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Archives of Cardiovascular Disease (2010) 103, 150—159
CLINICAL RESEARCH
Assessment of left ventricular non-compaction in
adults: Side-by-side comparison of cardiac magnetic
resonance imaging with echocardiography
Évaluation de la non-compaction isolée du ventricule gauche chez l’adulte :
comparaison de l’imagerie par résonance magnétique nucléaire avec
l’échographie cardiaque
Franck Thunya,∗
, Alexis Jacquierb
, Bertrand Jopa
,
Roch Giorgic
, Jean-Yves Gaubertb
, Jean-Michel
Bartolib
, Guy Moulinb
, Gilbert Habiba
a
Department of Cardiology, CHU la Timone, University of Marseille Méditerranée ,
264, rue Saint-Pierre, 13385 Marseille cedex 05, France
b
Department of Radiology, CHU la Timone, University of Marseille Méditerranée ,
264, rue Saint-Pierre, 13385 Marseille cedex 05, France
c
LERTIM, EA 3283, service de santé publique et d’information médicale, hôpital de la
Timone, Assistance publique—Hôpitaux de Marseille, faculté de médecine, université
d’Aix-Marseille, Marseille, France
Received 19 November 2009; received in revised form 10 January 2010; accepted 15 January
2010
Available online 10 March 2010
KEYWORDS
Non-compaction;
Cardiomyopathy;
Echocardiography;
Cardiac magnetic
resonance
Summary
Background. — Two-dimensional echocardiography images obtained at end-diastole and end-
systole and cardiac magnetic resonance (CMR) images obtained at end-diastole represent the
three imaging methodologies validated for diagnosis of left ventricular non-compaction (LVNC).
No study has compared these methodologies in assessing the magnitude of non-compaction.
Aims. — To compare two-dimensional echocardiography with CMR in the evaluation of patients
with suspected LVNC.
Abbreviations: C, compacted epicardial layer thickness; CMR, cardiac magnetic resonance; LV, left ventricular; LVNC, left ventricular
non-compaction; NC, non-compacted endocardial layer thickness; Echo-2D, two-dimensional echocardiography.
∗ Corresponding author. Fax: +33 491 384 764.
E-mail address: franck.thuny@wanadoo.fr (F. Thuny).
1875-2136/$ — see front matter © 2010 Elsevier Masson SAS. All rights reserved.
doi:10.1016/j.acvd.2010.01.002
Mutimodality imaging of non-compaction 151
Methods. — Sixteen patients (48 ± 17 years) with LVNC underwent echocardiography and CMR
within the same week. Echocardiography images obtained at end-diastole and end-systole
were compared in a blinded fashion with those obtained by CMR at end-diastole to assess
non-compaction in 17 anatomical segments.
Results. — All segments could be analysed by CMR, whereas only 238 (87.5%) and 237 (87.1%)
could be analysed by echocardiography at end-diastole and end-systole, respectively (p = 0.002).
Among the analysable segments, a two-layered structure was observed in 54.0% by CMR, 42.9%
by echocardiography at end-diastole and 41.4% by echocardiography at end-systole (p = 0.006).
Similar distribution patterns were observed with the two echocardiographic methodologies.
However, compared with echocardiography, CMR identified a higher rate of two-layered struc-
tures in the anterior, anterolateral, inferolateral and inferior segments. Echocardiography
at end-systole underestimated the NC/C maximum ratio compared with CMR (p = 0.04) and
echocardiography at end-diastole (p = 0.003). No significant difference was observed between
CMR and echocardiography at end-diastole (p = 0.83). Interobserver reproducibility of the NC/C
maximum ratio was similar for the three methodologies.
Conclusion. — CMR appears superior to standard echocardiography in assessing the extent of
non-compaction and provides supplemental morphological information beyond that obtained
with conventional echocardiography.
© 2010 Elsevier Masson SAS. All rights reserved.
MOTS CLÉS
Non-compaction ;
Cardiomyopathie ;
Échocardiographie ;
IRM cardiaque
Résumé
Contexte. — Les images d’échocardiographie bidimensionnelle (Echo-2D) obtenues à la fois en
télédiastole et en télésystole, ainsi que celles fournies par l’IRM cardiaque (IRMc) en télédias-
tole, sont les trois modalités validées pour le diagnostic de non-compaction isolée du ventricule
gauche (NCVG). Aucune étude n’a comparé ces trois modalités entre elles dans l’évaluation de
la distribution et de l’importance de la non-compaction.
Objectifs. — Comparer l’Echo-2D standard avec l’IRMc dans l’évaluation des patients atteints
de NCVG.
Méthodes. — Seize patients (48 ± 17 ans) avec une NCVG ont eu une Echo-2D et une IRMc durant
la même semaine. Les images échocardiographiques, obtenues en télédiastole et télésystole,
ont été comparées, en insu, avec celles obtenues par IRMc en télédiastole, pour évaluer la
non-compaction dans 17 segments anatomiques (soit n = 272 segments).
Résultats. — Tous les segments ont pu être analysés par l’IRMc, alors que seuls 238 (87,5 %)
et 237 (87,1 %) ont pu l’être par l’Echo-2D, respectivement, en télédiastole et en télésystole
(p = 0,002). Parmi les segments analysables, une structure en double couche a été observée chez
54 % des patients en IRMc, 42,9 % par Echo-2D en télédiastole et 41,4 % par Echo-2D en télésys-
tole (p = 0,006). Une distribution similaire de la non-compaction a été observée par les deux
modalités échocardiographiques. Cependant, en comparaison avec l’Echo-2D, l’IRMc identifiait
un taux de structure en double couche plus important dans les segments antérieur, antérolatéral
et inférieur. L’Echo-2D en télésystole sous-estimée le rapport NC/C maximal en comparaison
avec l’IRMc (p = 0,04) et l’Echo-2D en télédiastole (p = 0,003). En revanche, aucune différence
significative n’a été observée entre l’IRMc et l’Echo-2D en télédiastole. La reproductibilité
interobservateur du rapport NC/C maximal était similaire pour les trois modalités.
Conclusion. — L’IRMc apparaît supérieure à l’Echo-2D standard dans l’évaluation de l’extension
de la non-compaction en fournissant une information morphologique supplémentaire.
© 2010 Elsevier Masson SAS. Tous droits réservés.
