.edu
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Abstract
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A 62-year-old woman with a history of heart failure, hypertension
and stroke presented to hospital with dyspnea. Transthoracic
echocardiography revealed significant left ventricular dysfunction;
with contrast-enhanced 2- and 3-dimensional echocardiography,
extensive hypetrabeculation of the left ventricular myocardium
was visualized, leading to a diagnosis of noncompaction cardiomyopathy.
Apical thrombi were excluded with contrast imaging; however,
the patient was systemically anticoagulated owing to the presence
of noncompaction cardiomyopathy in the setting of prior stroke.
This case report demonstrates remarkable imaging of the left
ventricular myocardium achieved with contrast-enhanced 3-dimensional
echocardiography in the setting of noncompaction cardiomyopathy.
Keywords: 3-Dimensional echocardiography; Contrast echocardiography; Left ventricular noncompaction cardiomyopathy
A 62-year-old woman with a history of congestive heart failure,
hypertension, and ischaemic stroke presented with dyspnea. On
exam she was found to be significantly hypertensive (210/120
mmHg). Her electrocardiogram showed left ventricular hypertrophy.
Head computed tomography showed an old thalamic infarct. A 2-dimensional
transthoracic echocardiogram demonstrated a dilated left ventricle
(LV) with severely reduced systolic function. Significant LV
apical trabeculation was seen, with evidence of color Doppler
flow in the intertrabecular recesses, suggestive of LV noncompaction
cardiomyopathy (LVNC) (
Figure 1 a and
b;
Video 1 a, b).
LV cavity opacification was achieved using perflutren lipid
microspheres (Definity
®, Bristol-Myers Squibb, North Billerica,
MA, USA) injected intravenously (
Figure 2;
Video 2). Three-dimensional
echocardiography was performed with a General Electric Vivid
7 machine (General Electric, Milwaukee, WI, USA) (
Figure 3).
With contrast-enhanced 3-dimensional echocardiography, the segments
of noncompacted myocardium were more clearly visualized and
thrombus was excluded (
Figure 4;
Video 3a, b, c).
LVNC is an anomaly of endomyocardial morphogenesis, and is believed
to be an arrest in the compaction of the myocardial fibers.
1 LVNC can result in both systolic and diastolic dysfunction with
progression to clinical heart failure. The high incidence of
thromboembolic phenomena in LVNC likely results from formation
of local thrombi in the deep intertrabecular recesses. Based
on the reported frequency of embolic events, patients should
undergo systemic anticoagulation, independently of thrombi visualized
on echocardiography.
2,3 The major echocardiographic criterion
for a diagnosis of LVNC is a ratio of noncompacted-to-compacted
myocardium
2, measured at the site of maximal myocardial thickness.
4 In this case, a comprehensive echocardiographic approach, including
intravenous contrast and 3-dimensional imaging, provided remarkably
clear visualization of the hypertrabeculated myocardium, excluded
apical thrombi as a cause of the abnormal appearing LV apex,
and led to a diagnosis of LVNC. The patient was started on standard
medical heart failure therapy, as well as warfarin. No immediate
family members were available to perform familial screening.
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Supplementary material
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Supplementary data associated with this article can be found in the online version.
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References
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- Oechslin EN, Attenhofer Jost CH, Rojas JR, Kaufmann PA, Jenni R. Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis. J Am Coll Cardiol (2000) 36:493–500.[Abstract/Free Full Text]
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