Journal of the American College of Cardiology

2014 by the American College of Cardiology Foundation

Published by Elsevier Inc.

Vol. 63, No. 16, 2014

ISSN 0735-1097/$36.00
http://dx.doi.org/10.1016/j.jacc.2013.11.063

IMAGES IN CARDIOLOGY

Clinical Spectrum of Isolated

Left Ventricular Noncompaction
Thromboembolic Events, Malignant Left Ventricular Arrhythmias,
and Refractory Heart Failure
Daniele Muser, MD,*y Gaetano Nucifora, MD,* Enrico Gianfagna, MS,z Daisy Pavoni, MD,*
Luca Rebellato, MD,* Domenico Facchin, MD,* Elisabetta Daleffe, MD,* Alessandro Proclemer, MD*
Udine, Trieste, and Rome, Italy

From the *Cardiothoracic

Department, University
Hospital Santa Maria della
Misericordia, Udine, Italy;
yPostgraduate School of
Cardiovascular Sciences,
University of Trieste, Trieste,
Italy; and the zSchool of
Medicine, Catholic University
of the Sacred Heart,
Rome, Italy.
Manuscript received
September 11, 2013;
revised manuscript received
October 31, 2013,
accepted December 12, 2013.

17-year-old boy presented with cardioembolic stroke. Echocardiography and cardiac

magnetic resonance imaging (MRI) (A and B: arrows indicate left ventricular trabecular
meshwork with deep intertrabecular spaces; Online Videos 1, 2, 3, and 4) tted the
diagnostic criteria for isolated myocardial noncompaction. MRI showed late gadolinium
enhancement (LGE) of the apex with transmural distribution (C and D, arrows) and of the
interventricular septum with intramyocardial distribution (C, arrowhead). An implantable
cardioverter-debrillator was implanted after sustained ventricular tachycardia. Electroanatomic
voltage mapping showed an area of low voltage (<0.5 mV) consistent with scarred tissue and
corresponding to the LGE area at MRI (E, red dots: ablation sites). Five years later, he underwent
cardiac transplantation for worsening heart failure. Macroscopic examination of the explanted
heart conrmed the apical scar (F, asterisk) and showed broad anastomosing trabeculae with deep
recesses (G, arrow). Macroscopic (H, arrows) and microscopic examination (I: scar tissue in blue
in Massons trichrome stain) conrmed the presence of brosis; myocyte vacuolization was also
shown (J and K: arrows; hematoxylin and eosin stain).