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Late surgical fenestration for complications after the Fontan operation
J Rychik1, J J Rome, M L Jacobs
1Division of Cardiology, Children's Hospital of Philadelphia 19104, USA. jrychik@mail.med.upenn.edu
Insights
Late fenestration creation can resolve complications after Fontan operations. This surgical intervention improves outcomes for patients experiencing chronic effusions or protein-losing enteropathy, with success linked to the shunt size.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Surgical Intervention
Background:
- Fontan operation is associated with significant long-term morbidity.
- Initial fenestration creation reduces early morbidity and mortality.
- The efficacy of late fenestration in nonfenestrated Fontan patients is uncertain.
Purpose of the Study:
- To evaluate the effectiveness of late surgical fenestration in patients with post-Fontan morbidity.
- To assess outcomes in patients with chronic effusions and protein-losing enteropathy (PLE).
Main Methods:
- Retrospective review of 9 patients (5.2 ± 3.1 years) undergoing late fenestration.
- Fenestration created using coronary punch (3-mm defects for effusions, 5-mm for PLE).
- Follow-up ranged from 25 to 30 months.
Main Results:
- 3 of 4 patients with effusions resolved symptoms; 1 required re-fenestration.
- 3 of 5 patients with PLE achieved symptom resolution and normalized serum proteins.
- Two failures in PLE group had post-operative saturations >89%; successful cases had saturations ≤85%.
Conclusions:
- Late surgical fenestration can effectively resolve morbidity after Fontan operation.
- Patient improvement correlates with the degree of right-to-left shunt achieved.
- Fenestration patency and clinical improvement were sustained in successful PLE cases.
Background:
Significant morbidity after Fontan operation results in either takedown, heart transplantation, or death. Initial creation of a fenestration results in less morbidity and mortality; however, the role of late creation of a fenestration in aiding patients manifesting morbidity after an initial nonfenestrated Fontan operation is unclear.
Methods And Results:
We reviewed our experience with late creation of a surgical fenestration in 9 patients (5.2 +/- 3.1 years old) exhibiting chronic effusions (n = 4) or protein-losing enteropathy (PLE) (n = 5) after lateral tunnel-type Fontan operation. Patients with effusions had creation via coronary punch of two or three 3-mm defects; patients with PLE had creation of a large, 5-mm defect. One child with effusions and multisystem organ failure before fenestration died 7 weeks after surgery secondary to low cardiac output; the other 3 had resolution of effusions within 4 to 6 weeks. Of the 5 with PLE, 3 had normalization of serum proteins and resolution of symptoms at 2 to 6 weeks. The 2 failures had arterial saturations > 89% after surgery. Follow-up was from 25 to 30 months. Spontaneous closure of defects occurred in all 3 with effusions. No return of symptoms was noted in 2; however, the third reaccumulated effusions and has undergone refenestration with a large defect. All 3 patients with PLE have remained asymptomatic with patency of the fenestration (4 to 5 mm on echocardiography) and arterial saturation < or = 85% for > 2 years.
Conclusions:
Late surgical creation of fenestration results in resolution of morbidity after Fontan operation. Improvement is related to the degree of right-to-left shunt created.