Right heart characteristics and exercise parameters in adults with Ebstein anomaly: New perspectives from cardiac magnetic resonance imaging studies

doi:10.1016/j.ijcard.2011.08.004

International Journal of Cardiology

Volume 165, Issue 1, 30 April 2013, Pages 146-150

https://doi.org/10.1016/j.ijcard.2011.08.004 Get rights and content

Abstract

Background

The utility of cardiac magnetic resonance imaging (CMR) for assessment of adults with Ebstein anomaly is not well-defined. We sought to evaluate CMR characteristics in this population and to relate these to exercise parameters.

Methods

We analyzed CMR studies in adults with unrepaired Ebstein anomaly for measures of severity of Ebstein disease, including atrialized, functional and total right ventricular (RV) volumes, ejection fraction (EF) and severity index (area of atrialized RV + right atrium/functional RV + left ventricle + left atrium). We related these CMR values to cardiopulmonary exercise test measurements.

Results

Twenty-seven adults (mean age 41 ± 14 years, 70% female) were included. Functional RV end-diastolic volume (EDV) was 150 ± 68 mL/m² and atrialized RVEDV was 25 ± 24 mL/m². In 17 patients (63%), the functional RVEDV was enlarged (> 114 mL/m²). Percent predicted peak VO₂ for the population was 65 ± 20%. On univariable analysis, peak VO₂ was inversely related to atrialized RVEDV (p = 0.011), total RVEDV (p = 0.041), functional RVEDV/left ventricular EDV ratio (p = 0.015) and magnitude of tricuspid valve displacement (p = 0.031). In the multivariate model, the only CMR factor to relate to peak VO₂ was atrialized RVEDV (p = 0.011, β = − 0.48). No significant correlations were found between CMR measures and heart rate response or ventilatory response to exercise.

Conclusion

In adults with unrepaired Ebstein anomaly, atrialized RV volume was independently related to aerobic capacity. The volume of the atrialized RV is a novel CMR measure which may express severity of disease. Further research is needed to evaluate the prognostic relevance of this exploratory work.

Introduction

Ebstein anomaly is characterized by a wide spectrum of anatomic and functional disease affecting the tricuspid valve and development of the right ventricle (RV) [1], [2]. Embryologically, there is a failure of the delamination of the tricuspid valve leaflets resulting in varying degrees of apical displacement of the septal and posterior leaflets. Additionally, there is a rotation of the effective tricuspid valve annulus relative to the aortic root [3]. These features render the inlet portion of the RV atrialized, thereby compromising functional RV size early in life [4], [5]. In neonates with Ebstein anomaly, an association has been made between echocardiographic assessment of heart size and outcome [6], [7]; specifically, severity of clinical disease is thought to be inversely correlated to the size of the functional RV. In adults with Ebstein anomaly, severity of disease, as determined echocardiographically, has been related to aerobic capacity [8]. Although cardiac magnetic resonance imaging (CMR) is widely recognized as the reference standard for assessment of right heart size and function in congenital heart disease [9], studies focused on the utility of CMR imaging in adults with Ebstein anomaly are generally lacking and consequently, the role of CMR in this patient population remains suboptimally defined [10], [11], [12]. Indeed, contemporary consensus statements for Europe and North America feature cardiothoracic ratio as measured on chest X-ray as the sole imaging parameter to guide management of the adult with Ebstein anomaly [13], [14], [15]. Right heart characteristics in adults with Ebstein anomaly using CMR imaging techniques have been recently published [16]. The purpose of the present study, therefore, was to relate these CMR-derived characteristics to exercise parameters obtained from cardiopulmonary studies. We hypothesized that more severe disease, as determined by CMR, would relate to decreased exercise capacity.

Section snippets

Study population

Adults with a diagnosis of Ebstein anomaly were identified from an existing clinical database and patients in whom CMR and contemporary cardiopulmonary exercise testing was performed within 12 months were included in the study. In our practice, CMR studies are performed at the discretion of the treating physician but generally occur electively to obtain baseline data regarding right heart size and function. Patients were excluded if they had a history of tricuspid valve surgery prior to CMR or

Results

A total of 27 adults with Ebstein anomaly who have had CMR and contemporary cardiopulmonary exercise testing were identified. Baseline demographic, clinical and echocardiography data are summarized in Table 1. Although recognized associations of Ebstein anomaly, RV outflow tract obstruction, tricuspid valve stenosis and non-compaction of the LV [27] were not observed in this cohort.

