Long-term Outcome after Extracardiac Fontan Procedure Performed during Adulthood or Adolescence

Background: In univentricular patients the extracardiac Fontan operation is usually performed at the age of 2–5 years. Various reasons may lead to an incomplete Fontan circulation until adolescence or even adulthood in isolated cases. The aim of this retrospective single center study: Is the extracardiac Fontan procedure able to sustainably improve quality of life if performed in patients aged 16 years or older?

Methods: Between 07/1999 and 07/2021 in total 401 Fontan procedures have been performed in our center, 8 of these patients were 16 years or older at the time of operation. Their median age was 19 [17.8;22.4] years, weight 61 [47;58] kg. All of them had at least one previous operation. The mean preoperative saturation at rest was 81% (range 64–91%), NYHA class III-IV. In 5 patients we used a 24mm, in 3 patients a 22mm PTFE prosthesis as an extracardiac conduit. In 7 cases a fenestration was made. Together with the Fontan procedure several other procedures were performed: Take down of all shunts/ additional pulmonary blood flow (7 pts), ligation of vena azygos ± hemiazygos (3 pts), pulmonary artery patch plasty (2 pts), excision of interatrial septum, aortic arch repair, atrioventricular valve repair, confluence anastomosis (1 pt each). Median cardiopulmonary bypass time was 233 [208;298] min, aortic cross clamp time in 3 patients 23, 79, and 134 minutes.

Results: Median stay on intensive care unit was 3 [1;5] days, in hospital 30 [16;47] days. No patient died during the hospital stay. Median follow-up period was 15.7 [13.3;17.6] years. 2 fenestrations closed spontaneously, 4 were closed interventionally after 49–174 days. There was one case of late mortality 7.7 years postoperatively, the man died suddenly at home. At their last examination 7 patients presented with NYHA class I–II, one patient with II–III. The mean saturation at rest was 93% (range 88–95). One woman was pregnant, 1 man is pediatric surgeon.

Conclusion: The extracardiac Fontan procedure can safely be performed in adulthood or late adolescence if the preoperative requirements for Fontan completion are fulfilled. In our cohort, the improvement of the quality of life was impressive.

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Artikel online veröffentlicht:
13. Februar 2024