The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes

Iyengar, Ajay J., Winlaw, David S., Galati, John C., Wheaton, Gavin R., Gentles, Thomas L., Grigg, Leeanne E., Justo, Robert N., Radford, Dorothy J., Weintraub, Robert G., Bullock, Andrew, Celermajer, David S. and d'Udekem, Yves (2014) The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes. European Journal of Cardio-thoracic Surgery, 46 3: 465-473. doi:10.1093/ejcts/ezu015


Author Iyengar, Ajay J.
Winlaw, David S.
Galati, John C.
Wheaton, Gavin R.
Gentles, Thomas L.
Grigg, Leeanne E.
Justo, Robert N.
Radford, Dorothy J.
Weintraub, Robert G.
Bullock, Andrew
Celermajer, David S.
d'Udekem, Yves
Title The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes
Journal name European Journal of Cardio-thoracic Surgery   Check publisher's open access policy
ISSN 1873-734X
1010-7940
Publication date 2014-09-01
Year available 2014
Sub-type Article (original research)
DOI 10.1093/ejcts/ezu015
Volume 46
Issue 3
Start page 465
End page 473
Total pages 9
Place of publication Oxford, United Kingdom
Publisher Oxford University Press
Collection year 2015
Language eng
Formatted abstract
Objectives: To identify factors associated with hospital and long-term outcomes in a binational cohort of extracardiac conduit (ECC) Fontan recipients.

Methods: All patients who underwent an ECC Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified, and perioperative, follow-up, echocardiographic and reintervention data collected. Risk factors for early and late mortality, failure and adverse outcomes were analysed.

Results: A total of 570 patients were identified, and late follow-up was available in 529 patients. The mean follow-up was 6.7 years (standard deviation: 3.5) and completeness of the follow-up was 98%. There were seven hospital mortalities (1%) and 21 patients (4%) experienced early failure (death, Fontan takedown/revision or mechanical circulatory support). Prolonged length of stay occurred in 10% (57 patients), and prolonged effusions in 9% (51 patients). Overall survival at 14 years was 96% (95% confidence interval [CI]: 93-98%), and late survival for patients discharged with intact Fontan was 98% (95% CI: 94-99%). The rates of late failure (late death, transplantation, takedown, New York Heart Association class III/IV or protein-losing enteropathy) and adverse events (late failure, reoperation, percutaneous intervention, pacemaker, thromboembolic event or supraventricular tachycardia) per 100 patient-years were 0.8 and 3.8, and their 14-year freedoms were 83% (95% CI: 70-91%) and 53% (95% CI: 41-64%), respectively. After adjustment for confounders, hypoplastic left heart syndrome (HLHS) was strongly associated with prolonged effusions (OR: 2.9, 95% CI: 1.4-5.9), late failure (hazard ratio [HR]: 2.8, 95% CI: 1.1-7.5) and adverse events (HR: 3.6, 95% CI: 1.3-7.5).

Conclusions: The extracardiac Fontan procedure provides excellent survival into the second decade of life, but half of patients will suffer a late adverse event by 14 years. Patients with HLHS are at higher risk of late adverse events than other morphological groups, but their survival is still excellent.
Keyword Fontan procedure
Congenital heart disease
Retrospective studies
Survival rate
Follow-up studies
Disease-free survival
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Non HERDC
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