Tricuspid valve surgery improves cardiac output and exercise performance in patients with Ebstein's anomaly

doi:10.1016/j.ijcard.2011.11.033

International Journal of Cardiology

Volume 166, Issue 2, 20 June 2013, Pages 494-498

https://doi.org/10.1016/j.ijcard.2011.11.033 Get rights and content

Abstract

Background

Clinical, hemodynamic and functional effects of tricuspid valve surgery in patients with Ebstein's anomaly are not well understood.

Methods

Sixteen patients (median age of 27.7 years) were examined before and eight months after surgery by means of echocardiography, cardiovascular magnetic resonance (CMR) and cardiopulmonary exercise testing.

Results

Peak work load (1.87 to 2.0 W/kg; p = 0.026), maximum oxygen uptake (21 to 22 ml/kg/min; p = 0.034) as well as cardiac output (2.7 to 2.9 l/min/m²; p = 0.035) increased postoperatively. The reduction of tricuspid regurgitation led to a higher pulmonary stroke volume (29 to 42 ml/m², p = 0.005) and augmented the left ventricular (LV) volume (55 to 63 ml/min/m²; p = 0.001) with a trend to better ejection fraction (61 to 64%; p = 0.083). Right ventricular (RV) volume index (124 to 108 ml/m2; p = 0.034) and ejection fraction (50 to 42%; p = 0.036) decreased on CMR. Echocardiographic measurements of RV function also decreased (tricuspid annular plane systolic excursion 2.3 to 1.7; p = 0.002; isovolumic acceleration 0.98 to 0.65; p = 0.004; and 2-d longitudinal global strain − 19.3 to − 16.25; p = 0.006).

Conclusion

Tricuspid valve surgery improves exercise capacity in patients with Ebstein's anomaly. The reduction of tricuspid regurgitation decreases the volume of the right ventricle and increases pulmonary antegrade flow. As a result LV volume and cardiac output increase. This hemodynamic benefit occurs despite the preload dependent reduction in RV volume and ejection fraction.

Introduction

Following Ebstein's 1866 description of a post-mortem patient with cyanosis and tricuspid valve anomaly [1], another 100 years would pass until the first angiographic [2] and later echocardiographic [3] diagnoses of Ebstein's anomaly in living patients were made. Due to the wide variation in right ventricle and right atrioventricular (AV) junction morphology, the pathomorphology of the tricuspid valve (TrV) has remained a focus of scientific interest for many years.[4], [5], [6] Since the 1960s, surgical interventions on the TrV with either repair [7], [8] or replacement [9] have evolved continually and effective treatment options for this rare congenital malformation have become available.

Despite establishment of the technical success of these surgical interventions, there remains only limited data regarding the beneficial effect of surgery on long-term outcome in these patients. Recently, Müller et al. [10] showed an increase in peak oxygen uptake and work capacity in 21 patients after TrV surgery. The hemodynamic component of that improvement has not yet been examined.

We therefore decided to perform a prospective cohort study on patients before and after a surgical intervention on the TrV. Patients were assessed by means of conventional echocardiography, tissue Doppler imaging, 2-d speckle tracking, cardiovascular magnetic resonance (CMR) and cardiopulmonary exercise testing (CPET). The aim was to describe in detail changes in cardiac output, RV and LV volumes and function and their relation to peak oxygen uptake.

Section snippets

Patient population

Sixteen (8 male, 8 female) of 26 consecutive patients who underwent TrV surgery due to Ebstein´s disease at our institution from August 2005 to October 2009 were prospectively included in the study. Ten patients were not eligible for CMR study because of young age (below 8 years) or presence of a cardiac pacemaker and therefore were not included in this study. Median age at surgery was 27.7 years, (range from 8.2 to 69.1 years).

The anatomic severity of Ebstein´s anomaly was classified based on

Results

Following surgery, both tricuspid regurgitation (p = 0.001) and tricuspid ring diameters (p = 0.002) decreased significantly while LV diastolic diameters (measured using echocardiography) increased (p = 0.07). TAPSE, used as a longitudinal parameter of RV function, declined significantly after surgery. Table 1 shows pre- and post-operative values of conventional echocardiographic parameters.

