International Journal of Cardiology 186 (2015) 48–49

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Letter to the Editor

Noncompaction cardiomyopathy with apical aneurysm Jing Ping Sun b,1, Xianda Ni a,1, Xing Sheng Yang b, Cheuk-Man Yu b,⁎ a

Department of Ultrasonography, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, Zhejiang, China Institute of Vascular Medicine, Li Ka Shing Institute of Health and Sciences, and HEART Centre, Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, N.T., Hong Kong

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a r t i c l e

i n f o

Article history: Received 3 March 2015 Accepted 17 March 2015 Available online 18 March 2015 Keywords: Noncompaction cardiomyopathy Echocardiography Apical aneurysm

A 39 year-old asymptomatic woman was found incidentally of having an apical aneurysm by echocardiography. Her transthoracic echocardiographic examination revealed thickened left ventricular lateral wall with apical aneurysm and normal LV systolic function. There was excessively prominent trabeculations with sinusoids of blood flow in LV lateral and inferior wall (Fig. 1A, B; Videos 1 & 2). The left ventricular angiogram showed noncompaction myocardium (Fig. 1C). The cardiac magnetic resonance imaging confirmed that the thickness of the trabeculations was more than twice that of the underlying ventricular wall and LV apical aneurysm (Fig. 1D, E). Computed tomography with

Fig. 1. A. Parasternal short axis view showed excessively prominent trabeculations with sinusoids (arrows). B. Apical 2-chamber view indicated the apical aneurysm (arrow). C. Left ventricular angiogram showed noncompaction myocardium (arrows). D. The left ventricular short axis view of magnetic resonance imaging (MRI) showed that the thickness of the trabeculations is more than twice of the underlying ventricular wall (arrows). E. MRI revealed hyper delayed enhancement of LV apical aneurysm indicating fibrosis (arrows). F. Computed tomography: three dimensional reconstruction image showed apical aneurysm (arrow). ⁎ Corresponding author at: Institute of Vascular Medicine, Li Ka Shing Institute of Health and Sciences, and HEART Centre, Division of Cardiology, Department of Medicine and Therapeutics, Prince of Wales Hospital, The Chinese University of Hong Kong, Shatin, N.T., Hong Kong SAR. E-mail address: [email protected] (C.-M. Yu). 1 Co-first author.

http://dx.doi.org/10.1016/j.ijcard.2015.03.249 0167-5273/© 2015 Elsevier Ireland Ltd. All rights reserved.

J.P. Sun et al. / International Journal of Cardiology 186 (2015) 48–49

three dimensional reconstruction showed apical aneurysm. All of the above cardiac imaging studies were consistent with LV noncompaction cardiomyopathy complicated with apical aneurysm formation in this case. Discussion: Isolated left ventricular noncompaction is a congenital cardiomyopathy caused by a defect in endomyocardial morphogenesis [1]. Its clinical manifestations include heart failure, thromboembolism, ventricular tachyarrhythmia, and sudden death [2]. There were only a few cases about coexisting left ventricular aneurysm. We report a rare case of isolated left ventricular (LV) noncompaction associated with LV apical aneurysm. The echocardiographic criteria suggested by Jenni et al. [3] have become widely accepted. They are as follows: 1) thickened myocardium with a 2-layered structure consisting of a thin compacted epicardial layer [C] and a much thicker, noncompacted endocardial layer [N] or trabecular meshwork with deep endocardial spaces (N/C ratio N 2.0 at end-systole); 2) predominant location of the pathology in the mid-lateral, mid-inferior, and apical areas; 3) color Doppler evidence of deep intertrabecular recesses filled with blood from the LV cavity; and 4) absence of coexisting cardiac abnormalities (in isolated LV isolated noncompaction). The echocardiographic characteristics of our patient were consistent with the above criteria. Cardiac magnetic resonance imaging is increasingly recognized as an alternative modality for

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the noninvasive assessment of patients with noncompaction cardiomyopathy. Compared with traditional echocardiography, cardiac magnetic resonance imaging has less operator dependence, superior spatial resolution, and higher contrast between blood and myocardium, which can provide better delineation of the abnormal trabeculations in noncompaction cardiomyopathy patients. Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ijcard.2015.03.249. Conflict of interest None. References [1] T.K. Chin, J.K. Perloff, R.G. Williams, K. Jue, R. Mohrmann, Isolated noncompaction of left ventricular myocardium. A study of eight cases, Circulation 82 (1990) 507–513. [2] E.N. Oechslin, C.H. Attenhofer Jost, J.R. Rojas, P.A. Kaufmann, R. Jenni, Long-term follow-up of 34 adults with isolated left ventricular noncompaction: a distinct cardiomyopathy with poor prognosis, J. Am. Coll. Cardiol. 36 (2000) 493–500. [3] R. Jenni, E. Oechslin, J. Schneider, C. Attenhofer Jost, P.A. Kaufmann, Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy, Heart 86 (2001) 666–671.

Noncompaction cardiomyopathy with apical aneurysm.

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