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Long-term Outcome of Fontan-Associated Protein-Losing Enteropathy: Treatment Modality and Predictive Factor of
Ja-Kyoung Yoon1, Gi Beom Kim2, Mi Kyoung Song2
1Department of Pediatrics, Sejong General Hospital, Bucheon, Korea.
Insights
Protein-losing enteropathy (PLE) after the Fontan operation is a serious complication. While survival has improved with conservative care, treatments like heparin show promise for resolving PLE in some patients.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Gastroenterology
Background:
- Protein-losing enteropathy (PLE) is a significant complication following the Fontan operation.
- Fontan-associated PLE affects approximately 4.6% of patients, diagnosed at a mean age of 11.6 years.
Purpose of the Study:
- To investigate the clinical characteristics of Fontan-associated PLE.
- To evaluate treatment responses and outcomes in patients with Fontan-associated PLE.
Main Methods:
- Retrospective review of medical records for 38 patients with Fontan-associated PLE.
- Data collected from 1992 to 2018 across two institutions in Korea.
Main Results:
- Survival rates were 81.6% at 5 years and 76.5% at 10 years.
- NYHA class III/IV, low aortic oxygen saturation, and ventricular dysfunction predicted mortality.
- PLE resolved in 10 patients; initial heparin response was associated with survival, and heparin resolved PLE in 4 patients.
Conclusions:
- Survival rates for Fontan-associated PLE have improved due to advancements in conservative care.
- While no definitive treatment exists, some interventions resolved PLE in approximately one-fourth of patients.
- Further research is needed to establish optimal prevention and therapeutic strategies for Fontan-associated PLE.
Background And Objectives:
Protein-losing enteropathy (PLE) is a devastating complication after the Fontan operation. This study aimed to investigate the clinical characteristics, treatment response, and outcomes of Fontan-associated PLE.
Methods:
We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea.
Results:
PLE occurred in 4.6% of the total 832 patients after the Fontan operation. After a mean period of 7.7 years after Fontan operation, PLE was diagnosed at a mean age of 11.6 years. The mean follow-up period was 8.9 years. The survival rates were 81.6% at 5 years and 76.5% at 10 years. In the multivariate analysis, New York Heart Association Functional classification III or IV (p=0.002), low aortic oxygen saturation (<90%) (p=0.003), and ventricular dysfunction (p=0.032) at the time of PLE diagnosis were found as predictors of mortality. PLE was resolved in 10 of the 38 patients after treatment. Among medical managements, an initial heparin response was associated with survival (p=0.043). Heparin treatment resulted in resolution in 4 patients. We found no evidence on pulmonary vasodilator therapy alone. PLE was also resolved after surgical Fontan fenestration (2/6), aortopulmonary collateral ligation (1/1), and transplantation (1/1).
Conclusions:
The survival rate of patients with Fontan-associated PLE has improved with the advancement of conservative care. Although there is no definitive method, some treatments led to the resolution of PLE in one-fourth of the patients. Further investigations are needed to develop the best prevention and therapeutic strategies for PLE.
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