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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: Vandorn.charlotte@mayo.edu
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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
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Copyright
© Cambridge University Press 2019 

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