Extracardiac Conduit Fontan - Outcome Data in Early Adulthood

Abstract

Background: To describe the survival and health outcome status of young adults with an extracardiac Fontan procedure performed either as a primary or conversion (secondary) Fontan surgery.

Methods: The database of the Adult Congenital Heart disease service at the Royal Melbourne Hospital was interrogated to identify all adults who had undergone a primary extracardiac conduit Fontan (n=29) or a Fontan conversion with this procedure (n=8). We then determined vital status, age, original anatomy and functional status in early adulthood in both groups.

Results: Adults with an ECC Fontan procedure report reasonable NYHA functional class (84% NYHA I or II) though, objectively, exercise testing demonstrates a reduced exercise capacity, and desaturation on exertion is frequent. The majority (86%) have completed secondary education. Most (78%) are managed on warfarin and there is a preponderance of ACE inhibition use (62%). Atrial arrhythmias have been documented in 5 of the 29 primary ECC groups (17%); in 3 patients this preceded primary ECC and 2 patients developed post primary ECC (between 6 and 14 years postoperatively). At a lesser time of follow-up [median 4.5 years (IQR 3.3-6)], conversion to an ECC as a secondary Fontan procedure has successfully treated atrial arrhythmias in the 7 (of 8) patients where this was the surgical indication for conversion.

Conclusions: Though long-term data will require decades to establish, in young adulthood the functional outcomes of a primary ECC Fontan operation are encouraging. Secondary ECC conversion successfully mitigates atrial arrhythmias in the short to medium term.

Keywords: Congenital heart defect; Echocardiography; Exercise test; Fontan procedure; Survival; Young adult.