Hostname: page-component-78c5997874-s2hrs Total loading time: 0 Render date: 2024-11-10T05:42:52.906Z Has data issue: false hasContentIssue false

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*
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: yonibuber@gmail.com

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.

Type
Original Article
Copyright
© Cambridge University Press 2019 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Dearani, JA, Danielson, GK. Cong Heart Surgery Nomenclature and Database Project: Ebstein’s anomaly and tricuspid valve disease. Ann Thorac Surg 2000; 69: S106S117.CrossRefGoogle Scholar
Zuberbuhler, JR, Allwork, SP, Anderson, RH. The spectrum of Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg 1979; 77: 202211.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: 238254.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: 486491.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: 146150.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: 9297.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: e714e833.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: 29152957.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: 18841931.CrossRefGoogle ScholarPubMed
Andersen, MJ, Nishimura, RA, Borlaug, BA. The hemodynamic basis of exercise intolerance in tricuspid regurgitation. Circ Heart Fail 2014; 7: 911917.CrossRefGoogle ScholarPubMed
Ammash, NM, Warnes, CA, Connoly, HM, Danielson, GK, Sevard, JB. Mimics of Ebstein’s anomaly. Am Heart J 1997; 134: 508513.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: 10411046.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: 170176.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: e521e643.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: 11471159.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: e694e711.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: 15.CrossRefGoogle ScholarPubMed