OBJECTIVES
There is dearth of data on long-term outcomes of systemic semilunar (SS) or atrioventricular (AV) valve operation in adult patients with a Fontan circulation.
We describe a single centre experience of adults who underwent valve surgery late after a Fontan procedure.
METHODS
We retrospectively reviewed all patients with a Fontan circulation who had a reoperation for severe valve disease during adulthood in our centre. Demographic, anatomical, clinical and periprocedural data and long-term outcome were retrospectively retrieved from our dedicated electronic hospital database and examined.
RESULTS
Out of 233 adults with a Fontan operation, 8 (7 males [88%]) had valve surgery during the study period. Indication for surgery was severe symptomatic valve regurgitation in all patients (of the SS valve in 7 cases and of the left AV valve in 1). Mechanical prosthesis was implanted in all cases. At a median follow-up of 10 years (IQR 5-20), one patient died due to hepatitis C virus infection. All the remaining 7 patients experienced a significant improvement of the NYHA class compared to the pre-operative state(p = 0.015); echocardiography showed stable systemic ventricle ejection fraction in absence of volume overload (50.1 ± 7.9% vs 51.6 ± 8.7%, p = 0.399) and well-functioning prostheses. No patient needed further operation nor heart transplantation.
CONCLUSIONS
Surgical SS valve replacement late after a Fontan operation in highly selected adult patients with valvular regurgitation performed in a tertiary dedicated centre resulted to be safe in our population and conveyed clinical benefit. Close monitoring of all Fontan patients developing valvular dysfunction is warranted, whereas future prospective research may refine selection criteria, optimal timing and outcomes.