title: Late surgical fenestration for complications after the Fontan operation.
abstract: 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.
  
  
  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.
authors.affiliation: Division of Cardiology, Children's Hospital of Philadelphia 19104, USA. jrychik@mail.med.upenn.edu
authors.forename: J
authors.initials: J
authors.lastname: Rychik
authors.forename: J J
authors.initials: JJ
authors.lastname: Rome
authors.forename: M L
authors.initials: ML
authors.lastname: Jacobs
mesh_terms: D006328:Cardiac Catheterization; D002648:Child; D002675:Child, Preschool; D002908:Chronic Disease; D004452:Echocardiography; D005500:Follow-Up Studies; D018729:Fontan Procedure / Q000009:adverse effects*; D006325:Heart Atria / Q000601:surgery*; D006801:Humans; D010996:Pleural Effusion / Q000000981:diagnostic imaging / Q000209:etiology / Q000517:prevention & control*; D011504:Protein-Losing Enteropathies / Q000209:etiology / Q000517:prevention & control*; D012086:Reoperation