Discussion

To the best of our knowledge, this is the first study to relate CMR characteristics of a series of adults with unrepaired Ebstein anomaly to exercise parameters, specifically aerobic capacity, heart rate response and VE/VCO₂ slope as measured by cardiopulmonary exercise testing. Analysis by CMR provides detail on the components of the right heart affected by Ebstein anomaly, anatomy hitherto under-described using this imaging modality. As recently demonstrated, measurements of right heart size

Conclusions

In adults with unrepaired Ebstein anomaly, the volume of the functional RV was not diminutive but was, in fact, significantly enlarged in the majority of those studied and was associated with the magnitude of apical displacement of the septal leaflet of the tricuspid valve. Atrialized RV volume was independently related to aerobic capacity. The volume of the atrialized RV is a novel CMR measure which may express severity of disease. Further research is needed to evaluate the prognostic

Acknowledgment

The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology.

References (32)

J.R. Zuberbuhler et al.
The spectrum of Ebstein's anomaly of the tricuspid valve
J Thorac Cardiovasc Surg
(1979)
K.R. Anderson et al.
Pathologic anatomy of Ebstein's anomaly of the heart revisited
Am J Cardiol
(1978)
D.S. Celermajer et al.
Outcome in neonates with Ebstein's anomaly
J Am Coll Cardiol
(1992)
D.S. Celermajer et al.
Ebstein's anomaly: presentation and outcome from fetus to adult
J Am Coll Cardiol
(1994)
O. Trojnarska et al.
Adults with Ebstein's anomaly — cardiopulmonary exercise testing and BNP levels exercise capacity and BNP in adults with Ebstein's anomaly
Int J Cardiol
(2006)
S. Eustace et al.
Ebstein's anomaly presenting in adulthood: the role of cine magnetic resonance imaging in diagnosis
Clin Radiol
(1994)
C.K. Silversides et al.
Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan's syndrome
Can J Cardiol
(2010)
S. Yalonetsky et al.
Cardiac magnetic resonance imaging and the assessment of ebstein anomaly in adults
Am J Cardiol
(2011)
R.O. Bonow et al.
Focused update incorporated into the ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to revise the 1998 guidelines for the management of patients with valvular heart disease). Endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons
J Am Coll Cardiol
(2008)
G.P. Diller et al.
Heart rate response during exercise predicts survival in adults with congenital heart disease
J Am Coll Cardiol
(2006)

J. Therrien et al.

Pulmonary valve replacement in adults late after repair of tetralogy of fallot: are we operating too late?

J Am Coll Cardiol

(2000)

C.H. Attenhofer Jost et al.

Noncompacted myocardium in Ebstein's anomaly: initial description in three patients

J Am Soc Echocardiogr

(2004)

S.G. MacLellan-Tobert et al.

Exercise tolerance in patients with Ebstein's anomaly

J Am Coll Cardiol

(1997)

A.T. Yetman et al.

Outcome in cyanotic neonates with Ebstein's anomaly

Am J Cardiol

(1998)

R.M. Wald et al.

Relation of limiting ductal patency to survival in neonatal Ebstein's anomaly

Am J Cardiol

(2005)

E.L. Bove et al.

How I manage neonatal Ebstein's anomaly

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu

(2009)

Cited by (36)

