Korean Circ J.  2022 Aug;52(8):606-620. 10.4070/kcj.2021.0309.

Long-term Outcome of Fontan-Associated Protein-Losing Enteropathy: Treatment Modality and Predictive Factor of Mortality

  • 1Department of Pediatrics, Sejong General Hospital, Bucheon, Korea
  • 2Department of Pediatrics, Seoul National University Children’s Hospital, Seoul National University College of Medicine, Seoul, Korea
  • 3Department of Thoracic and Cardiovascular Surgery, Seoul National University Children’s Hospital, Seoul, Korea
  • 4Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Bucheon, Korea
  • 5Department of Pediatrics, Gachon University Gil Medical Center, Incheon, Korea


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.
We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea.
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).
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.


Fontan procedure; Protein-losing enteropathies; Survival rate; Risk factors; Treatment outcome


  • Figure 1 Overall survival curve in patients with PLE after the Fontan operation.PLE = protein losing enteropathy.

  • Figure 2 Comprehensive evaluation and multidisciplinary treatment for Fontan-associated PLE.AV = atrioventricular; CT = computed tomography; MRI = magnetic resonance imaging; PLE = protein losing enteropathy.

Cited by  1 articles

Protein-Losing Enteropathy: A Big Challenge in Fontan Circulation
Jae Young Lee
Korean Circ J. 2022;52(8):621-622.    doi: 10.4070/kcj.2022.0166.


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