Abstract
Background
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.
Methods
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.
Results
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).
Conclusions
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.
Résumé
Contexte
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.
Méthodologie
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.
Résultats
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).
Conclusions
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.
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Canadian Journal of CardiologyAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Ebstein’s anomaly in adults: Doppler-echocardiographic evaluation.Thorac Cardiovasc Surg. 2000; 48: 209-213
- Clinical aspects of myocardial fibrosis in adults with Ebstein’s anomaly.Heart Vessels. 2018; 33: 1076-1085
- The right ventricle in congenital heart disease.Heart. 2006; 92: i27-38
- QRS duration and QRS fractionation on surface electrocardiogram are markers of right ventricular dysfunction and atrialization in patients with Ebstein anomaly.Eur Heart J. 2013; 34: 191-200
- Atrio-ventricular deformation and heart failure in Ebstein’s anomaly—a cardiovascular magnetic resonance study.Int J Cardiol. 2018; 257: 54-61
- Doppler echocardiographic index for assessment of global right ventricular function.J Am Soc Echocardiogr. 1996; 9: 838-847
- Left atrial function: physiology, assessment, and clinical implications.Eur J Echocardiogr. 2011; 12: 421-430
- Comparison of right ventricular volume measurements between axial and short axis orientation using steady-state free precession magnetic resonance imaging.J Magn Reson Imaging. 2003; 18: 25-32
- The functional right ventricle and tricuspid regurgitation in Ebstein’s anomaly.Int J Cardiol. 2013; 167: 258-261
- More accurate quantification of pulmonary blood flow by magnetic resonance imaging than by lung perfusion scintigraphy in patients with Fontan circulation.Circulation. 2002; 106: 1510-1513
- Tricuspid flow and regurgitation in congenital heart disease and pulmonary hypertension: comparison of 4D flow cardiovascular magnetic resonance and echocardiography.J Cardiovasc Magn Reson. 2018; 20: 5
- Tricuspid valve surgery improves cardiac output and exercise performance in patients with Ebstein’s anomaly.Int J Cardiol. 2013; 166: 494-498
- Exercise capacity, quality of life, and daily activity in the long-term follow-up of patients with univentricular heart and total cavopulmonary connection.Eur Heart J. 2009; 30: 2915-2920
- Interpretation of left ventricular diastolic dysfunction in children with cardiomyopathy by echocardiography: problems and limitations.Circ Cardiovasc Imaging. 2013; 6: 254-261
- Systolic-diastolic functional coupling in healthy children and in those with dilated cardiomyopathy.J Appl Physiol (1985). 2016; 120: 1301-1318
- Severe tricuspid regurgitation shows significant impact in the relationship among peak systolic tricuspid annular velocity, tricuspid annular plane systolic excursion, and right ventricular ejection fraction.J Am Soc Echocardiogr. 2006; 19: 902-910
- The relationship between tricuspid regurgitation severity and right atrial mechanics: a speckle tracking echocardiography study.Int J Cardiovasc Imaging. 2015; 31: 1125-1135
- Abnormal right atrial performance in repaired tetralogy of Fallot: a CMR feature tracking analysis.Int J Cardiol. 2017; 248: 136-142
- Exercise performance in Ebstein’s anomaly in the course of time—deterioration in native patients and preserved function after tricuspid valve surgery.Int J Cardiol. 2016; 218: 79-82
- Reservoir and conduit function of right atrium: impact on right ventricular filling and cardiac output.Am J Physiol Heart Circ Physiol. 2005; 288: 2140-2145
- Relationship between ventricular contractility and early diastolic intraventricular pressure gradients: a diastolic link to systolic function.J Am Soc Echocardiogr. 2008; 21: 501-506
- Right ventricular strain rate and strain analysis in patients with repaired tetralogy of Fallot: possible interventricular septal compensation.J Am Soc Echocardiogr. 2004; 17: 338-344
- The overloaded right heart and ventricular interdependence.Cardiovasc Res. 2017; 113: 1474-1485
- Natural history of exercise function in patients with Ebstein anomaly: a serial study.Am Heart J. 2012; 163: 486-491
- Peak oxygen uptake correlates with disease severity and predicts outcome in adult patients with Ebstein’s anomaly of the tricuspid valve.Int J Cardiol. 2013; 163: 305-308
- Right atrial size relates to right ventricular end-diastolic pressure in an adult population with congenital heart disease.Echocardiography. 2011; 28 (109-16)
Article info
Publication history
Published online: June 11, 2019
Accepted:
May 31,
2019
Received:
April 17,
2019
Footnotes
See page 1832 for disclosure information.
Identification
Copyright
© 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.