Significant Improvements in Mortality After the Fontan Operation in Children With Down Syndrome

Ann Thorac Surg. 2020 Mar;109(3):835-841. doi: 10.1016/j.athoracsur.2019.07.085. Epub 2019 Sep 13.

Abstract

Background: Down syndrome (DS) is considered a risk factor for mortality associated with the Fontan operation. The objective was to show the contemporary short-term outcome of the Fontan operation for a functionally univentricular heart in patients with DS and non-DS, along with an analysis of significant predictors for in-hospital mortality.

Methods: This was a retrospective study using The Society of Thoracic Surgeons Congenital Database to assess in-hospital mortality and its predictors in patients with DS and non-DS undergoing the Fontan operation over 16 years (2001-2016). The primary outcome was in-hospital mortality. Statistical analysis was performed using univariable and multivariable logistic regression models.

Results: Our study cohort consisted of 12,074 patients (81 DS and 11,993 non-DS). The overall in-hospital mortality rate significantly improved in the recent era (2009-2016): 2.4% to 1.3%, P < .001. The DS group had a higher in-hospital mortality rate (12.3% vs 1.6%, P < .001) with an odds ratio of 8.6 (95% confidence interval, 4.4-17.0). The DS group had a higher 30-day mortality rate, a longer median postoperative length of stay, and a higher incidence of postoperative complications. The multivariable model showed that DS was the strongest predictor of in-hospital mortality, with an odds ratio of 11.6 (95% confidence interval, 5.1-26.4), adjusted for other significant variables including era effect, weight, and primary cardiac diagnosis.

Conclusions: The in-hospital mortality for the Fontan operation significantly improved in the contemporary era. DS was a significant risk factor for in-hospital morbidity and mortality associated with the Fontan operation.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple*
  • Child, Preschool
  • Down Syndrome / mortality*
  • Female
  • Follow-Up Studies
  • Fontan Procedure / methods*
  • Forecasting*
  • Heart Defects, Congenital / mortality
  • Heart Defects, Congenital / surgery*
  • Hospital Mortality / trends
  • Humans
  • Incidence
  • Male
  • Postoperative Complications / epidemiology*
  • Retrospective Studies
  • Risk Factors
  • Treatment Outcome
  • United States / epidemiology