The shape and function of the left ventricle in Ebstein's anomaly

doi:10.1016/j.ijcard.2013.12.037

International Journal of Cardiology

Volume 171, Issue 3, 15 February 2014, Pages 404-412

https://doi.org/10.1016/j.ijcard.2013.12.037 Get rights and content

Abstract

Background

Left ventricular (LV) failure is common in Ebstein's anomaly, though remains poorly understood. We investigated whether shape deformity impacts LV function.

Methods

Three-dimensional models of the right ventricle (RV) and LV from 29 adult Ebstein's patients and nine normal subjects were generated from cardiac magnetic resonance image tracings. LV end diastolic (ED) shape, systolic function, septal motion and ventricular interaction were analyzed.

Results

LV ED volume index was normal in Ebstein's (75 ± 19 vs. 78 ± 11 ml/m² in normals, p = 0.50) but the LV was basally narrowed and modestly dilated apically. LV function was reduced globally (ejection fraction (EF) 41 ± 7 vs. 57 ± 5% in normals, p < 0.0001) and regionally (decreased mean segment displacement at end systole (ES) in 12/16 segments, basal Z-scores − 2.1 to − 1.0). Septal dyskinesis was suggested by outward mean segment displacement in at least one basal septal segment in 25 patients (86%) but refuted by septal thickening in 14 (48%), normal septal curvature at ED and ES, and by visually evident basal LV anterior translation in 27 patients (93%). LV EF correlated better with normalized tricuspid annular plane systolic excursion (r = 0.70) than with RV EF (r = 0.42) or RVEDVI (r = 0.18).

Conclusions

Although the Ebstein's LV has preserved volume, it exhibits basal narrowing, modest apical dilation and global hypokinesis. The apparent basal septal dyskinesis observed in most patients is likely attributable to anterior cardiac translation rather than true paradoxical motion. LV EF is unaffected by RV volume, correlating well instead with RV longitudinal shortening.

Introduction

While Ebstein's anomaly is classically considered a malformation of the right heart, the left ventricle (LV) is also affected [1]. As the natural history with Ebstein's anomaly is most commonly heart failure that is often left sided, an understanding of failure modes is important [1], [2]. A few attempts have been made to describe LV abnormalities but were limited by the use of two-dimensional (2D) modalities [2], [3], [4], [5], [6], [7], [8], [9], [10]. Techniques based on three-dimensional (3D) reconstructions of cardiac magnetic resonance imaging (CMR) and 3D echocardiography (echo) permit more comprehensive descriptions of global and regional ventricular shape and function but have not been employed within this population. Therefore, the present study was performed to assess LV global and regional shape and function with particular emphasis on septal behavior, in comparison to healthy controls.

Section snippets

Patient populations

The study population comprises 29 patients with known Ebstein's anomaly and nine healthy subjects whose data were collected previously for investigation of the right ventricle (RV) in Ebstein's anomaly [11]. The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology.

Imaging

CMR images were acquired in 8–12 consecutive short axis slices covering both ventricles in addition to long axis and oblique views targeted to

Statistical methods

Two-tailed t tests were used to compare continuous variables between groups. Correlation between parameters was assessed via linear regression, reported as the Pearson product–moment correlation coefficient, r. Statistical significance was defined as p < 0.05.

Patient population

The normal subjects were slightly older than the Ebstein's patients (46 ± 14 vs. 36 ± 14 years, respectively, p = NS) and more often female (78 vs. 41%, respectively, p < 0.001). BSA was similar in the normal and Ebstein's groups (1.7 ± 0.2 vs. 1.8 ± 0.2 m², respectively, p = NS). Although eight of the 29 Ebstein's patients had undergone surgical repairs, including closure of atrial septal defect, tricuspid or pulmonary valve repair, and/or bidirectional Glenn, there was no statistical difference in 233 of 241

Discussion

We performed an analysis of LV shape at ED and of LV systolic function in Ebstein's anomaly. Our patients displayed irregularly shaped LVs, which appear related to right-sided dilation. LV function was globally depressed with apparent paradoxical septal motion at the base. Upon investigation, however, this septal behavior appeared to be an artifact of anterior LV translation in the majority of patients. LV RV interaction was evidenced by the influence of RV dysfunction on LV function,

Study limitations

The present study utilized data from a small set of healthy subjects. No effort was made to control for concurrent cardiac abnormalities beyond the presence of mitral regurgitation. We only performed analysis at ED and ES because LV function is routinely assessed from these time points alone, and we wished to assist in the interpretation of perceived abnormalities in systolic function. However, we potentially missed important functional behavior during other times within the cardiac cycle.

Conclusions

Shape analysis of the Ebstein's LV demonstrated basal narrowing, mild apical dilation, mild elongation and increased long axis bowing. Functional analysis revealed generally global hypokinesis that appears unrelated to the LV shape irregularities or to the extent of RV dilation. Instead, LV EF correlated best with normalized TAPSE. The apparent basal septal dyskinesis observed in most patients is likely due to cardiac translation.

Disclosures

Dr. Sheehan is a founder and the Chief Scientist of VentriPoint, Inc. She holds equity in the company and provides consultative services with compensation.

