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The impact of tricuspid valve regurgitation severity on exercise capacity and cardiac-related hospitalisations among adults with non-operated Ebstein’s anomaly

Published online by Cambridge University Press:  04 June 2019

Jonathan Buber ,
Ori Vatury ,
Robert Klempfner  and
Shai Tejman-Yarden
Jonathan Buber*
Affiliation:
Leviev Heart Center, Sheba Medical Center, Ramat-Gan, Israel Edmond and Lily Safra International Center of Congenital Heart Diseases, Sheba Medical Center, Ramat-Gan, Israel Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel Division of Cardiology, Department of Medicine, University of Washington School of Medicine, Seattle, WA, USA
Ori Vatury
Affiliation:
Leviev Heart Center, Sheba Medical Center, Ramat-Gan, Israel Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Robert Klempfner
Affiliation:
Leviev Heart Center, Sheba Medical Center, Ramat-Gan, Israel Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
Shai Tejman-Yarden
Affiliation:
Edmond and Lily Safra International Center of Congenital Heart Diseases, Sheba Medical Center, Ramat-Gan, Israel Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
*
Author for correspondence: Jonathan Buber, MD, Division of Cardiology, Department of Medicine, University of Washington School of Medicine, 1959 NE Pacific Street, Seattle, WA 98195, USA. Tel: +1 206 685 3228; Fax: +1 206 6161012; E-mail: [email protected]
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Abstract

Background:

Tricuspid valve regurgitation is an inherent part of Ebstein’s anomaly, yet whether the severity of the regurgitation further impairs exercise capacity and contributes to long-term morbidity on top of the lesion severity per se is unknown.

Methods:

To evaluate for this potential effect, we included 30 patients with Ebstein’s anomaly who did not undergo any form of surgical interventions and had a cardiopulmonary exercise test and echocardiographic studies in this retrospective analysis. Echocardiographic studies and cardiopulmonary exercise tests were critically reviewed for lesion severity grade, tricuspid regurgitation degree, and exercise parameters. Cardiac-related hospitalisations were recorded from computerised medical records and during clinic visits.

Results:

Fourteen patients (47%) had moderate and 8 (27%) had severe regurgitation. Patients with ≥ moderate regurgitation exhibited significantly lower exercise capacity (median % predicted maximal oxygen consumption, 62 versus 79%, p = 0.03) and venilatory efficiency at exercise. When stratifying exercise results by regurgitation degree, a stepwise decrease in oxygen consumption and ventilatory efficiency with increasing regurgitation severity was observed, regardless of the anatomic lesion severity. During a median follow-up of 4.6 years, > moderate tricuspid regurgitation was associated with significantly lower cumulative probability of freedom from cardiac hospitalisations.

Conclusions:

We report that among non-operated Ebstein’s anomaly patients, greater tricuspid regurgitation severity was associated with worse exercise capacity and with overall higher probability of cardiac-related hospitalisations independent from the underlying lesion severity.

Keywords

Ebstein’s anomalytricuspid regurgitationexercise
Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 6 , June 2019 , pp. 800 - 807
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Copyright
© Cambridge University Press 2019 

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References

Dearani, JA, Danielson, GK. Cong Heart Surgery Nomenclature and Database Project: Ebstein’s anomaly and tricuspid valve disease. Ann Thorac Surg 2000; 69: S106–S117.CrossRefGoogle Scholar
Zuberbuhler, JR, Allwork, SP, Anderson, RH. The spectrum of Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979; 77: 202–211.Google ScholarPubMed
Ebstein, W. Ueber einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedinct drch eine angeborene hochgradige Missbildung derselben. Arch Anat Physiol 1866: 238–254.Google Scholar
Kipps, AK, Graham, DA, Lewis, E, Marx, GR, Banka, P, Rhodes, J. Natural history of exercise function in patients with Ebstein anomaly: a serial study. Am Heart J 2012; 163: 486–491.CrossRefGoogle ScholarPubMed
Tobler, D, Yalonetsky, S, Crean, AM, et al. Right heart characteristics and exercise parameters in adults with Ebstein anomaly: new perspectives from cardiac magnetic resonance imaging studies. Int J Cardiol 2013; 165: 146–150.CrossRefGoogle ScholarPubMed
Trojnarska, O, Szyszka, A, Gwizdała, A, 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; 111: 92–97.CrossRefGoogle ScholarPubMed
Warnes, CA, Williams, RG, Bashore, TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association task force on practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation 2008; 118: e714–e833.Google Scholar
Baumgartner, H, Bonhoeffer, P, De Groot, NM, et al. ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31: 2915–2957.Google Scholar
Bhatt, AB, Foster, E, Kuehl, K, et al. Congenital heart disease in the older adult: a scientific statement from the American Heart Association. Circulation 2015; 131: 1884–1931.CrossRefGoogle ScholarPubMed
Andersen, MJ, Nishimura, RA, Borlaug, BA. The hemodynamic basis of exercise intolerance in tricuspid regurgitation. Circ Heart Fail 2014; 7: 911–917.CrossRefGoogle ScholarPubMed
Ammash, NM, Warnes, CA, Connoly, HM, Danielson, GK, Sevard, JB. Mimics of Ebstein’s anomaly. Am Heart J 1997; 134: 508–513.CrossRefGoogle ScholarPubMed
Celermajer, DS, Cullen, S, Sullivan, ID, Spiegelhalter, DJ, Wyse, RK, Deanfield, JE. Outcome in neonates with Ebstein’s anomaly. J Am Coll Cardiol 1992; 19: 1041–1046.CrossRefGoogle ScholarPubMed
Celermajer, DS, Bull, C, Till, JA, et al. Ebstein’s anomaly: presentation and outcome from fetus to adult. J Am Coll Cardiol 1994; 23: 170–176.CrossRefGoogle ScholarPubMed
Nishimura, RA, Otto, CM, Bonow, RO, et al. 2014 AHA/ACC guideline 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. Circulation 2014; 129: e521–e643.Google Scholar
Attenhofer Jost, CH, Edmister, WD, Julsrud, PR, et al. Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein’s anomaly. Int J Cardiovasc Imaging 2012; 28: 1147–1159.CrossRefGoogle ScholarPubMed
Guazzi, M, Arena, R, Halle, M, Piepoli, MF, Myers, J, Lavie, CJ. 2016 Focused update: clinical recommendations for cardiopulmonary exercise testing data assessment in specific patient populations. Circulation 2016; 133: e694–e711.CrossRefGoogle ScholarPubMed
Chen, SS, Dimopoulos, K, Sheehan, FH, Gatzoulis, MA, Kilner, PJ. Physiologic determinants of exercise capacity in patients with different types of right-sided regurgitant lesions: Ebstein’s malformation with tricuspid regurgitation and repaired tetralogy of Fallot with pulmonary regurgitation. Int J Cardiol 2016; 205: 1–5.CrossRefGoogle ScholarPubMed