Introduction
Left ventricular non-compaction (LVNC) is a recently rec-
ognized cardiomyopathy characterized by a distinctive
(‘‘spongy’’) morphological appearance of the left ventricu-
lar (LV) myocardium [1]. Prominent LV trabeculae and deep
intertrabecular recesses are present and the myocardial wall
is often thickened with a thin compacted epicardial layer
and a thickened non-compacted endocardial layer. In some
patients, LVNC is associated with LV dilatation and systolic
dysfunction, leading to heart failure, ventricular arrhythmia
and thrombo-embolic complications [2—8]. This myocardial
disorder has been described by two-dimensional echocar-
diography (Echo-2D) and although many echo criteria have
been proposed [4,8,9], a poor correlation exists between
them [10]. These discrepancies may be explained by differ-
ences not only in the definition of abnormal trabeculation,
but also in the echo planes and phase of the cardiac cycle
in which they are applied (end-diastole or end-systole) [10].
Thus, there is no clear consensus for LVNC diagnosis. More-
over, Echo-2D evaluation can be limited by poor acoustic
windows. Recently, cardiac magnetic resonance (CMR) imag-
ing has been proposed for the diagnosis of LVNC [11—13] and
new criteria have been proposed [14]. This technique has the
152 F. Thuny et al.
capability of acquiring tomographic images of the LV cham-
bers, with border definition that is often superior to that
achievable by echocardiography. However, while Echo-2D
criteria have been validated either at end-diastole [8,9,15]
or end-systole [4], CMR criteria have only been validated
at end-diastole [14]. Thus, no previous study has compared
the two imaging techniques at different times of the cardiac
cycle in the assessment of patients with suspected LVNC. The
aim of the present study was to compare standard Echo-2D
with CMR in the evaluation of patients with isolated LVNC.
Materials and methods
Patient selection
Between January 2003 and January 2008, 16 consecutive
patients for whom diagnosis of LVNC was suspected on
echocardiographic criteria and without CMR contraindica-
tions were enrolled. Each patient was assessed clinically
by echocardiography first and CMR studies were performed
during the same week.
Among the 16 patients included, 12 fulfilled Jenni’s
criteria [4,5]. These specific echocardiographic diagnostic
criteria are (in the absence of coexisting cardiac anomalies):
• a typical two-layered myocardial structure with a thin
compacted outer (epicardial) band and a much thicker,
non-compacted inner (endocardial) layer consisting of
trabecular meshing with deep endocardial spaces;
• a maximum end-systolic non-compacted epicardial layer
thickness/compacted epicardial layer thickness (NC/C)
ratio >2;
• colour Doppler evidence of deeply perfused intertrabec-
ular recesses.
Four other patients were also included, despite having a
maximum NC/C ratio ≤2. In these patients, LVNC was highly
suspected due to the presence of a marked two-layered
structure associated with more than three prominent tra-
beculations [8] and a family history of LVNC.
This study complies with the declaration of Helsinki and
was approved by our institutional review board. Written
informed consent was obtained from all patients.
Two-dimensional echocardiographic imaging
Echo-2D were obtained using commercially available instru-
ments (GE Medical Systems, Milwaukee, Wis) with 3.5-MHz
transducers equipped with harmonic imaging. Images were
acquired in the long-axis parasternal view, the three short-
axis views (basal, mid, apical), and the 2-, 3- and 4-chamber
apical views. These views were used to visualize all 17
segments according to American Heart Association recom-
mendations [16]. Offline analysis was then performed on
digitally stored images (EchoPAC, GE Vingmed, Horthen,
Norway).
CMR imaging
All imaging was performed on a 1.5-T MR scanner (Sym-
phony TIM, Siemens, Erlangen, Germany with a 12-element
phased array cardiac coil and Achieva, Philips, Best, The
Netherlands with a 5-element phased array cardiac coil).
Cine steady-state free precession sequences were acquired
on long-axis, 2-chamber, 4-chamber and short-axis views to
cover the whole left ventricle without any gap between
images. For both scans, cine sequences with retrospective
cardiac gating were used with the following parameters:
• TR/TE = 40 ms/1.8 ms, slice thickness = 6 mm, no gap
between slice, flip angle = 65◦
, matrix = 148 × 256, field of
view = 350 mm × 350 mm, temporal resolution = 30 ms
• TR/TE = 35/1.5 ms, slice thickness = 6 mm, no gap
between slice, flip angle = 60◦
, matrix = 148 × 256, field
of view = 350 mm × 350 mm, temporal resolution = 30 ms,
for the Siemens and Philips scans, respectively.
All examinations were transferred to a dedicated
workstation. LV volumes, ejection fraction and LV tra-
beculation were determined using ArgusTM
post-processing
software (Siemens, Erlangen, Germany). The cine loops
were reviewed and the end-diastolic and end-systolic frames
were identified.
Image analysis
Qualitative and quantitative analyses of all 17 segments
were performed using the three imaging methodologies:
Echo-2D at end-diastole, Echo-2D at end-systole and CMR at
end-diastole. CMR analysis was not performed at end-systole
because, in most cases, the trabeculations and two-layered
structure could not be visualized at this time in the car-
diac cycle and because the measurements were validated at
this point by a previous study. For each segment (n = 272),
the following criteria were assessed with the three imaging
methodologies: analysable segment (yes/no), two-layered
structure (yes/no) and NC/C maximum ratio. A segment was
considered to be non-analysable if the imaging methodology
failed to assess the presence or absence of the two-layered
structure. The two-layered structure was assessed with one
of the long- or short-axis views. The NC/C ratio was mea-
sured in short-axis views when possible, but long-axis views
were used in some cases (Fig. 1). Only segment 17 was
always measured using 2- or 4-chamber views. For each
patient and each imaging methodology, only the maximal
NC/C ratio was used for subsequent analysis.
LV volumes and LV ejection fractions were calculated by
Echo-2D from the 2- and 4-chamber images using the biplane
Simpson’s rule and by CMR from the short-axis views using
the disks method.
The same observer performed the entire analysis for each
imaging methodology and was blinded to the data obtained
for the alternative imaging test.
Pathological analysis
Pathological analysis was performed in the three patients
who underwent cardiac transplantation. After perfusion and
fixation of the hearts with formalin, the macroscopic prepa-
rations were analysed.
Statistical analysis
All continuous variables are expressed as means ± standard
deviations; non-continuous variables are presented as per-
Mutimodality imaging of non-compaction 153
Figure 1. Assessment of the two-layered structure and the non-compacted epicardial layer thickness/compacted epicardial layer thickness
(NC/C) ratio in short-axis views by the three imaging methodologies. Red lines are non-compacted layers; yellow lines are compacted layers.
CMR: cardiac magnetic resonance; Echo-2D: two-dimensional echocardiography.
centages. Comparisons between non-continuous variables
were performed by the 2
-test. Comparisons of NC/C max-
imum ratios between the imaging methodologies (CMR at
end-diastole, Echo-2D at end-diastole and Echo-2D at end-
systole) were performed using a Friedman test and then,
when significant, by the Wilcoxon rank sum test for post hoc
analysis. In this case, to ensure an overall type I error rate
of 5%, Bonferroni correction was applied and an adjusted
p < 0.05/3 = 0.016 was considered significant.
