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Effect of anti-heart failure therapy on diastolic function in children with single-ventricle circulations

Published online by Cambridge University Press:  05 March 2015

Deane L.S. Yim ,
Bryn O. Jones ,
Peta M.A. Alexander ,
Yves d’Udekem  and
Michael M.H. Cheung
Deane L.S. Yim
Affiliation:
Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia
Bryn O. Jones
Affiliation:
Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Heart Research Group, Murdoch Children’s Research Institute, Victoria, Australia
Peta M.A. Alexander
Affiliation:
Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia Heart Research Group, Murdoch Children’s Research Institute, Victoria, Australia
Yves d’Udekem
Affiliation:
Department of Paediatrics, The University of Melbourne, Melbourne, Australia Heart Research Group, Murdoch Children’s Research Institute, Victoria, Australia Department of Cardiac Surgery, Royal Children’s Hospital, Melbourne, Australia
Michael M.H. Cheung*
Affiliation:
Department of Cardiology, Royal Children’s Hospital, Melbourne, Australia Department of Paediatrics, The University of Melbourne, Melbourne, Australia Heart Research Group, Murdoch Children’s Research Institute, Victoria, Australia
*
Correspondence to: A/Prof. M. Cheung, Department of Cardiology, Royal Children’s Hospital Flemington Road, Parkville Melbourne, VIC 3052, Australia. Tel: +61 3 93455718; Fax: +61 3 93456001; E-mail: [email protected]
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Abstract

Background

Children with functionally univentricular circulations have chronic volume loading of the systemic ventricle, potentially affecting ventricular function. Medications including angiotensin-converting enzyme inhibitors and β-blockers are used to treat ventricular dysfunction, despite limited evidence of their efficacy in this population.

Objective

To determine the effects of angiotensin-converting enzyme inhibitors on elevated filling pressures in children with single ventricle physiology.

Methods

We performed a single-centre, retrospective review of patients with single ventricle physiology who underwent multiple cardiac catheterisations between 1991 and 2013. Study population comprised of patients who commenced or had optimised dosing of angiotensin-converting enzyme inhibitors between assessments in response to high ventricular filling pressures. Patients undergoing interventions influencing loading conditions between assessments were excluded.

Results

A total of 17 patients were identified, with dominant morphologic right ventricle in eight patients (47.1%). Among them, 11 (64.7%) were pre-Fontan and six (35.3%) were post-Fontan completion. Median inter-assessment interval was 9.4 months (range 7.3–19.1). There was a reduction in end-diastolic pressure from 13 to 10 mmHg (p=0.002), mean pulmonary artery pressure from 16 to 13 mmHg (p=0.049), and mean atrial pressure from 12 to 9 mmHg (p=0.001). There was one cardiac transplant, and there were no patient deaths at median follow-up after 31 months.

Conclusions

We observed a reduction in ventricular end-diastolic pressure, pulmonary artery pressure, and mean atrial pressure following treatment with angiotensin-converting enzyme inhibitors in patients with single ventricle physiology. Our study provides insights into the potential impact of anti-heart failure therapy in single ventricle circulations and calls for larger, controlled studies to assess for a therapeutic response.

Keywords

Fontandiastolic functionsingle ventricleuniventricularACE inhibitor
Type
Original Articles
Information
Cardiology in the Young , Volume 25 , Issue 7 , October 2015 , pp. 1293 - 1299
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Copyright
© Cambridge University Press 2015 

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