Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study

J Thorac Cardiovasc Surg. 2022 Mar;163(3):1166-1175. doi: 10.1016/j.jtcvs.2021.05.015. Epub 2021 May 18.


Objective: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect.

Methods: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation.

Results: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years.

Conclusions: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

Keywords: Fontan; single ventricle.

Publication types

  • Multicenter Study
  • Video-Audio Media

MeSH terms

  • Belgium / epidemiology
  • Cardiac Surgical Procedures* / adverse effects
  • Cardiac Surgical Procedures* / mortality
  • Cardiac Valve Annuloplasty
  • Databases, Factual
  • Female
  • Fontan Procedure
  • Heart Septal Defects / diagnostic imaging
  • Heart Septal Defects / mortality
  • Heart Septal Defects / physiopathology
  • Heart Septal Defects / surgery*
  • Hospital Mortality
  • Humans
  • Incidence
  • Male
  • Netherlands / epidemiology
  • Reoperation
  • Retrospective Studies
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • Treatment Outcome
  • Univentricular Heart / diagnostic imaging
  • Univentricular Heart / mortality
  • Univentricular Heart / physiopathology
  • Univentricular Heart / surgery*

Supplementary concepts

  • Atrioventricular Septal Defect