Agreement in the NC/C maximum ratio between the
imaging methodologies was determined by Bland-Altman
analysis.
Two observers reviewed each imaging modality (Echo-2D
and CMR) independently in all patients. The two observers
were senior cardiologists who were experienced in echocar-
diography and CMR. All NC/C maximum ratio measurements
were repeated by these two observers who were blinded
to the previous results. The intraclass correlation coeffi-
cient and Bland-Altman analysis were used to assess the
interobserver reproducibility for each imaging methodol-
ogy. Bias was estimated by the mean difference between
two measurements and paired t-tests were used to evaluate
significance.
A two-sided p-value of <0.05 was established as the level
of statistical significance for all tests. All analyses were per-
formed with SPSS for Windows 15.0 (Chicago, IL, USA).
Results
Patient characteristics
Our sample consisted of 16 consecutive patients aged
48 ± 17 years with LVNC. No patient had symptoms or signs
of neuromuscular disease. Only one patient had LV thrombo-
sis, which was identified by both CMR and Echo-2D. Patient
characteristics are reported in Table 1.
Analysable segments
CMR assessed significantly more segments than Echo-2D.
Among the 272 segments, all (100%) could be assessed
by CMR, whereas only 238 (87.5%) and 237 (87.1%) could
Table 1 Characteristics of the 16 patients with definite diagnosis or suspicion of left ventricular non-compaction.
Patient Age (years) Sex (M/F) Family history LBBB NYHA class LVEF (%) (Echo-2D) LVEF (%) (CMR) LVT
1 21 F − + III/IV 65 36 −
2 22 F − + I/II 42 45 −
3a
29 M − − I/II 44 59 −
4 38 F + − III/IV 44 53 −
5 40 M − + I/II 34 21 −
6 41 M − − III/IV 43 34 −
7 42 M + + III/IV 5 8 −
8 42 M − + III/IV 65 63 −
9 45 M − + I/II 20 32 +
10 50 M + + III/IV 25 29 −
11 51 M − + III/IV 18 18 −
12 54 M + + III/IV 45 45 −
13 70 F + − I/II 73 74 −
14a
71 M − − III/IV 27 27 −
15a
72 M + − I/II 44 10 −
16a
74 F − + III/IV 13 11 −
CMR: cardiac magnetic resonance; F: female; LBBB: left bundle branch block; LVEF: left ventricular ejection fraction; LVT: left ventricle
thrombosis; M: male; NYHA: New York Heart Association; Echo-2D: two-dimensional echocardiography.
a Patients not fulfilling all Jenni echocardiography criteria.
154 F. Thuny et al.
Figure 2. Percentage of patients for whom the segmental two-layered structure could not be assessed. The results are expressed for each
segment. CMR: cardiac magnetic resonance; Echo-2D: two-dimensional echocardiography.
be analysed by Echo-2D at end-diastole and end-systole
(p = 0.002), respectively. These segments were more diffi-
cult to analyse by Echo-2D due to poor acoustic windows.
No significant difference was observed between Echo-2D at
end-diastole and Echo-2D at end-systole (p = 0.90). The dif-
ferences between CMR and Echo-2D were observed mainly
for the anterior, anterolateral and inferolateral segments
(Fig. 2).
Extent of the two-layered structure
CMR identified significantly more segments with a two-
layered structure. Among the analysable segments, a
two-layered structure was observed in 54.0% of cases by
CMR, 42.9% by Echo-2D at end-diastole and 41.4% by Echo-
2D at end-systole (p = 0.006). No significant difference was
observed between Echo-2D at end-diastole and Echo-2D at
end-systole (p = 0.74). Fig. 3 illustrates the distribution of
the two-layered structure according to the imaging method-
ology used. Similar distribution patterns were observed with
the two echocardiographic methodologies. However, com-
pared with echocardiography, CMR identified a higher rate of
two-layered structures in the anterior, anterolateral, infer-
olateral and inferior segments.
Assessment of the NC/C maximum ratio
The NC/C maximum ratio was higher when measured by CMR
at end-diastole compared with Echo-2D at end-diastole and
Echo-2D at end-systole (p = 0.01) (Table 2). Echo-2D at end-
systole underestimated the NC/C maximum ratio compared
Table 2 NC/C maximum ratio according to the imaging
methodologies.
Imaging methodology NC/C maximum ratio
Mean ± standard
deviation
Range
CMR at end-diastole 3.91 ± 2.29 1.63 to 9.33
Echo-2D at end-diastole 3.60 ± 1.60 2.0 to 7.33
Echo-2D at end-systole 2.81 ± 0.83 1.71 to 4.57
Global comparison between the three imaging methodologies,
p = 0.01. CMR at end-diastole vs Echo-2D at end-diastole,
p = 0.83. CMR at end-diastole vs Echo-2D at end-systole,
p = 0.04. Echo-2D at end-diastole vs Echo-2D at end-systole,
p = 0.003. C: compacted layer thickness; CMR: cardiac magnetic
resonance; NC: non-compacted layer thickness; Echo-2D:
two-dimensional echocardiography.
with CMR (p = 0.04) and Echo-2D at end-diastole (p = 0.003)
(Table 2 and Fig. 4). No significant difference was observed
between CMR and Echo-2D at end-diastole (p = 0.83) (Table 2
and Fig. 4).
The interobserver variability of the NC/C maximum ratio
was assessed in all patients, with similar correlation and
agreement observed for the three imaging methodologies
(Table 3 and Fig. 5).
Fig. 6 shows a representative example of the comparison
between the three imaging methodologies and the patho-
logical examination.
Table 3 Interobserver NC/C maximum ratio according to the imaging methodologies.
Observer 1a
Observer 2a
Mean difference (95% CI) ICC
CMR at end-diastole 3.91 ± 2.29 4.36 ± 2.0 −0.45 (−1.18 to 0.29) 0.80
Echo-2D at end-diastole 3.60 ± 1.60 4.30 ± 2.03 −0.70 (−1.19 to −0.21)b
0.87
Echo-2D at end-systole 2.81 ± 0.83 3.39 ± 1.38 −0.58 (−1.04 to −0.12)b
0.71
C: compacted layer thickness; CI: confidence interval; CMR: cardiac magnetic resonance; ICC: interclass correlation coefficient; NC:
non-compacted layer thickness; Echo-2D: two-dimensional echocardiography.
a Data are mean ± standard deviation.
b p < 0.05.
