Skip to main content Accessibility help

Quality of life in young patients after cone reconstruction for Ebstein anomaly

  • Kristen L. Sessions (a1), Charlotte Van Dorn (a2) (a3), Joseph A. Dearani (a4), Simrit Warring (a1), Kaitlin Leopold (a1), Philip L. Wackel (a2), Frank Cetta (a2) and Jonathan N. Johnson (a2)...



To evaluate the health status and quality of life of young patients who had cone reconstruction for Ebstein anomaly.


We reviewed all patients who had cone reconstruction from 2007 to 2016 at our institution. Prospective surveys were mailed to all eligible patients. Quality of life was assessed using the PedsQL 4.0 Generic Core Scales, including four domains: physical, emotional, social, and school functioning.


Of 116 eligible patients, 72 (62%) responded. About 96% reported their health as excellent or good, and 52% were symptom-free. Only 37% of patients were taking any medications, the most common of which was aspirin (30%). Only 19% had been hospitalised for cardiac reasons following cone reconstruction. The average self-reported quality of life was 85.3/100, whereas the average parent proxy-reported quality of life was 81.8/100. There was no difference by self or parent proxy-report in quality of life between cone reconstruction patients and healthy children; however, quality of life was significantly better compared with children with other chronic health conditions. By self-report and parent proxy-report, 15.1 and 16.7% of patients were deemed “at risk” for reduced quality of life, respectively. Socially, 63/64 (98%) patients over 5 years old were either full-time students or working full-time.


Children with Ebstein anomaly following cone reconstruction have excellent quality of life comparable with healthy peers and significantly better than other children with chronic health conditions. Families of children with Ebstein anomaly can expect excellent quality of life, long-term health status, and social functioning following cone reconstruction.


Corresponding author

Author for Correspondence: Charlotte Van Dorn, Department of Pediatric Cardiology, Department of Critical Care, Mayo Clinic, 200 1st St SW, Rochester, MN 55905, USA. Tel: 507 266 0676; Fax: 507 284 3968; E-mail:


Hide All
1. Boston, US, Dearani, JA, O’Leary, PW, et al. Tricuspid valve repair for Ebstein’s anomaly in young children: a 30-year experience. Ann Thorac Surg 2006; 81(2): 690696.
2. da Silva, JP, Baumgratz, JF, da Fonseca, L, et al. The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 2007; 133(1): 215223.
3. Dearani, JA, Bacha, E, da Silva, JP. Cone reconstruction of the tricuspid valve for Ebstein’s anomaly: anatomic repair. Oper Tech Thorac Cardiovasc Surg 2008; 13(2): 109125.
4. Dearani, JA, Said, SM, Burkhart, HM, et al. Strategies for tricuspid re-repair in Ebstein malformation using the cone technique. Ann Thorac Surg 2013; 96(1): 202210.
5. Anderson, HN, Dearani, JA, Said, SM, et al. Cone reconstruction in children with Ebstein anomaly: the Mayo Clinic experience. Congenit Heart Dis 2014; 9(3): 266271.
6. Badiu, CC, Schreiber, C, Hörer, J, et al. Early timing of surgical intervention in patients with Ebstein’s anomaly predicts superior long-term outcome. European Journal of Cardio-Thoracic 2010; 37(1): 186192.
7. Brown, ML, Dearani, JA, Danielson, GK, et al. The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 2008; 135(5): 11201136. e7.
8. Brown, ML, Dearani, JA, Danielson, GK, et al. Functional status after operation for Ebstein anomaly: the Mayo Clinic experience. J Am Coll Cardiol 2008; 52(6): 460466.
9. Dearani, JA, Said, SM, O’Leary, PW, etal. Anatomic repair of Ebstein’s malformation: lessons learned with cone reconstruction. Ann Thorac Surg 2013; 95(1): 220-226; discussion 26–8. doi: 10.1016/j.athoracsur.2012.04.146 [published Online First: 2012/12/04]
10. Silva, JP, Silva, LdF, Moreira, LF, et al. Cone reconstruction in Ebstein’s anomaly repair: early and long-term results. Arq Bras Cardiol 2011; 97(3): 199208.
11. Marino, BS, Lipkin, PH, Newburger, JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management. Circulation 2012; 126(9): 11431172.
12. Spijkerboer, A, Utens, E, Bogers, A, et al. Long-term behavioural and emotional problems in four cardiac diagnostic groups of children and adolescents after invasive treatment for congenital heart disease. Int J Cardiol 2008; 125(1): 6673.
13. Varni, JW, Limbers, CA, Burwinkle, TM. Impaired health-related quality of life in children and adolescents with chronic conditions: a comparative analysis of 10 disease clusters and 33 disease categories/severities utilizing the PedsQL™ 4.0 Generic Core Scales. Health Qual Life Outcomes 2007; 5(1): 43.
14. Guerra, GG, Robertson, CM, Alton, GY, et al. Quality of life 4 years after complex heart surgery in infancy. J Thorac Cardiovasc Surg 2013; 145(2): 482488. e2.
15. Guerra, GG, Robertson, CM, Alton, GY, et al. Health-related quality of life in pediatric cardiac extracorporeal life support survivors. Pediatr Crit Care Med 2014; 15(8): 720727.
16. Bowman, JL, Dearani, JA, Burkhart, HM, et al. Should repair of partial atrioventricular septal defect be delayed until later in childhood? Am J Cardiol 2014; 114(3): 463467. doi: 10.1016/j.amjcard.2014.05.020 [published Online First: 2014/07/16]
17. Driscoll, DJ, Mottram, CD, Danielson, GK. 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 1988; 11(4): 831836.
18. Muller, J, Kuhn, A, Tropschuh, A, et al. 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: 7982. doi: 10.1016/j.ijcard.2016.05.014 [published Online First: 2016/05/28]
19. Muller, J, Kuhn, A, Vogt, M, et al. Improvements in exercise performance after surgery for Ebstein anomaly. J Thorac Cardiovasc Surg 2011; 141(5): 11921195. doi: 10.1016/j.jtcvs.2010.08.083[published Online First: 2010/11/26]
20. MacLellan-Tobert, SG, Driscoll, DJ, Mottram, CD, et al. Exercise tolerance in patients with Ebstein’s anomaly. J Am Coll Cardiol 1997; 29(7): 16151622.
21. Li, X, Wang, SM, Schreiber, C, et al. More than valve repair: effect of cone reconstruction on right ventricular geometry and function in patients with Ebstein anomaly. Int J Cardiol 2016; 206: 131137. doi: 10.1016/j.ijcard.2016.01.032 [published Online First: 2016/01/21]
22. Varni, JW, Burwinkle, TM, Seid, M, et al. The PedsQL™* 4.0 as a pediatric population health measure: feasibility, reliability, and validity. Ambul Pediatr 2003; 3(6): 329341.
23. Varni, JW, Limbers, CA. The PedsQL™ 4.0 generic core scales young adult version feasibility, reliability and validity in a university student population. J Health Psychol 2009; 14(4): 611622.
24. Varni, JW, Seid, M, Kurtin, PS. PedsQL™ 4.0: reliability and validity of the Pediatric Quality of Life Inventory™ Version 4.0 Generic Core Scales in healthy and patient populations. Med Care 2001; 39(8): 800812.



Altmetric attention score

Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed