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. 2022 Aug 3;35(3):ivac098.
doi: 10.1093/icvts/ivac098.

Surgical and interventional rescue strategies for Fontan failure

Gregor Gierlinger  1   2 , Eva Sames-Dolzer  1   2 , Michaela Kreuzer  1   2 , Roland Mair  1   2 , Mohammad-Paimann Nawrozi  3 , Andreas Tulzer  2   4 , Christoph Bauer  2   4 , Gerald Tulzer  4 , Rudolf Mair  1
Affiliations

Surgical and interventional rescue strategies for Fontan failure

Gregor Gierlinger et al. Interact Cardiovasc Thorac Surg. .

Abstract

Objectives: Fontan patients are at lifelong risk for developing complications, which may result in Fontan failure. Survival rates after heart transplantation (HTX) are still unsatisfying in these patients. Long-term survival of extracardiac Fontan patients in the modern era was investigated. The objective of this study was to investigate if surgical and interventional procedures in patients with protein-losing enteropathy (PLE) and/or plastic bronchitis (PB) and a failing Fontan circulation can postpone or avoid HTX.

Methods: Retrospective data collection of all children who underwent a Fontan procedure between January 1999 and July 2021 at our centre was performed. Patients were surveyed regarding the occurrence of PLE or PB and their outcome was reported descriptively. HTX-free survival of patients who underwent a rescue procedure due to PLE/PB was evaluated.

Results: Three hundred and seventy [94.1% (95% confidence interval, 91.4-96.3)] Fontan patients were free of HTX or death at last follow-up after a median follow-up time of 6.7 years. PB/PLE was diagnosed in 34 patients during the observation period. A rescue procedure was undertaken in 16 pts. at a median time of 6.5 months (range: 1 day to 9.4 years) since the initial diagnosis of PLE/PB. In these patients, HTX-free survival was 75% (95% confidence interval, 47.6-92.7) at a median follow-up time of 4.0 years after the procedure. Range: 3.5 months to 13.9 years.

Conclusions: Extracardiac Fontan patients in the modern era expect reasonable HTX-free survival rates. Surgical and/or interventional rescue strategies for Fontan failure can postpone HTX for a sustained period of time.

Keywords: Fontan failure; Fontan operation; Plastic bronchitis; Protein-losing enteropathy; Single ventricle palliation.

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Figures

Figure 1:
Figure 1:
Open fenestration after Fontan procedure.
Figure 2:
Figure 2:
Lymphatic fistula draining into the intestine.
Figure 3:
Figure 3:
Thoracic duct decompression procedure, reprinted from Bauer et al. [14] by permission of the European Society of Cardiology.
Figure 4:
Figure 4:
(A, B) Long-term survival after diagnosis of PLE/PB. HTX: heart transplantation; PB: plastic bronchitis; PLE: protein-losing enteropathy.
Figure 5:
Figure 5:
Long-term survival after Fontan rescue therapy. HTX: heart transplantation.
None
See this image and copyright information in PMC

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