Hostname: page-component-78c5997874-j824f Total loading time: 0 Render date: 2024-11-17T17:25:43.392Z Has data issue: false hasContentIssue false
  • English
  • Français

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

Published online by Cambridge University Press:  14 June 2019

Kristen L. Sessions Open the ORCID record for Kristen L. Sessions [Opens in a new window] ,
Charlotte Van Dorn Open the ORCID record for Charlotte Van Dorn [Opens in a new window] ,
Joseph A. Dearani ,
Simrit Warring ,
Kaitlin Leopold ,
Philip L. Wackel ,
Frank Cetta  and
Jonathan N. Johnson Open the ORCID record for Jonathan N. Johnson [Opens in a new window]
Kristen L. Sessions
Affiliation:
School of Medicine, Mayo Clinic, Rochester, MN, USA
Charlotte Van Dorn*
Affiliation:
Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA Division of Pediatric Critical Care, Mayo Clinic, Rochester, MN, USA
Joseph A. Dearani
Affiliation:
Department of Cardiothoracic Surgery, Mayo Clinic, Rochester, MN, USA
Simrit Warring
Affiliation:
School of Medicine, Mayo Clinic, Rochester, MN, USA
Kaitlin Leopold
Affiliation:
School of Medicine, Mayo Clinic, Rochester, MN, USA
Philip L. Wackel
Affiliation:
Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA
Frank Cetta
Affiliation:
Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA
Jonathan N. Johnson
Affiliation:
Division of Pediatric Cardiology, Mayo Clinic, Rochester, MN, USA
*
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: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Objective:

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

Methods:

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.

Results:

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.

Conclusion:

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.

Keywords

Ebstein anomalyquality of lifecone reconstruction
Type
Original Article
Information
Cardiology in the Young , Volume 29 , Issue 6 , June 2019 , pp. 756 - 760
Check for updates
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

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): 690–696.CrossRefGoogle ScholarPubMed
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): 215–223.CrossRefGoogle ScholarPubMed
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): 109–125.CrossRefGoogle Scholar
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): 202–210.CrossRefGoogle ScholarPubMed
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): 266–271.CrossRefGoogle ScholarPubMed
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): 186–192.CrossRefGoogle ScholarPubMed
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): 1120–1136. e7.CrossRefGoogle ScholarPubMed
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): 460–466.CrossRefGoogle ScholarPubMed
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]CrossRefGoogle ScholarPubMed
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): 199–208.CrossRefGoogle ScholarPubMed
Marino, BS, Lipkin, PH, Newburger, JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management. Circulation 2012; 126(9): 1143–1172.CrossRefGoogle ScholarPubMed
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): 66–73.CrossRefGoogle ScholarPubMed
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.CrossRefGoogle Scholar
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): 482–488. e2.CrossRefGoogle Scholar
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): 720–727.CrossRefGoogle Scholar
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): 463–467. doi: 10.1016/j.amjcard.2014.05.020 [published Online First: 2014/07/16]CrossRefGoogle Scholar
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): 831–836.CrossRefGoogle ScholarPubMed
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: 79–82. doi: 10.1016/j.ijcard.2016.05.014 [published Online First: 2016/05/28]CrossRefGoogle ScholarPubMed
Muller, J, Kuhn, A, Vogt, M, et al. Improvements in exercise performance after surgery for Ebstein anomaly. J Thorac Cardiovasc Surg 2011; 141(5): 1192–1195. doi: 10.1016/j.jtcvs.2010.08.083[published Online First: 2010/11/26]CrossRefGoogle ScholarPubMed
MacLellan-Tobert, SG, Driscoll, DJ, Mottram, CD, et al. Exercise tolerance in patients with Ebstein’s anomaly. J Am Coll Cardiol 1997; 29(7): 1615–1622.CrossRefGoogle ScholarPubMed
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: 131–137. doi: 10.1016/j.ijcard.2016.01.032 [published Online First: 2016/01/21]CrossRefGoogle ScholarPubMed
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): 329–341.2.0.CO;2>CrossRefGoogle Scholar
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): 611–622.CrossRefGoogle Scholar
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): 800–812.CrossRefGoogle Scholar