Effect of concomitant atrial septal defect on left ventricular function in adult patients with unrepaired Ebstein's anomaly: a cardiovascular magnetic resonance imaging study
2023, Journal of Cardiovascular Magnetic Resonance
Due to the heterogeneity of anatomic anomalies in Ebstein's anomaly (EA), particularly in the subset of patients with atrial septal defect (ASD), hemodynamic changes, which ultimately cause left ventricular (LV) deterioration remain unclear. The current study aimed to investigate the effect of concomitant ASD on LV function using cardiovascular magnetic resonance (CMR) imaging in patients with EA.
This study included 31 EA patients with ASD, 76 EA patients without ASD, 35 patients with simple ASD and 40 healthy controls. Left/right ventricular (RV, the RV was defined as a summation of the functional RV and atrialized RV in EA patients) volumes and functional parameters, LV strain parameters, and echocardiogram indices were compared among the four groups. Associations between variables were evaluated via Spearman or Pearson correlation analyses. The association between risk factors and LV ejection fraction (EF) was determined via multivariate linear regression analysis.
Both EA patients and ASD patients had a higher RV/LV end-diastolic volume (RVEDV/LVEDV) as well as lower LV and RV ejection fractions (LVEF/RVEF) compared to healthy controls (all p < 0.05). Moreover, the EA patients with ASD had a significantly higher RVEDV/LVEDV and a lower LVEF and RVEF than those without ASD (all p < 0.05). Multivariate linear regression analysis revealed that the presence of ASD was independently associated with LVEF (β = − 0.337, p < 0.001). The RVEDV/LVEDV index was associated with LVEF (r = − 0.361, p < 0.001). Furthermore, the LV longitudinal peak diastolic strain rate (PDSR) was lower in EA patients with ASD than those without ASD, patients with simple ASD, and healthy controls (p < 0.05).
Concomitant ASD is an important risk factor of LV dysfunction in patients with EA, and diastolic dysfunction is likely the predominate mechanism related to LV dysfunction.
Left, but not right, ventricular status determines heart failure in adults with Ebstein anomaly – A case-control study based on magnetic resonance
2022, International Journal of Cardiology
Citation Excerpt :
This fact prompted us to conduct a precise analysis of the anatomy and function of both ventricles in the Ebstein anomaly (Fig. 4) Because of the limitations of echocardiographic assessment of the right ventricle, particularly in the congenitally distorted hearts, we performed the multidimensional volumetric and functional analysis using cardiac magnetic resonance [24,25]. Our study showed a significantly enlarged functional right ventricle with a decreased stroke volume, consistent with previously published papers [15,26,27]. Interestingly, we also demonstrated a markedly reduced left ventricular volume and impairment of its systolic function.
Ebstein anomaly (EA) is a congenital heart defect affecting the right heart. Heart failure (HF) is a significant complication in adults with EA. It may result not only from the right ventricle (RV), but also from the left ventricle (LV) abnormalities. We evaluate the size and function of both ventricles in patients with EA in cardiac magnetic resonance (CMR); to assess their association with the clinical markers of HF.
Study group: 37 unoperated adults with EA (mean age 43.0 ± 14.4y, 21[56.8%] males). Controls: 25 volunteers (mean age 39.9 ± 10.9y, 15[60%] males). Study protocol included: CMR [ejection fraction (EF), end-diastolic (EDVind) and stroke volumes (SVind) indexed by body surface area]; cardiopulmonary test (peak VO2, %peak VO2, VE/VCO2 slope).
Size and systolic function of LV were reduced comparing to the controls [LVEDVind (ml/m2): 63.7(range 38.7–94.2) vs. 79.3(48.7–105.1), p < 0.001; LV SVind (ml/m2): 35.8(22.9–55.1) vs. 49.2(37.8–71.7), p < 0.0001; LVEF(%): 58.3(34–70.5) vs. 62.0(52.0–77.0), p = 0.009]. RV was enlarged comparing to the controls [RVEDVind (ml/m2): 124.3(52.8–378.9) vs. 83.0(64.0–102.0), p < 0.0001) with impaired systolic function (RV SVind (ml/m2): 22.7(11.1–74.1) vs. 48.0(37.8–71.7), p < 0.0001; RVEF(%): 38.0(21.0–66.1) vs. 59.0(49.0–69.0), p < 0.0001). A significant correlation was found between LVEDVind vs. peakVO2 (r = 0.52, p = 0.001); LV SVind vs. peakVO2 (r = 0.47,p = 0.005). There was no correlation between the right ventricular status and exercise capacity.
In adults with Ebstein anomaly the size of left ventricle is reduced, right ventricle is enlarged; the function of both is impaired. Abnormal exercise capacity is associated with left ventricular status. Ventricular interdependence probably plays a role in heart failure pathomechanism.
Left Ventricular Dysfunction in Neonatal Ebstein's Anomaly and Tricuspid Valve Dysplasia
2022, Journal of the American Society of Echocardiography
The mechanisms and prognostic importance of left ventricular (LV) dysfunction in neonatal Ebstein’s anomaly (EA) and tricuspid valve dysplasia (TVD) are not well understood. The authors recently demonstrated reduced cardiac output and dyssynchrony to be common in fetal EA/TVD and therefore hypothesized that LV dysfunction may be associated with worse outcomes in neonatal EA/TVD.
A multicenter retrospective case-control study was conducted among neonatal patients with EA/TVD (n = 32) and a healthy control cohort (n = 17) encountered from 2004 to 2019. The left ventricle was assessed in the first 48 hours after birth using two-dimensional, Doppler-derived, six-segment global and segmental longitudinal strain and circumferential strain (CS) and dyssynchrony indices (the SD of time-to-peak strain and a novel global dyssynchrony index [DI], calculated as [peak segmental average − peak global average]/peak segmental average).
