Objectives: Techniques and strategies in the Fontan procedure have evolved over the years, including the evolution from a one- to two-stage procedure. With such adjustments made, attention should shift towards survival analysis, including bidirectional cavopulmonary shunt (BCPS) and interstage mortality. The purpose of this study was to investigate the trends in the overall mortality of all patients who underwent a BCPS or one-stage Fontan procedure at our institution in the period of 1975-2011.
Methods: Using a single-institution, retrospective design, we reviewed the patient records of 203 patients from the University Medical Center Groningen, Netherlands, who underwent a Fontan procedure or a BCPS (with the intention to complete cavopulmonary connection at a second later stage) between 1975 and 2011. Trends in mortality were investigated by comparing survival rates during four consecutive decades (1975-84; 1985-94; 1995-2004; 2005-11), and predictors for mortality were identified.
Results: During a mean follow-up of 12 years, survival was 69%. Overall mortality declined significantly during the past decades (P = 0.017). This was driven by a decrease in early mortality (P = 0.016), whereas no changes in late mortality could be demonstrated. Multivariate analyses identified a diagnosis of heterotaxy (P = 0.049) and an atriopulmonary connection type of Fontan circulation (P = 0.015) as independent risk factors for overall mortality.
Conclusions: We demonstrate that, with the inclusion of first-stage and interstage mortality also, overall survival after Fontan procedures improved over time. This improvement, however, is mainly caused by a decline in early mortality. Improvement in long-term survival of patients operated on over the past four decades could not (yet) be demonstrated in this series.
Keywords: Congenital heart disease; Fontan; Survival.