Mutimodality imaging of non-compaction 155
Figure 3. Distribution of the two-layered structure according to the imaging methodology used. The bars represent the percentage of
patients in each imaging methodology with a two-layered structure in the indicated segments. CMR: cardiac magnetic resonance; Echo-2D:
two-dimensional echocardiography.
156 F. Thuny et al.
Figure 4. Bland—Altman plots comparing the measurements of
two observers in assessment of the non-compacted epicardial
layer thickness/compacted epicardial layer thickness (NC/C) max-
imum ratio. C: compacted layer thickness; CMR: cardiac magnetic
resonance; NC: non-compacted layer thickness; Echo-2D: two-
dimensional echocardiography.
Discussion
This is the first study to compare the three validated imaging
methodologies for the diagnosis of LVNC. We showed that,
in patients with suspected LVNC, CMR provides a more accu-
rate and reliable evaluation of the extent of non-compacted
myocardium than Echo-2D images performed either at end-
diastole or end-systole. Moreover, this work revealed a good
agreement between Echo-2D at end-diastole and CMR mea-
surements.
Figure 5. Bland—Altman plots comparing the measurements
of two observers in assessment of the non-compacted epicar-
dial layer thickness/compacted epicardial layer thickness (NC/C)
maximum ratio in each of the three imaging methodologies. C:
compacted layer thickness; CMR: cardiac magnetic resonance; NC:
non-compacted layer thickness; Echo-2D: two-dimensional echocar-
diography.
Study rationale
LVNC is a cardiomyopathy for which various diagnostic crite-
ria have been proposed [4,8,9,14]. These criteria are based
on imaging data provided by different techniques at dif-
ferent times during the cardiac cycle. Thus, among the
three Echo-2D definitions published, two have attempted to
describe non-compaction at end-diastole [8,9] and one at
end-systole [4]. A recent study has validated a CMR crite-
rion by using an adapted version of existing Echo-2D criteria
Mutimodality imaging of non-compaction 157
Figure 6. Short-axis, 4-chamber and 2-chamber views of a patient with a definite diagnosis of isolated left ventricular non-compaction.
Each view is compared according to the three imaging methodologies and the pathological specimen after transplantation (white arrow
shows the defibrillator lead). The two-layered structure and trabeculations are best visualized by cardiac magnetic resonance at the level of
the anterior and anterolateral segments, particularly in the short-axis views. CMR: cardiac magnetic resonance; Echo-2D: two-dimensional
echocardiography.
at end-diastole [14]. There is a lack of data comparing
the three imaging methodologies (Echo-2D at end-diastole,
Echo-2D at end-systole and CMR at end-diastole), hence no
consensus exists as to which is the best method for evaluat-
ing LVNC.
Only one previous work reported better delineation of
the extent of abnormal trabeculation by CMR compared with
Echo-2D [11]. However, this study had several limitations,
such as inclusion of only four paediatric patients, evalua-
tion of only nine LV segments and measurements performed
exclusively at end-systole. Our study offers the advantage of
comparing for the first time, in a larger sample size, the data
obtained using CMR at end-diastole, Echo-2D at end-diastole
and Echo-2D at end-systole.
Two-dimensional echocardiography at
end-diastole vs end-systole
In the present study, when the two-layered structure was
evaluated as a criterion of LVNC, no significant differ-
ences between Echo-2D at end-diastole and Echo-2D at
end-systole were observed with regard to the number of
analysable segments, extent of non-compaction and inter-
observer reproducibility. However, the NC/C maximum ratio
obtained by Echo-2D at end-diastole was obviously higher
than that obtained at end-systole. This result implies that
the measurements of the NC cannot be performed equally
at end-diastole and end-systole.
Two-dimensional echocardiography vs cardiac
magnetic resonance
In the present work, we demonstrated that CMR was supe-
rior to Echo-2D methodologies with regard to the number
of segments that could be analysed and the evaluation of
the extent of the two-layered structure. These differences
between CMR and Echo-2D were observed especially dur-
ing assessment of anterior, anterolateral and inferolateral
segments.
Two-dimensional echocardiography is generally regarded
as the first diagnostic test used for LVNC [5,17,18]; however,
it has certain technical limitations. Reliable quantitative
delineation of LV wall thickness is dependent on adequate
acoustic windows, particularly for the anterior and lateral
segments that are easier to assess by CMR. This issue was
described particularly in the assessment of hypertrophic
cardiomyopathy [19]. On the other hand, CMR provides non-
oblique images with excellent and uniform contrast at the
endocardial borders that encompass all levels and regions
of the LV and permit virtually complete reconstruction of
the chamber [20]. Therefore, CMR imaging has the poten-
tial to detect segmental non-compaction in any area of the
LV wall and can provide critical supplemental morpholog-
ical information beyond that obtained from conventional
Echo-2D studies.
However, due to its higher cost, long procedure duration
and relatively reduced availability.
158 F. Thuny et al.
CMR should remain a technique of second intention after
Echo-2D. Moreover, in some cases, CMR can overlook non-
compaction as a consequence of respiration and cardiac
movement artifacts or atrial fibrillation [21]. Therefore,
CMR might be indicated in the case of poor acoustic windows
or subnormal Echos-2D in first-degree relatives of patients
with typical LVNC. In addition, it is not clear whether the
extent of non-compaction or the NC/C ratio has prognostic
value; however, CMR might be an appropriate technique for
testing this hypothesis.
Limitations
The relatively small sample size limits the statistical power
of the analysis. Moreover, only adults were included in this
study and a few patients had a minor form of LVNC.
Because Echo-2D was used to screen patients for study
entry, we were not able to define whether CMR is superior
to Echo-2D in screening for LVNC among a large population
of patients.
We did not analyse LVNC according to the three morpho-
logical groups described previously [22] (spongy, meshwork,
prominent trabeculations only) because there is consider-
able subjectivity in defining such patterns [10]. We preferred
to consider the two-layered structure aspect, which we
found to be an easier and more reproducible criterion for
defining the extent of non-compaction.
Finally, contrast echocardiography was not performed
in this work. This technique could have been helpful for
improving the visualization of endocardial borders and may
have allowed for a clearer analysis of the extent of non-
compaction [23].
Conclusions
In summary, both echocardiography and CMR are useful
for the assessment of patients with LVNC. Echocardiog-
raphy must be used as a screening method due to its
large availability and because it allows a similar assess-
ment of the distribution of the non-compacted segments
compared with CMR. CMR appears superior to standard
Echo-2D in assessing the extent of non-compaction and
provides supplemental morphological information beyond
that obtained from conventional Echo-2D studies. For this
reason, we suggest that CMR should be performed in all
patients with LVNC diagnosed by echocardiography to bet-
ter assess the extent of non-compaction, and in all patients
in whom the diagnosis of LVNC remains doubtful after an
echocardiographic study, or in patients with a poor acoustic
window.