As shown in Table 2 patients after tricuspid valve repair or tricuspid valve replacement showed significantly

Discussion

To the best of our knowledge, we present the first results proving a significant clinical and hemodynamic benefit of tricuspid valve surgery in patients with Ebstein's anomaly. Our clinical hypothesis is that the increase in working capacity and maximum oxygen uptake is attributable to a postoperative increase in pulmonary forward flow as measured by CMR-determined stroke volumes. An effective reduction of tricuspid regurgitation may lead to an increase in cardiac output despite a reduction of

Acknowledgement

The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology. We would like to thank in particular Dr. Mac Guill for proofreading and revising the manuscript.

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Right Ventricular Electromechanical Dyssynchrony and Its Relation to Right Ventricular Remodeling, Dysfunction, and Exercise Capacity in Ebstein Anomaly
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Abnormal atrioventricular and intraventricular electrical conduction and dysfunction of the functional right ventricle (fRV) are common in Ebstein anomaly (EA). However, fRV mechanical dyssynchrony and its relation to fRV function are poorly characterized. We evaluated fRV mechanical dyssynchrony in EA patients in relation to fRV remodeling, dysfunction, and exercise intolerance.
We retrospectively analyzed data from nonoperated EA patients and age-matched controls who underwent echocardiography, cardiovascular magnetic resonance imaging, and cardiopulmonary exercise testing to quantify right ventricular (RV) remodeling, dysfunction, and exercise capacity. The relation of these to fRV dyssynchrony was retrospectively investigated. Right ventricular mechanical dyssynchrony was defined by early fRV septal activation (right-sided septal flash), RV lateral wall prestretch/late contraction, postsystolic shortening, and intra-RV delay using two-dimensional strain echocardiography. The SD of time to peak shortening among the fRV segments was calculated as a parameter of mechanical dispersion.
Thirty-five EA patients (10 of whom were <18 years of age) and 35 age-matched controls were studied. Ebstein anomaly patients had worse RV function and increased intra-RV dyssynchrony versus controls. Nineteen of 35 (54%) EA patients had early septal activation with simultaneous stretch and consequent late activation and postsystolic shortening of RV lateral segments. Intra-fRV mechanical delay correlated with fRV end-diastolic volume index (r = 0.43, P < .05) and fRV end-systolic volume index (r = 0.63, P < .001). The fRV ejection fraction was lower in EA with versus without right-sided septal flash (44.9 ± 11.0 vs 54.2 ± 8.2, P = .012). The fRV mechanical dispersion correlated with the percentage of predicted peak VO₂ (r = −0.35, P < .05).
In EA, fRV mechanical dyssynchrony is associated with fRV remodeling, dysfunction, and impaired exercise capacity. Mechanical dyssynchrony as a therapeutic target in selected EA patients warrants further study.
Effect of concomitant atrial septal defect on left ventricular function in adult patients with unrepaired Ebstein's anomaly: a cardiovascular magnetic resonance imaging study
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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.
Cell cycle defects underlie childhood-onset cardiomyopathy associated with Noonan syndrome
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Childhood-onset myocardial hypertrophy and cardiomyopathic changes are associated with significant morbidity and mortality in early life, particularly in patients with Noonan syndrome, a multisystemic genetic disorder caused by autosomal dominant mutations in genes of the Ras-MAPK pathway. Although the cardiomyopathy associated with Noonan syndrome (NS-CM) shares certain cardiac features with the hypertrophic cardiomyopathy caused by mutations in sarcomeric proteins (HCM), such as pathological myocardial remodeling, ventricular dysfunction, and increased risk for malignant arrhythmias, the clinical course of NS-CM significantly differs from HCM. This suggests a distinct pathophysiology that remains to be elucidated. Here, through analysis of sarcomeric myosin conformational states, histopathology, and gene expression in left ventricular myocardial tissue from NS-CM, HCM, and normal hearts complemented with disease modeling in cardiomyocytes differentiated from patient-derived PTPN11^N308S/+ induced pluripotent stem cells, we demonstrate distinct disease phenotypes between NS-CM and HCM and uncover cell cycle defects as a potential driver of NS-CM.
Right Ventricular Diastolic Function and Right Atrial Function and Their Relation With Exercise Capacity in Ebstein Anomaly
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Citation Excerpt :
