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
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Article info
Publication history
Published online: May 03, 2017
Accepted:
March 20,
2017
Received:
November 14,
2016
Identification
Copyright
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.
