Article Text

PDF

Original research article
Predictors of poor outcome among children with heterotaxy syndrome: a retrospective review
  1. Eiméar McGovern1,
  2. Eoin Kelleher1,
  3. James E Potts2,
  4. John O'Brien3,
  5. Kevin Walsh1,
  6. Lars Nolke1 and
  7. Colin J McMahon1
  1. 1Department of Paediatric Cardiology, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland
  2. 2Department of Cardiology, British Columbia Children's Hospital, Vancouver, British Columbia, Canada
  3. 3StatisticaMedica Ltd, Dublin, Ireland
  1. Correspondence to Dr Eiméar McGovern; emcgovern{at}doctors.org.uk

Abstract

Objective To determine predictors of poor outcome in patients with heterotaxy syndrome.

Methods A retrospective review of children with heterotaxy syndrome, in a single tertiary paediatric cardiology centre, was conducted between 1 January 1997 and 1 January 2014 to determine predictors of poor outcome. Poor outcome was defined as death, cardiac transplantation or New York Heart Association (NYHA) functional class III or IV.

Results There were 35 patients diagnosed with heterotaxy syndrome, 17 of whom were diagnosed antenatally. 22 patients had right atrial isomerism and 13 had left atrial isomerism. The median age of postnatal diagnosis was 2.5 days old (1 day to 19 months). 12 patients had a poor outcome; 6 patients died, 1 underwent cardiac transplantation and 5 had an NYHA functional class of >III. 5 patients had a biventricular repair and the remaining 30 had a univentricular repair. Type of atrial isomerism, univentricular or biventricular anatomy, severity of atrioventricular valve regurgitation or ventricular dysfunction, obstructed pulmonary venous return, occurrence of arrhythmia and presence of pulmonary atresia did not predict poor outcome. Fetal diagnosis also did not confer a survival advantage. The median duration of follow-up in this cohort was 65 months (2 days to 16.8 years).

Conclusions Survival for patients with heterotaxy syndrome was 83% over a median follow-up of 65 months. 34% of patients had a poor outcome. None of the variables studied were predictive of death, transplantation or NYHA classification III or IV.

  • CONGENITAL HEART DISEASE
  • Heterotaxy
  • Right atrial isomerism
  • Left atrial isomerism
  • Survival

This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/

View Full Text

Statistics from Altmetric.com

Footnotes

  • Competing interests None declared.

  • Ethics approval Our Lady's Hospital For Sick Children, Crumlin Ethics Committee.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement No additional data are available.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.