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Case Series: Heart Transplantation After Fontan Operation-Single-Center Experience
S-N Lin1, S-C Huang1, Y-S Chen1
1Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan.
Insights
Heart transplantation is a successful therapy for rare Fontan failure (FF). While survival rates are high, protein-losing enteropathy may persist post-transplant.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiac Surgery
Background:
- Fontan failure (FF) is a rare but serious complication following the Fontan operation.
- Heart transplantation is considered the primary therapeutic option for patients with FF.
- This study reviews the experience of heart transplantation in patients with prior Fontan procedures.
Observation:
- Four patients with FF underwent orthotopic heart transplantation (OHT) between 1987 and 2014.
- Data collected included clinical history, laboratory results, surgical techniques, perioperative variables, and long-term outcomes.
- Primary outcomes assessed were hospital mortality, and 1- and 4-year survival rates.
Findings:
- Orthotopic heart transplantation (OHT) in FF patients had 0% hospital mortality.
- One-year survival was 100%, and 4-year survival was 50%.
- One patient succumbed to post-transplantation lymphoproliferative disorder; protein-losing enteropathy improved in one of three patients.
Implications:
- Heart transplantation is technically feasible and offers survival benefits for patients with Fontan failure.
- Protein-losing enteropathy may not resolve rapidly after heart transplantation, requiring continued management.
- Successful OHT in Fontan patients underscores the importance of advanced cardiac surgical interventions.
Background:
Fontan failure (FF) occurs rarely. In patients with Fontan failure, heart transplantation is believed to be the most effective therapy. We review our experience in heart transplantations after the Fontan operation.
Methods:
From July 1987 to December 2014, 4 of 513 patients underwent orthotopic heart transplantation (OHT). Among them, 4 were due to FF. We reviewed these 4 cases via retrospective chart review. Clinical history, laboratory data, surgical technique, perioperative variables, and outcomes of long-term follow-up are presented herein. The primary outcomes were hospital mortality, 1-year-survival rate, and 4-year-survival rate. The secondary outcome is the improvement in patients with protein-losing enteropathy.
Results:
The hospital mortality rate was 0% in the 4 FF patients receiving OHT. No surgically related hemorrhage or infection was observed. The 1-year-survival rate was 100% (n = 4) and the 4-year-survival rate 50% (n = 2). One patient died of posttransplantation lymphoproliferative disorder. Hypoalbuminemia improved in 1 of 3 patients 4 months after OHT.
Conclusions:
Despite technical challenges, heart transplantation can be performed successfully in patients with Fontan operation. However, protein-losing enteropathy might not be resolved quickly after heart transplantation.
