Tricuspid valve surgery improves cardiac output and exercise performance in patients with Ebstein's anomaly
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.
References (30)
- et al.
Two-dimensional echocardiographic spectrum of Ebstein's anomaly: detailed anatomic assessment
J Am Coll Cardiol
(1984) - et al.
Ebstein's anomaly: presentation and outcome from fetus to adult
J Am Coll Cardiol
(1994) - et al.
Morphologic spectrum of Ebstein's malformation: revisitation relative to surgical repair
J Thorac Cardiovasc Surg
(1999) - et al.
Ebstein's Anomaly: A Functional Concept and Successful Definitive Repair
J Thorac Cardiovasc Surg
(1964) - et al.
Results after surgical repair of Ebstein's anomaly
Ann Thorac Surg
(1997) - et al.
Improvements in exercise performance after surgery for Ebstein anomaly
J Thorac Cardiovasc Surg
(2011) - et al.
Recommendations for evaluation of the severity of native valvular regurgitation with two-dimensional and Doppler echocardiography
J Am Soc Echocardiogr
(2003) - et al.
Quantification of regional left and right ventricular radial and longitudinal function in healthy children using ultrasound-based strain rate and strain imaging
J Am Soc Echocardiogr
(2002) - et al.
Assessment of right ventricular function using two-dimensional echocardiography
Am Heart J
(1984) - et al.
Comparison of accuracy of axial slices versus short-axis slices for measuring ventricular volumes by cardiac magnetic resonance in patients with corrected tetralogy of fallot
Am J Cardiol
(2009)
Cardiac magnetic resonance imaging and the assessment of ebstein anomaly in adults
Am J Cardiol
Exercise tolerance in patients with Ebstein's anomaly
J Am Coll Cardiol
Spectrum of exercise intolerance in 45 patients with Ebstein's anomaly and observations on exercise tolerance in 11 patients after surgical repair
J Am Coll Cardiol
Ventricular volumes in Ebstein's anomaly: x-ray multislice computed tomography before and after repair
Ann Thorac Surg
SchwerzmannM, et al
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
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The first two authors contributed equally to the study.