Reintervention and survival in 1428 patients in the Australian and New Zealand Fontan Registry

Heart. 2020 May;106(10):751-757. doi: 10.1136/heartjnl-2019-315430. Epub 2019 Oct 29.


Objective: Patients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients.

Methods: A retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival.

Results: Of the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%.

Conclusions: A substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.

Keywords: complex congenital heart disease; congenital heart disease; congenital heart disease surgery; fontan physiology.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Australia / epidemiology
  • Cost of Illness
  • Female
  • Fontan Procedure* / adverse effects
  • Fontan Procedure* / methods
  • Fontan Procedure* / statistics & numerical data
  • Heart Defects, Congenital* / mortality
  • Heart Defects, Congenital* / surgery
  • Humans
  • Long Term Adverse Effects* / epidemiology
  • Long Term Adverse Effects* / surgery
  • Longitudinal Studies
  • Male
  • Mortality
  • Needs Assessment
  • New Zealand / epidemiology
  • Pacemaker, Artificial / statistics & numerical data
  • Registries / statistics & numerical data
  • Reoperation / statistics & numerical data