Surgical valve replacement in adults late after total cavo-pulmonary connection/Fontan procedure

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.

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.

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 seven cases and of the left AV valve in 1). Mechanical prosthesis was implanted in all cases. At a median follow-up of 10 years (interquartile range 5-20), one patient died due to hepatitis C virus infection. All the remaining 7 patients experienced a significant improvement of the New York Heart Association 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.

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.