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.
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.
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.
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.
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Published online: May 03, 2017
Accepted: March 20, 2017
Received: November 14, 2016
Crown Copyright © 2017 Published by Elsevier B.V. on behalf of Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.