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Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome
Hsin-Chia Lin1, Mei-Hwan Wu2, Jou-Kou Wang2
1Department of Pediatrics, National Taiwan University Children's Hospital, Taipei, Taiwan; Department of Pediatrics, National Taiwan University Hospital Yunlin Branch, Yunlin, Taiwan.
Insights
The Fontan operation shows improved outcomes, but patients with heterotaxy syndrome (HS) have poorer perioperative results. However, HS is not a predictor of mortality after accounting for hemodynamic factors.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Surgery
- Cardiac Physiology
Background:
- The Fontan operation is a critical palliative procedure for single-ventricular complex congenital heart diseases (SV-CHD).
- Heterotaxy syndrome (HS) presents unique challenges in patients undergoing complex cardiac surgeries.
Purpose of the Study:
- To evaluate perioperative outcomes after the Fontan operation.
- To assess the specific impact of heterotaxy syndrome (HS) on Fontan operation outcomes in a pediatric tertiary center.
Main Methods:
- Retrospective review of medical records for patients undergoing Fontan operation between 1997 and 2017.
- Analysis of preoperative, operative, and postoperative risk factors for mortality and morbidity.
- Multivariable regression analysis to identify predictors of outcomes.
Main Results:
- Overall perioperative event-free survival to discharge was 91.6%, with lower survival in HS patients (84.3%) compared to other SV-CHD (95.1%).
- Postoperative arrhythmia was significantly more common in the HS group (31.4% vs. 12.6%).
- Heterotaxy syndrome was not a predictor of worse outcomes after adjusting for preoperative pulmonary artery pressure and operation era.
Conclusions:
- Surgical outcomes for the Fontan operation have significantly improved over time.
- While perioperative outcomes are poorer in patients with HS, it is not an independent predictor of mortality when hemodynamic factors are considered.
Background/Purpose:
Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.
Methods:
Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.
Results:
Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.
Conclusion:
Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors.
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