Finally, the good agreement between Echo-2D at
end-diastole and CMR measurements justifies the use of end-
diastolic measurements, whatever the technique. Whether
CMR is superior to standard echocardiography for the diag-
nosis of LVNC requires further large prospective studies
including genetic and/or pathological correlations.
Conflict of interest statement
None.
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CMR vs Echo in Assessing LV Non-compaction

  • 1. Archives of Cardiovascular Disease (2010) 103, 150—159 CLINICAL RESEARCH Assessment of left ventricular non-compaction in adults: Side-by-side comparison of cardiac magnetic resonance imaging with echocardiography Évaluation de la non-compaction isolée du ventricule gauche chez l’adulte : comparaison de l’imagerie par résonance magnétique nucléaire avec l’échographie cardiaque Franck Thunya,∗ , Alexis Jacquierb , Bertrand Jopa , Roch Giorgic , Jean-Yves Gaubertb , Jean-Michel Bartolib , Guy Moulinb , Gilbert Habiba a Department of Cardiology, CHU la Timone, University of Marseille Méditerranée , 264, rue Saint-Pierre, 13385 Marseille cedex 05, France b Department of Radiology, CHU la Timone, University of Marseille Méditerranée , 264, rue Saint-Pierre, 13385 Marseille cedex 05, France c LERTIM, EA 3283, service de santé publique et d’information médicale, hôpital de la Timone, Assistance publique—Hôpitaux de Marseille, faculté de médecine, université d’Aix-Marseille, Marseille, France Received 19 November 2009; received in revised form 10 January 2010; accepted 15 January 2010 Available online 10 March 2010 KEYWORDS Non-compaction; Cardiomyopathy; Echocardiography; Cardiac magnetic resonance Summary Background. — Two-dimensional echocardiography images obtained at end-diastole and end- systole and cardiac magnetic resonance (CMR) images obtained at end-diastole represent the three imaging methodologies validated for diagnosis of left ventricular non-compaction (LVNC). No study has compared these methodologies in assessing the magnitude of non-compaction. Aims. — To compare two-dimensional echocardiography with CMR in the evaluation of patients with suspected LVNC. Abbreviations: C, compacted epicardial layer thickness; CMR, cardiac magnetic resonance; LV, left ventricular; LVNC, left ventricular non-compaction; NC, non-compacted endocardial layer thickness; Echo-2D, two-dimensional echocardiography. ∗ Corresponding author. Fax: +33 491 384 764. E-mail address: franck.thuny@wanadoo.fr (F. Thuny). 1875-2136/$ — see front matter © 2010 Elsevier Masson SAS. All rights reserved. doi:10.1016/j.acvd.2010.01.002
  • 2. Mutimodality imaging of non-compaction 151 Methods. — Sixteen patients (48 ± 17 years) with LVNC underwent echocardiography and CMR within the same week. Echocardiography images obtained at end-diastole and end-systole were compared in a blinded fashion with those obtained by CMR at end-diastole to assess non-compaction in 17 anatomical segments. Results. — All segments could be analysed by CMR, whereas only 238 (87.5%) and 237 (87.1%) could be analysed by echocardiography at end-diastole and end-systole, respectively (p = 0.002). Among the analysable segments, a two-layered structure was observed in 54.0% by CMR, 42.9% by echocardiography at end-diastole and 41.4% by echocardiography at end-systole (p = 0.006). Similar distribution patterns were observed with the two echocardiographic methodologies. However, compared with echocardiography, CMR identified a higher rate of two-layered struc- tures in the anterior, anterolateral, inferolateral and inferior segments. Echocardiography at end-systole underestimated the NC/C maximum ratio compared with CMR (p = 0.04) and echocardiography at end-diastole (p = 0.003). No significant difference was observed between CMR and echocardiography at end-diastole (p = 0.83). Interobserver reproducibility of the NC/C maximum ratio was similar for the three methodologies. Conclusion. — CMR appears superior to standard echocardiography in assessing the extent of non-compaction and provides supplemental morphological information beyond that obtained with conventional echocardiography. © 2010 Elsevier Masson SAS. All rights reserved. MOTS CLÉS Non-compaction ; Cardiomyopathie ; Échocardiographie ; IRM cardiaque Résumé Contexte. — Les images d’échocardiographie bidimensionnelle (Echo-2D) obtenues à la fois en télédiastole et en télésystole, ainsi que celles fournies par l’IRM cardiaque (IRMc) en télédias- tole, sont les trois modalités validées pour le diagnostic de non-compaction isolée du ventricule gauche (NCVG). Aucune étude n’a comparé ces trois modalités entre elles dans l’évaluation de la distribution et de l’importance de la non-compaction. Objectifs. — Comparer l’Echo-2D standard avec l’IRMc dans l’évaluation des patients atteints de NCVG. Méthodes. — Seize patients (48 ± 17 ans) avec une NCVG ont eu une Echo-2D et une IRMc durant la même semaine. Les images échocardiographiques, obtenues en télédiastole et télésystole, ont été comparées, en insu, avec celles obtenues par IRMc en télédiastole, pour évaluer la non-compaction dans 17 segments anatomiques (soit n = 272 segments). Résultats. — Tous les segments ont pu être analysés par l’IRMc, alors que seuls 238 (87,5 %) et 237 (87,1 %) ont pu l’être par l’Echo-2D, respectivement, en télédiastole et en télésystole (p = 0,002). Parmi les segments analysables, une structure en double couche a été observée chez 54 % des patients en IRMc, 42,9 % par Echo-2D en télédiastole et 41,4 % par Echo-2D en télésys- tole (p = 0,006). Une distribution similaire de la non-compaction a été observée par les deux modalités échocardiographiques. Cependant, en comparaison avec l’Echo-2D, l’IRMc identifiait un taux de structure en double couche plus important dans les segments antérieur, antérolatéral et inférieur. L’Echo-2D en télésystole sous-estimée le rapport NC/C maximal en comparaison avec l’IRMc (p = 0,04) et l’Echo-2D en télédiastole (p = 0,003). En revanche, aucune différence significative n’a été observée entre l’IRMc et l’Echo-2D en télédiastole. La reproductibilité interobservateur du rapport NC/C maximal était similaire pour les trois modalités. Conclusion. — L’IRMc apparaît supérieure à l’Echo-2D standard dans l’évaluation de l’extension de la non-compaction en fournissant une information morphologique supplémentaire. © 2010 Elsevier Masson SAS. Tous droits réservés. Introduction Left ventricular non-compaction (LVNC) is a recently rec- ognized cardiomyopathy characterized by a distinctive (‘‘spongy’’) morphological appearance of the left ventricu- lar (LV) myocardium [1]. Prominent LV trabeculae and deep intertrabecular recesses are present and the myocardial wall is often thickened with a thin compacted epicardial layer and a thickened non-compacted endocardial layer. In some patients, LVNC is associated with LV dilatation and systolic dysfunction, leading to heart failure, ventricular arrhythmia and thrombo-embolic complications [2—8]. This myocardial disorder has been described by two-dimensional echocar- diography (Echo-2D) and although many echo criteria have been proposed [4,8,9], a poor correlation exists between them [10]. These discrepancies may be explained by differ- ences not only in the definition of abnormal trabeculation, but also in the echo planes and phase of the cardiac cycle in which they are applied (end-diastole or end-systole) [10]. Thus, there is no clear consensus for LVNC diagnosis. More- over, Echo-2D evaluation can be limited by poor acoustic windows. Recently, cardiac magnetic resonance (CMR) imag- ing has been proposed for the diagnosis of LVNC [11—13] and new criteria have been proposed [14]. This technique has the