Acknowledgments

We wish to acknowledge the committed devotion of our programmer, Edward Bolson, who came out of retirement to write “one more program” for the curvature analysis.

References (38)

D.S. Celermajer et al.
Ebstein's anomaly: presentation and outcome from fetus to adult
J Am Coll Cardiol
(1994)
L. Daliento et al.
Angiographic and morphologic features of the left ventricle in Ebstein's malformation
Am J Cardiol
(1997)
R.A. Hurwitz
Left ventricular function in infants and children with symptomatic Ebstein's anomaly
Am J Cardiol
(1994)
A. Saxena et al.
Late noninvasive evaluation of cardiac performance in mildly symptomatic older patients with Ebstein's anomaly of tricuspid valve: role of radionuclide imaging
J Am Coll Cardiol
(1991)
A. Shiina et al.
Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment
J Am Coll Cardiol
(1984)
S. Sharma et al.
Angiographic abnormalities of the morphologically left ventricle in the presence of Ebstein's malformation
Int J Cardiol
(1989)
B.I. Munt et al.
Left ventricular shape analysis from three-dimensional echocardiograms
J Am Soc Echocardiogr
(1998)
P.S. Douglas et al.
Left ventricular shape, afterload and survival in idiopathic dilated cardiomyopathy
J Am Coll Cardiol
(1989)
M. Hubka et al.
Three-dimensional echocardiographic measurement of left ventricular wall thickness: in vitro and in vivo validation
J Am Soc Echocardiogr
(2002)
S. Sasayama et al.
Dynamic changes in left ventricular wall thickness and their use in analyzing cardiac function in the conscious dog
Am J Cardiol
(1976)

P. Yang et al.

The effect of normalization in reducing variability in regional wall thickening

J Am Soc Echocardiogr

(1997)

G.B. Mancini et al.

A comparison of traditional wall motion assessment and quantitative shape analysis: a new method for characterizing left ventricular function in humans

Am Heart J

(1987)

A. Razdan et al.

Curvature estimation scheme for triangle meshes using biquadratic Bézier patches

Comput Aided Des

(2005)

R.V. Milani et al.

Left ventricular geometry and survival in patients with normal left ventricular ejection fraction

Am J Cardiol

(2006)

C.H. Attenhofer Jost et al.

Ebstein's anomaly — review of a multifaceted congenital cardiac condition

Swiss Med Wkly

(2005)

A.A. Monibi et al.

Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve

Circulation

(1978)

E. Oechslin et al.

Ebstein's anomaly in adults: Doppler-echocardiographic evaluation

Thorac Cardiovasc Surg

(2000)

L.N. Benson et al.

Left ventricular geometry and function in adults with Ebstein's anomaly of the tricuspid valve

Circulation

(1987)

C.M. Lee et al.

The shape and function of the right ventricle in Ebstein's anomaly

Int J Cardiol

(2012)

Cited by (28)

Ventricular-Ventricular Interactions in Critically Ill Infants with Ebstein Anomaly or Dysplasia of the Tricuspid Valve: Time to Remember the Forgotten Left Ventricle
2024, Journal of the American Society of Echocardiography
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 :
Interestingly, we also demonstrated a markedly reduced left ventricular volume and impairment of its systolic function. Similar observations were published by other researchers [28–30], while the team from the Royal Brompton Hospital showed only a decreased left ventricular ejection fraction with its preserved end-diastolic volume [31]. However, this study investigated a younger and more heterogeneous population (with nearly 1/3 patients having undergone cardio surgery) compared to our team's study group.
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.
Lumping vs Splitting in Adult Congenital Heart Disease Research
2022, Journal of the American College of Cardiology
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 :
A single parameter that will account for the complex mechanokinetic interdependency, but which simultaneously remains simple and accessible in everyday practice, is yet to be established. In patients with EA, the pathomechanism of heart failure is not solely related to chamber volumes.24,25 A leftward shift of the interventricular septum (as a consequence of the enlarged aRV) affects the geometry and function of the LV.
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.

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¹: These authors take responsibility for all aspects of the reliability and freedom from bias of the data presented and their discussed interpretation.

²: Founder and Chief Scientist of VentriPoint, Inc. Equity holder and compensated consultant.

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The shape and function of the left ventricle in Ebstein's anomaly

Abstract

Background

Methods

Results

Conclusions

Introduction

Section snippets

Patient populations

Imaging

Statistical methods

Patient population

Discussion

Study limitations

Conclusions

Disclosures

Acknowledgments

J Am Coll Cardiol

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Int J Cardiol

J Am Soc Echocardiogr

J Am Coll Cardiol

J Am Soc Echocardiogr

Am J Cardiol

J Am Soc Echocardiogr

Am Heart J

Comput Aided Des

Am J Cardiol

Ebstein's anomaly — review of a multifaceted congenital cardiac condition

Swiss Med Wkly

Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve

Circulation

Ebstein's anomaly in adults: Doppler-echocardiographic evaluation

Thorac Cardiovasc Surg

Left ventricular geometry and function in adults with Ebstein's anomaly of the tricuspid valve

Circulation

The shape and function of the right ventricle in Ebstein's anomaly

Int J Cardiol