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Published on: May 2, 2025
Protocols associated with no mortality in 100 consecutive Fontan procedures
Marshall L Jacobs1, Glenn J Pelletier, Kamal K Pourmoghadam
1The Heart Center for Children, St. Christopher's Hospital for Children, Drexel University College of Medicine, Erie Avenue at Front Street, Philadelphia, PA 19134, USA. marshall.jacobs@tenethealth.com
Insights
This study demonstrates a zero-mortality rate for 100 consecutive Fontan procedures using specific operative and intensive care protocols. These strategies successfully managed high-risk pediatric cardiac surgery patients, including those with complex anatomy.
Area of Science:
- Pediatric Cardiac Surgery
- Congenital Heart Disease Management
- Cardiovascular Surgery Outcomes
Background:
- The Fontan procedure is a complex surgery for single-ventricle congenital heart defects.
- While outcomes have improved, perioperative mortality remains a concern due to complications like ventricular dysfunction and multi-organ failure.
- Optimized perioperative management protocols are crucial for improving Fontan procedure survival rates.
Purpose of the Study:
- To evaluate the efficacy of a standardized operative and intensive care unit (ICU) management protocol for the Fontan procedure.
- To assess the feasibility of achieving zero mortality in a consecutive series of Fontan operations, including high-risk patients.
Main Methods:
- A cohort of 100 consecutive Fontan operations were performed between 1996 and 2006.
- The protocol involved specific surgical techniques such as aortic and single atrial cannulation, hypothermic circulatory arrest, and avoidance of central venous lines.
- Postoperative care included prolonged inotropic/vasodilator support, exclusive aspirin use for anti-thrombotic therapy, and small pigtail catheters for pleural drainage.
Main Results:
- The study achieved zero mortality in 100 consecutive Fontan operations.
- All patients were extubated on postoperative day 1, with an average hospital stay of 10 days.
- Minor complications included bleeding, reintubation, pericardial effusion, and seizures; no high-risk candidates were excluded.
Conclusions:
- The implemented operative and ICU management strategies enable the safe performance of the Fontan procedure in diverse and high-risk patient groups.
- These protocols have demonstrated the potential to eliminate operative mortality in consecutive Fontan operations.
Objectives:
Results of Fontan's procedure have improved considerably, but perioperative mortality still occurs, attributed to ventricular dysfunction, stroke, arrhythmia, thromboembolism, and multi-organ dysfunction. Our protocols of operative and intensive care unit management address these potential issues, and have been associated with zero mortality, even with many high-risk candidates.
Methods:
From 1996 to 2006, all Fontan patients were managed as follows: operative strategy based on aortic and single atrial cannulation, cooling on full-flow bypass, and hypothermic circulatory arrest to create the Fontan pathway. No direct caval cannulation. Use of central venous lines was completely avoided. Fresh whole blood was used for pump prime and for volume restoration. Inotropic and vasodilator therapy was continued for at least 48 h. Aspirin was used exclusively as anti-thrombotic therapy. Postoperative pleural drainage was accomplished with small pigtail catheters. The usual Fontan pathway was by lateral atrial tunnel (84), with extra-cardiac conduit when dictated by anatomy (16).
Results:
One hundred Fontan operations were performed with no mortality. All patients were extubated by postoperative day 1. Hospital stay was 10+/-5 days. Complications were: bleeding (1), reintubation (1), emergent fenestration closure (1), pericardial effusion (4), and seizures (1). Risk factors included Fontan connection to one lung (3), diminutive pulmonary arteries (PAs) and unifocalized major aortopulmonary collateral arteries (MAPCAs) (1), discontinuous PAs (3), right ventricle dependent coronaries (3), neonatal pulmonary venous obstruction (3), Trisomy 21 (1), preoperative pacemaker dependence (2), and heterotaxy (10). No candidate was excluded.
Conclusions:
While many surgeons try to avoid bypass or aortic clamping when performing Fontan operations, the strategies we have employed facilitate safe accomplishment of Fontan's operation in diverse anatomic groups with multiple risk factors, with avoidance of operative mortality in 100 consecutive cases.
