Copyright © Italian Federation of Cardiology. Unauthorized reproduction of this article is prohibited.
Biventricular non-compaction demonstrated on multi-slice
computed tomography with echocardiographic correlation
Saverio Grillone
a
, Gaetano Nucifora
b
, Gianluca Piccoli
a
, Pasquale Gianfagna
b
,
Fjoralba Hysko
c
, Daisy Pavoni
b
, Gianaugusto Slavich
b
, Alessandro Proclemer
b
and Daniele Gasparini
a
Left ventricular non-compaction (LVNC) is a myocardial
disorder characterized by prominent trabeculations and
deep intertrabecular recesses within the left ventricular wall.
Multi-slice computed tomography (CT) might represent a
valid non-invasive imaging technique for the diagnostic
work-up of these patients, being able to visualize the
compacted and non-compacted layers and to
simultaneously rule out the presence of associated
coronary artery disease and congenital heart disease. In the
present report, the CT features of LVNC are described.
J Cardiovasc Med 2013, 14:677–680
Keywords: echocardiography, left ventricular non-compaction, magnetic
resonance imaging, multi-slice computed tomography
a
Division of Diagnostic Angiography and Interventional Radiology, Department of
Diagnostic Imaging,
b
Division of Cardiology, Cardiothoracic Department,
University Hospital ‘Santa Maria della Misericordia’, Udine, Italy and
c
Division of
Radiology, Latisana General Hospital, Latisana, Italy
Correspondence to Gaetano Nucifora, MD, FESC, Division of Cardiology,
Cardiothoracic Department, University Hospital ‘Santa Maria della Misericordia’,
Udine, Italy. P.le Santa Maria della Misericordia, 33100 Udine, Italy
Tel: +39432552441; fax: +39432482353; e-mail: gnucifora@cardionet.it
Received 17 February 2012 Revised 18 April 2012
Accepted 5 June 2012
A 63-year-old man without known coronary risk factors
was referred to our hospital because of progressively
worsening heart failure symptoms. His medical history
was remarkable for pacemaker implantation 5 years
before because of complete atrioventricular block. The
patient was initially evaluated with transthoracic echo-
cardiography, which showed biventricular dilatation and
severe impairment of systolic function of both the left
(LV) and right ventricle (RV). Sponge-like trabeculae
within the LV anterior, inferior and lateral wall, with a
non-compacted to compacted myocardium end-systolic
ratio more than 2, and marked trabeculation of the RV
were observed (Fig. 1). Colour Doppler showed flow
between the ventricular cavities and the intertrabecular
spaces (Fig. 2). These features led to the suspicion of
biventricular non-compaction. In order to provide a
better characterization of the cardiomyopathy, and to
rule out the presence of associated coronary artery disease
(CAD) and congenital heart disease (CHD), ECG-gated
64-slice computed tomography (CT) was performed. The
coronary arteries were normal, although a persistent left
inferior vena cava with hemiazygos continuation draining
into the right atrium through an enlarged coronary sinus
was observed (Figs 3 and 4). The presence of a non-
compacted endocardial layer, consisting of a trabecular
meshwork and deep intertrabecular spaces, and a com-
pacted epicardial layer, for both the LV and RV was
documented (Fig. 1), confirming the diagnosis of biven-
tricular non-compaction. A non-compacted to compacted
myocardium ratio more than 2.3 was measured for the
anterior, inferior and lateral LV wall.
Left ventricular non-compaction (LVNC) is a myocardial
disorder included among the primary genetic cardiomyo-
pathies and the unclassified cardiomyopathies in the
World Health Organization and European Society of
Cardiology classifications, respectively.
1,2
It is character-
ized by prominent trabeculations and deep intertrabecu-
lar recesses within the LV wall; these features are thought
to be related to an arrest in the normal embryogenesis of
the endocardium and myocardium.
1,2
LVNC has a broad spectrum of clinical and pathophy-
siological findings; the age at onset of symptoms in
LVNC is also highly variable.
3,4
LV systolic function at
the time of diagnosis may range from completely normal
to severely reduced and clinical manifestations may
encompass asymptomatic LV dysfunction, severe con-
gestive heart failure, arrhythmias and embolic events.
3,4
The onset of cardiac symptoms is frequently delayed
until adulthood and patients older than 60 years at
diagnosis have been described.
3
Late onset of symptoms
in LVNC may be related to progressive ventricular dys-
function caused by impaired microvascular function
superimposed on the primary cardiomyopathic process;
3
microcirculatory dysfunction has indeed been postulated
to occur LVNC, accounting for subendocardial ischaemia
and scarring.
4
Echocardiography, eventually with the use of echo con-
trast agent, is the key non-invasive tool for the diagnosis
of LVNC.
5
Several echocardiographic criteria for the
diagnosis of LVNC have been proposed previously,
mainly relying on location of the pathology (mid-lateral,
Images in cardiovascular medicine
1558-2027 ß 2013 Italian Federation of Cardiology DOI:10.2459/JCM.0b013e328356a4a6