The severity of TR was classified as follows: < 20% (mild); 20% to 40% (moderate); > 40% (severe).11 Patients prospectively underwent a symptom-limited cardiopulmonary exercise test on an upright bicycle.12 Breath-by-breath gas exchange analysis was performed (Vmax229, SensorMedics; Cardinal Health, Dublin, OH).
Right ventricular (RV) diastolic function and right atrial (RA) function are poorly characterized in patients with Ebstein anomaly (EA) but may influence functional capacity. We aimed to evaluate RV diastolic function and RA function in EA and study their relationship with biventricular systolic function and exercise capacity.
Seventy-two patients with EA and 69 controls prospectively underwent echocardiography, cardiovascular magnetic resonance imaging, and cardiopulmonary exercise testing to investigate RV systolic and diastolic function, RA function, and exercise capacity.
Altered RV diastolic function was indicated by the reduced tricuspid valve E/A ratio, percentage RV filling time, and early and late diastolic strain rate; and by the increased tricuspid valve E/E′, isovolumic relaxation time, and RV myocardial performance index. The average of 6-RV-segment early diastolic strain rate correlated modestly with peak VO₂ (r = 0.38, P < 0.01), RV ejection fraction (r = 0.41, P < 0.01), and left ventricular ejection fraction (r = 0.33, P < 0.05). Patients with EA had impaired RA reservoir, conduit, and pump function, which were associated with peak VO₂ (r = 0.54, P < 0.001 for reservoir function).
Altered RV diastolic function and RA function in patients with EA are associated with impaired biventricular systolic function and exercise capacity. The stronger correlation of RA vs RV function with exercise capacity suggests that it may be important to evaluate RA function in this population.
La fonction diastolique du ventricule droit et la fonction de l’oreillette droite sont mal caractérisées chez les patients atteints de l’anomalie d’Ebstein, mais pourraient avoir une incidence sur la capacité fonctionnelle. Notre objectif était d’évaluer la fonction diastolique du ventricule droit et la fonction de l’oreillette droite et d’étudier leur lien avec la fonction systolique biventriculaire et la capacité d’effort.
Soixante-douze patients atteints de l’anomalie d’Ebstein et 69 sujets témoins se sont soumis de façon prospective à une échocardiographie, à un examen cardiovasculaire par imagerie par résonnance magnétique et à une épreuve d’effort cardiorespiratoire pour évaluer les fonction systolique et diastolique du ventricule droit, la fonction de l’oreillette droite et la capacité d’effort.
La présence d’une réduction du rapport E/A au niveau de la valve tricuspide, du pourcentage du temps de remplissage du ventricule droit et du taux de surcharge diastolique précoce et tardive ainsi que la présence d’une augmentation du rapport E/E′ au niveau de la valve tricuspide, du temps de relaxation isovolumique et de l’indice de performance myocardique du ventricule droit indiquaient une atteinte de la fonction diastolique du ventricule droit. La corrélation a été modeste entre la moyenne du taux de surcharge diastolique précoce sur 6 segments du ventricule droit et la consommation d’oxygène maximal (volume d’oxygène; VO₂) (coefficient de corrélation [r] = 0,38, p < 0,01), la fraction d’éjection du ventricule droit (r = 0,41, p < 0,01) et la fraction d’éjection du ventricule gauche (r = 0,33, p < 0,05). Les patients atteints de l’anomalie d’Ebstein présentaient une altération de la fonction de l’oreillette droite en ce qui concerne le réservoir, la conduite et le pompage, altération qui était associée à un VO₂ maximal (r = 0,54, p < 0,001 pour la fonction de réservoir).
L’altération de la fonction diastolique du ventricule droit et de la fonction de l’oreillette droite chez les patients atteints de l’anomalie d’Ebstein est associée à une atteinte de la fonction systolique biventriculaire et de la capacité d’effort. La corrélation plus étroite entre la capacité d’effort et le fonctionnement de l’oreillette droite plutôt que celle du ventricule droit laisse supposer qu’il pourrait être important d’évaluer la fonction de l’oreillette droite chez cette population.
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¹: The first two authors contributed equally to the study.

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Tricuspid valve surgery improves cardiac output and exercise performance in patients with Ebstein's anomaly

Abstract

Background

Methods

Results

Conclusion

Introduction

Section snippets

Patient population

Results

Discussion

Acknowledgement

J Am Coll Cardiol

J Am Coll Cardiol

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Ann Thorac Surg

J Thorac Cardiovasc Surg

J Am Soc Echocardiogr

J Am Soc Echocardiogr

Am Heart J

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Ann Thorac Surg

Assessment of right ventricular systolic function:comparison between cardiacmagnetic resonance derived ejection fraction and pulsed-wave tissue Doppler imaging of the tricuspid annulus. Int J Cardiol