  • 3. 152 F. Thuny et al. capability of acquiring tomographic images of the LV cham- bers, with border definition that is often superior to that achievable by echocardiography. However, while Echo-2D criteria have been validated either at end-diastole [8,9,15] or end-systole [4], CMR criteria have only been validated at end-diastole [14]. Thus, no previous study has compared the two imaging techniques at different times of the cardiac cycle in the assessment of patients with suspected LVNC. The aim of the present study was to compare standard Echo-2D with CMR in the evaluation of patients with isolated LVNC. Materials and methods Patient selection Between January 2003 and January 2008, 16 consecutive patients for whom diagnosis of LVNC was suspected on echocardiographic criteria and without CMR contraindica- tions were enrolled. Each patient was assessed clinically by echocardiography first and CMR studies were performed during the same week. Among the 16 patients included, 12 fulfilled Jenni’s criteria [4,5]. These specific echocardiographic diagnostic criteria are (in the absence of coexisting cardiac anomalies): • a typical two-layered myocardial structure with a thin compacted outer (epicardial) band and a much thicker, non-compacted inner (endocardial) layer consisting of trabecular meshing with deep endocardial spaces; • a maximum end-systolic non-compacted epicardial layer thickness/compacted epicardial layer thickness (NC/C) ratio >2; • colour Doppler evidence of deeply perfused intertrabec- ular recesses. Four other patients were also included, despite having a maximum NC/C ratio ≤2. In these patients, LVNC was highly suspected due to the presence of a marked two-layered structure associated with more than three prominent tra- beculations [8] and a family history of LVNC. This study complies with the declaration of Helsinki and was approved by our institutional review board. Written informed consent was obtained from all patients. Two-dimensional echocardiographic imaging Echo-2D were obtained using commercially available instru- ments (GE Medical Systems, Milwaukee, Wis) with 3.5-MHz transducers equipped with harmonic imaging. Images were acquired in the long-axis parasternal view, the three short- axis views (basal, mid, apical), and the 2-, 3- and 4-chamber apical views. These views were used to visualize all 17 segments according to American Heart Association recom- mendations [16]. Offline analysis was then performed on digitally stored images (EchoPAC, GE Vingmed, Horthen, Norway). CMR imaging All imaging was performed on a 1.5-T MR scanner (Sym- phony TIM, Siemens, Erlangen, Germany with a 12-element phased array cardiac coil and Achieva, Philips, Best, The Netherlands with a 5-element phased array cardiac coil). Cine steady-state free precession sequences were acquired on long-axis, 2-chamber, 4-chamber and short-axis views to cover the whole left ventricle without any gap between images. For both scans, cine sequences with retrospective cardiac gating were used with the following parameters: • TR/TE = 40 ms/1.8 ms, slice thickness = 6 mm, no gap between slice, flip angle = 65◦ , matrix = 148 × 256, field of view = 350 mm × 350 mm, temporal resolution = 30 ms • TR/TE = 35/1.5 ms, slice thickness = 6 mm, no gap between slice, flip angle = 60◦ , matrix = 148 × 256, field of view = 350 mm × 350 mm, temporal resolution = 30 ms, for the Siemens and Philips scans, respectively. All examinations were transferred to a dedicated workstation. LV volumes, ejection fraction and LV tra- beculation were determined using ArgusTM post-processing software (Siemens, Erlangen, Germany). The cine loops were reviewed and the end-diastolic and end-systolic frames were identified. Image analysis Qualitative and quantitative analyses of all 17 segments were performed using the three imaging methodologies: Echo-2D at end-diastole, Echo-2D at end-systole and CMR at end-diastole. CMR analysis was not performed at end-systole because, in most cases, the trabeculations and two-layered structure could not be visualized at this time in the car- diac cycle and because the measurements were validated at this point by a previous study. For each segment (n = 272), the following criteria were assessed with the three imaging methodologies: analysable segment (yes/no), two-layered structure (yes/no) and NC/C maximum ratio. A segment was considered to be non-analysable if the imaging methodology failed to assess the presence or absence of the two-layered structure. The two-layered structure was assessed with one of the long- or short-axis views. The NC/C ratio was mea- sured in short-axis views when possible, but long-axis views were used in some cases (Fig. 1). Only segment 17 was always measured using 2- or 4-chamber views. For each patient and each imaging methodology, only the maximal NC/C ratio was used for subsequent analysis. LV volumes and LV ejection fractions were calculated by Echo-2D from the 2- and 4-chamber images using the biplane Simpson’s rule and by CMR from the short-axis views using the disks method. The same observer performed the entire analysis for each imaging methodology and was blinded to the data obtained for the alternative imaging test. Pathological analysis Pathological analysis was performed in the three patients who underwent cardiac transplantation. After perfusion and fixation of the hearts with formalin, the macroscopic prepa- rations were analysed. Statistical analysis All continuous variables are expressed as means ± standard deviations; non-continuous variables are presented as per-
  • 4. Mutimodality imaging of non-compaction 153 Figure 1. Assessment of the two-layered structure and the non-compacted epicardial layer thickness/compacted epicardial layer thickness (NC/C) ratio in short-axis views by the three imaging methodologies. Red lines are non-compacted layers; yellow lines are compacted layers. CMR: cardiac magnetic resonance; Echo-2D: two-dimensional echocardiography. centages. Comparisons between non-continuous variables were performed by the 2 -test. Comparisons of NC/C max- imum ratios between the imaging methodologies (CMR at end-diastole, Echo-2D at end-diastole and Echo-2D at end- systole) were performed using a Friedman test and then, when significant, by the Wilcoxon rank sum test for post hoc analysis. In this case, to ensure an overall type I error rate of 5%, Bonferroni correction was applied and an adjusted p < 0.05/3 = 0.016 was considered significant. Agreement in the NC/C maximum ratio between the imaging methodologies was determined by Bland-Altman analysis. Two observers reviewed each imaging modality (Echo-2D and CMR) independently in all patients. The two observers were senior cardiologists who were experienced in echocar- diography and CMR. All NC/C maximum ratio measurements were repeated by these two observers who were blinded to the previous results. The intraclass correlation coeffi- cient and Bland-Altman analysis were used to assess the interobserver reproducibility for each imaging methodol- ogy. Bias was estimated by the mean difference between two measurements and paired t-tests were used to evaluate significance. A two-sided p-value of <0.05 was established as the level of statistical significance for all tests. All analyses were per- formed with SPSS for Windows 15.0 (Chicago, IL, USA). Results Patient characteristics Our sample consisted of 16 consecutive patients aged 48 ± 17 years with LVNC. No patient had symptoms or signs of neuromuscular disease. Only one patient had LV thrombo- sis, which was identified by both CMR and Echo-2D. Patient characteristics are reported in Table 1. Analysable segments CMR assessed significantly more segments than Echo-2D. Among the 272 segments, all (100%) could be assessed by CMR, whereas only 238 (87.5%) and 237 (87.1%) could Table 1 Characteristics of the 16 patients with definite diagnosis or suspicion of left ventricular non-compaction. Patient Age (years) Sex (M/F) Family history LBBB NYHA class LVEF (%) (Echo-2D) LVEF (%) (CMR) LVT 1 21 F − + III/IV 65 36 − 2 22 F − + I/II 42 45 − 3a 29 M − − I/II 44 59 − 4 38 F + − III/IV 44 53 − 5 40 M − + I/II 34 21 − 6 41 M − − III/IV 43 34 − 7 42 M + + III/IV 5 8 − 8 42 M − + III/IV 65 63 − 9 45 M − + I/II 20 32 + 10 50 M + + III/IV 25 29 − 11 51 M − + III/IV 18 18 − 12 54 M + + III/IV 45 45 − 13 70 F + − I/II 73 74 − 14a 71 M − − III/IV 27 27 − 15a 72 M + − I/II 44 10 − 16a 74 F − + III/IV 13 11 − CMR: cardiac magnetic resonance; F: female; LBBB: left bundle branch block; LVEF: left ventricular ejection fraction; LVT: left ventricle thrombosis; M: male; NYHA: New York Heart Association; Echo-2D: two-dimensional echocardiography. a Patients not fulfilling all Jenni echocardiography criteria.
  • 5. 154 F. Thuny et al. Figure 2. Percentage of patients for whom the segmental two-layered structure could not be assessed. The results are expressed for each segment. CMR: cardiac magnetic resonance; Echo-2D: two-dimensional echocardiography. be analysed by Echo-2D at end-diastole and end-systole (p = 0.002), respectively. These segments were more diffi- cult to analyse by Echo-2D due to poor acoustic windows. No significant difference was observed between Echo-2D at end-diastole and Echo-2D at end-systole (p = 0.90). The dif- ferences between CMR and Echo-2D were observed mainly for the anterior, anterolateral and inferolateral segments (Fig. 2). Extent of the two-layered structure CMR identified significantly more segments with a two- layered structure. Among the analysable segments, a two-layered structure was observed in 54.0% of cases by CMR, 42.9% by Echo-2D at end-diastole and 41.4% by Echo- 2D at end-systole (p = 0.006). No significant difference was observed between Echo-2D at end-diastole and Echo-2D at end-systole (p = 0.74). Fig. 3 illustrates the distribution of the two-layered structure according to the imaging method- ology used. Similar distribution patterns were observed with the two echocardiographic methodologies. However, com- pared with echocardiography, CMR identified a higher rate of two-layered structures in the anterior, anterolateral, infer- olateral and inferior segments. Assessment of the NC/C maximum ratio The NC/C maximum ratio was higher when measured by CMR at end-diastole compared with Echo-2D at end-diastole and Echo-2D at end-systole (p = 0.01) (Table 2). Echo-2D at end- systole underestimated the NC/C maximum ratio compared Table 2 NC/C maximum ratio according to the imaging methodologies. Imaging methodology NC/C maximum ratio Mean ± standard deviation Range CMR at end-diastole 3.91 ± 2.29 1.63 to 9.33 Echo-2D at end-diastole 3.60 ± 1.60 2.0 to 7.33 Echo-2D at end-systole 2.81 ± 0.83 1.71 to 4.57 Global comparison between the three imaging methodologies, p = 0.01. CMR at end-diastole vs Echo-2D at end-diastole, p = 0.83. CMR at end-diastole vs Echo-2D at end-systole, p = 0.04. Echo-2D at end-diastole vs Echo-2D at end-systole, p = 0.003. C: compacted layer thickness; CMR: cardiac magnetic resonance; NC: non-compacted layer thickness; Echo-2D: two-dimensional echocardiography. with CMR (p = 0.04) and Echo-2D at end-diastole (p = 0.003) (Table 2 and Fig. 4). No significant difference was observed between CMR and Echo-2D at end-diastole (p = 0.83) (Table 2 and Fig. 4). The interobserver variability of the NC/C maximum ratio was assessed in all patients, with similar correlation and agreement observed for the three imaging methodologies (Table 3 and Fig. 5). Fig. 6 shows a representative example of the comparison between the three imaging methodologies and the patho- logical examination. Table 3 Interobserver NC/C maximum ratio according to the imaging methodologies. Observer 1a Observer 2a Mean difference (95% CI) ICC CMR at end-diastole 3.91 ± 2.29 4.36 ± 2.0 −0.45 (−1.18 to 0.29) 0.80 Echo-2D at end-diastole 3.60 ± 1.60 4.30 ± 2.03 −0.70 (−1.19 to −0.21)b 0.87 Echo-2D at end-systole 2.81 ± 0.83 3.39 ± 1.38 −0.58 (−1.04 to −0.12)b 0.71 C: compacted layer thickness; CI: confidence interval; CMR: cardiac magnetic resonance; ICC: interclass correlation coefficient; NC: non-compacted layer thickness; Echo-2D: two-dimensional echocardiography. a Data are mean ± standard deviation. b p < 0.05.
  • 6. Mutimodality imaging of non-compaction 155 Figure 3. Distribution of the two-layered structure according to the imaging methodology used. The bars represent the percentage of patients in each imaging methodology with a two-layered structure in the indicated segments. CMR: cardiac magnetic resonance; Echo-2D: two-dimensional echocardiography.
  • 7. 156 F. Thuny et al. Figure 4. Bland—Altman plots comparing the measurements of two observers in assessment of the non-compacted epicardial layer thickness/compacted epicardial layer thickness (NC/C) max- imum ratio. C: compacted layer thickness; CMR: cardiac magnetic resonance; NC: non-compacted layer thickness; Echo-2D: two- dimensional echocardiography. Discussion This is the first study to compare the three validated imaging methodologies for the diagnosis of LVNC. We showed that, in patients with suspected LVNC, CMR provides a more accu- rate and reliable evaluation of the extent of non-compacted myocardium than Echo-2D images performed either at end- diastole or end-systole. Moreover, this work revealed a good agreement between Echo-2D at end-diastole and CMR mea- surements. Figure 5. Bland—Altman plots comparing the measurements of two observers in assessment of the non-compacted epicar- dial layer thickness/compacted epicardial layer thickness (NC/C) maximum ratio in each of the three imaging methodologies. C: compacted layer thickness; CMR: cardiac magnetic resonance; NC: non-compacted layer thickness; Echo-2D: two-dimensional echocar- diography. Study rationale LVNC is a cardiomyopathy for which various diagnostic crite- ria have been proposed [4,8,9,14]. These criteria are based on imaging data provided by different techniques at dif- ferent times during the cardiac cycle. Thus, among the three Echo-2D definitions published, two have attempted to describe non-compaction at end-diastole [8,9] and one at end-systole [4]. A recent study has validated a CMR crite- rion by using an adapted version of existing Echo-2D criteria
  • 8. Mutimodality imaging of non-compaction 157 Figure 6. Short-axis, 4-chamber and 2-chamber views of a patient with a definite diagnosis of isolated left ventricular non-compaction. Each view is compared according to the three imaging methodologies and the pathological specimen after transplantation (white arrow shows the defibrillator lead). The two-layered structure and trabeculations are best visualized by cardiac magnetic resonance at the level of the anterior and anterolateral segments, particularly in the short-axis views. CMR: cardiac magnetic resonance; Echo-2D: two-dimensional echocardiography. at end-diastole [14]. There is a lack of data comparing the three imaging methodologies (Echo-2D at end-diastole, Echo-2D at end-systole and CMR at end-diastole), hence no consensus exists as to which is the best method for evaluat- ing LVNC. Only one previous work reported better delineation of the extent of abnormal trabeculation by CMR compared with Echo-2D [11]. However, this study had several limitations, such as inclusion of only four paediatric patients, evalua- tion of only nine LV segments and measurements performed exclusively at end-systole. Our study offers the advantage of comparing for the first time, in a larger sample size, the data obtained using CMR at end-diastole, Echo-2D at end-diastole and Echo-2D at end-systole. Two-dimensional echocardiography at end-diastole vs end-systole In the present study, when the two-layered structure was evaluated as a criterion of LVNC, no significant differ- ences between Echo-2D at end-diastole and Echo-2D at end-systole were observed with regard to the number of analysable segments, extent of non-compaction and inter- observer reproducibility. However, the NC/C maximum ratio obtained by Echo-2D at end-diastole was obviously higher than that obtained at end-systole. This result implies that the measurements of the NC cannot be performed equally at end-diastole and end-systole. Two-dimensional echocardiography vs cardiac magnetic resonance In the present work, we demonstrated that CMR was supe- rior to Echo-2D methodologies with regard to the number of segments that could be analysed and the evaluation of the extent of the two-layered structure. These differences between CMR and Echo-2D were observed especially dur- ing assessment of anterior, anterolateral and inferolateral segments. Two-dimensional echocardiography is generally regarded as the first diagnostic test used for LVNC [5,17,18]; however, it has certain technical limitations. Reliable quantitative delineation of LV wall thickness is dependent on adequate acoustic windows, particularly for the anterior and lateral segments that are easier to assess by CMR. This issue was described particularly in the assessment of hypertrophic cardiomyopathy [19]. On the other hand, CMR provides non- oblique images with excellent and uniform contrast at the endocardial borders that encompass all levels and regions of the LV and permit virtually complete reconstruction of the chamber [20]. Therefore, CMR imaging has the poten- tial to detect segmental non-compaction in any area of the LV wall and can provide critical supplemental morpholog- ical information beyond that obtained from conventional Echo-2D studies. However, due to its higher cost, long procedure duration and relatively reduced availability.
  • 9. 158 F. Thuny et al. CMR should remain a technique of second intention after Echo-2D. Moreover, in some cases, CMR can overlook non- compaction as a consequence of respiration and cardiac movement artifacts or atrial fibrillation [21]. Therefore, CMR might be indicated in the case of poor acoustic windows or subnormal Echos-2D in first-degree relatives of patients with typical LVNC. In addition, it is not clear whether the extent of non-compaction or the NC/C ratio has prognostic value; however, CMR might be an appropriate technique for testing this hypothesis. Limitations The relatively small sample size limits the statistical power of the analysis. Moreover, only adults were included in this study and a few patients had a minor form of LVNC. Because Echo-2D was used to screen patients for study entry, we were not able to define whether CMR is superior to Echo-2D in screening for LVNC among a large population of patients. We did not analyse LVNC according to the three morpho- logical groups described previously [22] (spongy, meshwork, prominent trabeculations only) because there is consider- able subjectivity in defining such patterns [10]. We preferred to consider the two-layered structure aspect, which we found to be an easier and more reproducible criterion for defining the extent of non-compaction. Finally, contrast echocardiography was not performed in this work. This technique could have been helpful for improving the visualization of endocardial borders and may have allowed for a clearer analysis of the extent of non- compaction [23]. Conclusions In summary, both echocardiography and CMR are useful for the assessment of patients with LVNC. Echocardiog- raphy must be used as a screening method due to its large availability and because it allows a similar assess- ment of the distribution of the non-compacted segments compared with CMR. CMR appears superior to standard Echo-2D in assessing the extent of non-compaction and provides supplemental morphological information beyond that obtained from conventional Echo-2D studies. For this reason, we suggest that CMR should be performed in all patients with LVNC diagnosed by echocardiography to bet- ter assess the extent of non-compaction, and in all patients in whom the diagnosis of LVNC remains doubtful after an echocardiographic study, or in patients with a poor acoustic window. Finally, the good agreement between Echo-2D at end-diastole and CMR measurements justifies the use of end- diastolic measurements, whatever the technique. Whether CMR is superior to standard echocardiography for the diag- nosis of LVNC requires further large prospective studies including genetic and/or pathological correlations. Conflict of interest statement None. References [1] Maron BJ, Towbin JA, Thiene G, et al. 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