Surgical management of failing Fontan circulation: results from 30 cases with 285 patient-years follow-up

Abstract   OBJECTIVES Fontan patients are known to suffer from clinical attrition over the years, which has been characterized as Fontan failure. We sought to evaluate the clinical outcomes of such Fontan patients undergoing surgical management in a 25-year, single-centre experience. METHODS A retro...

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Bibliographic Details
Published in:European journal of cardio-thoracic surgery 2022-01, Vol.61 (2), p.338-345
Main Authors: Padalino, Massimo A, Ponzoni, Matteo, Castaldi, Biagio, Leoni, Loira, Chemello, Liliana, Toscano, Giuseppe, Gerosa, Gino, Di Salvo, Giovanni, Vida, Vladimiro L
Format: Article
Language:English
Subjects:
Adult
Follow-Up Studies
Fontan Procedure - methods
Heart Defects, Congenital
Heart Transplantation - adverse effects
Humans
Postoperative Complications - epidemiology
Postoperative Complications - etiology
Postoperative Complications - surgery
Reoperation - adverse effects
Retrospective Studies
Treatment Outcome
Young Adult
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Summary:Abstract   OBJECTIVES Fontan patients are known to suffer from clinical attrition over the years, which has been characterized as Fontan failure. We sought to evaluate the clinical outcomes of such Fontan patients undergoing surgical management in a 25-year, single-centre experience. METHODS A retrospective single-centre analysis of patients undergoing surgical treatment for failing Fontan between 1995 and 2020, including any reoperations when ventricular function was preserved, or a heart transplant (HTx), when ventricular contractility was impaired. We analysed survival, indications for surgery and early and late complication rates. RESULTS We collected 30 patients (mean age 24.7 years) who required surgery after a mean time of 19.3 years from the original Fontan procedure: Fontan conversion in 21 (70%, extracardiac conduit in 19, lateral tunnel in 2), a HTx in 4 (13.3%) and other reoperations in 5 (16.7%). The most common indications for surgery were tachyarrhythmias (63.3%) and severe right atrial dilatation (63.3%). Overall survival at the 1-, 5-, 10- and 20-year follow-up examinations were 75.9% [95% confidence interval (CI): 91.4–60.4%], 75.9% (95% CI: 91.4–60.4%), 70% (95% CI: 78–52%) and 70% (95% CI: 78–52%), respectively. The most frequent complications were postoperative tachyarrhythmias (50%) and late Fontan-associated liver disease (56.5%). HTx and Fontan conversion provided comparably good outcomes compared to other reoperations (P = 0.022). CONCLUSIONS Surgery for failing Fontan can be performed effectively with overall good long-term survival. However, early and late morbidities are still a significant burden. Because other reoperations performed when patients presented with contraindications for a HTx have carried high mortality, close clinical follow-up is mandatory, and an earlier indication for Fontan conversion or a HTx is advisable to optimize outcomes. Since its first description in 1971, the Fontan operation has enabled thousands of children to survive life-threatening congenital heart diseases and reach adulthood.
ISSN:1010-7940
1873-734X
DOI:10.1093/ejcts/ezab450