Abstract
Background
The severity score of Ebstein anomaly (EA) that corresponds to clinical status is
still under research, with the Celermajer index (Cel-ind) being one of those. The
agreement between echocardiographic and cardiac magnetic resonance (CMR) assessment
of Cel-ind is not known. We determined the agreement between echocardiography- and
CMR-derived Cel-ind and its relationship with heart failure markers.
Methods
A total of 37 unoperated adults with EA (mean age, 43.0 ± 14.4 years) underwent echocardiography,
CMR, and cardiopulmonary tests. For the Cel-ind, end-diastolic areas in echocardiography
or end-diastolic volumes in CMR were used according to the following formula: Cel-ind =
(right atrium + atrialized right ventricle)/(functional right ventricle + left atrium +
left ventricle). On the basis of this assumption, patients were classified as follows:
grade 1 = Cel-ind < 0.5, grade 2 = 0.5 to 0.99, grade 3 = 1.0 to 1.49, grade 4 > 1.5.
The agreement between echocardiographic and CMR was determined with the intraclass
correlation coefficient or Cohen’s kappa (<0.2 poor agreement; 0.2-0.4 fair agreement;
0.4-0.6 moderate agreement; 0.6-0.8 good agreement; 0.8-1.0 very good agreement).
Results
The median echoCel-ind was 0.9 (range, 0.4-2.3), and the median cmrCel-ind was 0.7
(range, 0.3-5.3). Grade 1 or 2 was found in 19 patients (51.3%) by echocardiography
and in 27 patients (72.9%) by CMR. The agreement between imaging methods was only
fair (kappa = 0.39, P = 0.002) for the 4-grade classification and moderate (intra-class correlation coefficient =
0.43; 95% confidence interval, 0.13-0.66) for Cel-ind calculation. Significant correlations
between Cel-ind in CMR and cardiopulmonary parameters were found (for peak oxygen
uptake: R = −0.35, P = 0.034; for the ventilation/carbon dioxide slope: R = 0.46, P = 0.005). Neither of them correlated with echocardiographic severity score.
Conclusions
The agreement between echocardiographic and CMR assessment of the Cel-ind is at most
moderate; echocardiography usually overestimates, but rarely underestimates, EA severity.
Cel-ind by CMR seems to be more valuable, because it is associated with heart failure
markers.
Résumé
Contexte
Les scores d’évaluation de la sévérité de la maladie d’Ebstein correspondant aux observations
cliniques sont toujours à l’étude; l’indice de Celermajer fait partie de ces scores.
On ne sait pas dans quelle mesure les évaluations de l’indice de Celermajer obtenues
par échocardiographie et par résonance magnétique cardiaque (RMC) concordent. Nous
avons donc déterminé la concordance entre l’indice de Celermajer évalué par échocardiographie
et le même indice évalué par RMC, ainsi que le lien entre cet indice et les marqueurs
de l’insuffisance cardiaque.
Méthodologie
Au total, 37 adultes non opérés présentant une maladie d’Ebstein (âge moyen : 43,0
± 14,4 ans) ont été soumis à une échocardiographie, à un examen par RMC et à des épreuves
cardiopulmonaires. Pour calculer l’indice de Celermajer, nous avons utilisé les aires
en fin de diastole obtenues par échocardiographie ou les volumes en fin de diastole
obtenus par RMC selon la formule suivante : indice de Celermajer = (oreillette droite +
portion « auricularisée » du ventricule droit)/(ventricule droit fonctionnel + oreillette
gauche + ventricule gauche). Sur la base de cette hypothèse, les patients ont été
classés comme suit : grade 1 = indice de Celermajer < 0,5; grade 2 = de 0,5 à 0,99;
grade 3 = de 1,0 à 1,49; grade 4 > 1,5. La concordance entre les évaluations obtenues
par échocardiographie et par RMC a été déterminée au moyen d’un coefficient de corrélation
intraclasse ou du kappa de Cohen (< 0,2 : faible concordance; 0,2-0,4 : concordance
passable; 0,4-0,6 : concordance modérée; 0,6-0,8 : bonne concordance; 0,8-1,0 : très
bonne concordance).
Résultats
L’indice de Celermajer médian obtenu par échocardiographie s’établissait à 0,9 (min.-max.
: 0,4-2,3) et à 0,7 (min.-max. : 0,3-5,3) dans le cas de la RMC. Selon l’évaluation
par échocardiographie et l’évaluation par RMC, 19 patients (51,3 %) et 27 patients
(72,9 %), respectivement, présentaient une affection de grade 1 ou 2. La concordance
entre les deux techniques d’imagerie était seulement passable (kappa = 0,39, p = 0,002) pour les affections de grade 4 et modérée (coefficient de corrélation intraclasse =
0,43; intervalle de confiance à 95 % : de 0,13 à 0,66) pour le calcul de l’indice
de Celermajer. Des corrélations significatives entre l’indice de Celermajer obtenu
par RMC et les paramètres cardiopulmonaires ont été observées (consommation maximale
d’oxygène : R = -0,35, p = 0,034; pente ventilation/dioxyde de carbone : R = 0,46, p = 0,005). Aucune corrélation avec le score de sévérité obtenu par échocardiographie
n’a été observée.
Conclusions
La concordance entre les indices de Celermajer obtenus par échocardiographie et par
RMC est au mieux modérée; la sévérité de la maladie d’Ebstein évaluée par échocardiographie
est habituellement surestimée, mais rarement sous-estimée. L’indice de Celermajer
obtenu par RMC semble être plus utile, parce qu’il est associé à des marqueurs de
l’insuffisance cardiaque.
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Article info
Publication history
Published online: August 14, 2019
Accepted:
August 7,
2019
Received:
March 28,
2019
Footnotes
See page 1840 for disclosure information.
Identification
Copyright
© 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