Neonates with EA/TVD demonstrated reduced combined cardiac index (4.2 ± 1.5 L/min/m² vs 6.5 ± 2.2 L/min/m² in control subjects, P < .001), impaired LV CS (−15.4 ± 6.9 vs −26.2 ± 5.8, P < .001), and increased circumferential dyssynchrony (CS DI 0.20 ± 0.16 vs 0.09 ± 0.04 [P = .019]; SD of time-to-peak CS 63 ± 25 vs 40 ± 15 [P = .003]). Transplantation-free survival occurred in 20 of 32 patients (63%) at 6 months. Increased CS DI and absence of pulmonary valve flow (PVF) were most predictive of mortality; CS DI > 0.2 was associated with 25% survival in subjects without PVF, whereas all patients with CS DI < 0.1 survived.
In neonates with EA/TVD and absence of PVF, there is abnormal LV deformation and compromised cardiac output in association with increased dyssynchrony. Increased CS DI is associated with increased risk for mortality in EA/TVD with no forward PVF.
SCMR Position Paper (2020) on clinical indications for cardiovascular magnetic resonance
2020, Journal of Cardiovascular Magnetic Resonance
The Society for Cardiovascular Magnetic Resonance (SCMR) last published its comprehensive expert panel report of clinical indications for CMR in 2004. This new Consensus Panel report brings those indications up to date for 2020 and includes the very substantial increase in scanning techniques, clinical applicability and adoption of CMR worldwide. We have used a nearly identical grading system for indications as in 2004 to ensure comparability with the previous report but have added the presence of randomized controlled trials as evidence for level 1 indications. In addition to the text, tables of the consensus indication levels are included for rapid assimilation and illustrative figures of some key techniques are provided.
Severity Scores for Ebstein Anomaly: Credibility and Usefulness of Echocardiographic vs Magnetic Resonance Assessments of the Celermajer Index
2019, Canadian Journal of Cardiology
Citation Excerpt :
Thus, for many decades a misleading assumption held true, namely, that the aRV was believed to encroach on the fRV, thus significantly reducing the size of the former.2,7 With the CMR technique, however, researchers have recently proved that in the majority of EA population, the volume of RV exceeds normal ranges with the outflow tract significantly contributing to this enlargement.4,5,17 This dilatation can be explained mostly by a rotational displacement of the septal and posterior tricuspid leaflets, resulting in very eccentric regurgitant jets directed toward the infundibular part of RV, which is simply not appreciated in 4-chamber echocardiographic views.18,19
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.
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).
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.
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.
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.
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).
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.
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.
Atrio-ventricular deformation and heart failure in Ebstein's Anomaly — A cardiovascular magnetic resonance study
2018, International Journal of Cardiology
Citation Excerpt :
Ebstein's Anomaly (EA) of the tricuspid valve (TV) accounts for approximately 0.5% of congenital heart disease (CHD) patients. Many EA patients suffer from progressive right heart failure and impaired physical exercise capacity at some stage in their life [1,2]. The pathophysiology comprises a dysplastic and tethered TV displaced towards the right ventricular (RV) apex with subsequent “atrialization” of the RV (aRV), higher grade tricuspid valve regurgitation (TVR) and RA and RV volume overload [3].
We aimed to quantify atrial and ventricular myocardial deformation in Ebstein's Anomaly (EA) in a case-control study with cardiovascular magnetic resonance (CMR) feature tracking and to correlate changes in cardiac performance with the severity of disease and clinical heart failure parameters.
Atrial and ventricular deformation was measured using CMR feature tracking in 30 EA and 20 healthy control subjects. Atrial performance was characterized using longitudinal strain and strain rate parameters for reservoir function, conduit function and booster pump function. Ventricular performance was characterized using RV and LV global longitudinal strain (εl) and LV circumferential and radial strain (εc and εr). Volumetric measurements for the ventricles including the Total Right/Left-Volume-Index (R/L-Volume-Index) and heart failure markers (BNP, NYHA class) were also quantified.
EA patients showed significantly impaired right atrial performance, which correlated with heart failure markers (NYHA, BNP, R/L-Volume-Index). LA function in EA patients was also impaired with atrial contractile function correlating with NYHA class. EA patients exhibited impaired RV myocardial deformation, also with a significant correlation with heart failure markers.
CMR feature tracking can be used to quantify ventricular and atrial function in a complex cardiac malformation such as EA. EA is characterized by impaired quantitative right heart atrio-ventricular deformation, which is associated with heart failure severity. While LV function remains preserved, there is also significant impairment of LA function. These quantitative performance parameters may represent early markers of cardiac deterioration of potential value in the clinical management of EA.

View all citing articles on Scopus

^☆: Relationship with industry policy: no industry conflicts to declare.

View full text

Right heart characteristics and exercise parameters in adults with Ebstein anomaly: New perspectives from cardiac magnetic resonance imaging studies☆

Abstract

Background

Methods

Results

Conclusion

Introduction

Section snippets

Study population

Results

Discussion

Conclusions

Acknowledgment

J Thorac Cardiovasc Surg

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Int J Cardiol

Clin Radiol

Can J Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

J Am Soc Echocardiogr

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu