Use of ACE inhibitors in Fontan: Rational or irrational?

Wilson, Thomas G., Iyengar, Ajay J., Winlaw, David S., Weintraub, Robert G., Wheaton, Gavin R., Gentles, Thomas L., Ayer, Julian, Grigg, Leeanne E., Justo, Robert N., Radford, Dorothy J., Bullock, Andrew, Celermajer, David S., Dalziel, Kim, Schilling, Chris and d'Udekem, Yves (2016) Use of ACE inhibitors in Fontan: Rational or irrational?. International Journal of Cardiology, 210 95-99. doi:10.1016/j.ijcard.2016.02.089

Author Wilson, Thomas G.
Iyengar, Ajay J.
Winlaw, David S.
Weintraub, Robert G.
Wheaton, Gavin R.
Gentles, Thomas L.
Ayer, Julian
Grigg, Leeanne E.
Justo, Robert N.
Radford, Dorothy J.
Bullock, Andrew
Celermajer, David S.
Dalziel, Kim
Schilling, Chris
d'Udekem, Yves
Title Use of ACE inhibitors in Fontan: Rational or irrational?
Journal name International Journal of Cardiology   Check publisher's open access policy
ISSN 0167-5273
Publication date 2016-05
Sub-type Article (original research)
DOI 10.1016/j.ijcard.2016.02.089
Volume 210
Start page 95
End page 99
Total pages 5
Place of publication Shannon, Clare, Ireland
Publisher Elsevier Ireland
Collection year 2017
Language eng
Formatted abstract
Despite a lack of evidence supporting the use of angiotensin-converting enzyme (ACE) inhibitors in patients with a Fontan circulation, their use is frequent. We decided to identify the rationale for ACE inhibitor therapy in patients within the Australia and New Zealand Fontan Registry.

All patients in the Registry taking an ACE inhibitor at last follow up were identified, and a review of medical records was undertaken to determine the rationale for treatment initiation and reasons for treatment continuation or dose increase.

In 2015, 36% of the surviving patients in the Registry (462/1268) were taking an ACE inhibitor. Indications for initiation of therapy were ventricular systolic or diastolic dysfunction (29%), atrioventricular valve regurgitation (19%), preservation of normal ventricular function (7%), prolonged effusions at Fontan (6%), hypertension (6%), other (6%) and unknown (2%). No indication was stated in the remaining patients (25%). Those with hypoplastic left heart syndrome were more likely to be on an ACE inhibitor than those with an alternative primary morphology (70% vs 32%; p < 0.001). Only 36% of the patients treated with an ACE inhibitor at last follow up (166/462) had an indication that would generally justify treatment in a two-ventricle circulation.

It is likely that the use of ACE inhibitors in patients with a Fontan circulation is excessive within our region. The coordination of prospective, multicentre studies and initiatives such as the Australia and New Zealand Fontan Registry will facilitate further investigations to guide treatment decisions in the growing Fontan population.
Keyword Fontan procedure
Heart single ventricle
Angiotensin-converting enzyme inhibitors
Heart ventricles abnormalities
Heart defects congenital
Multicenter randomized-trial
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
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