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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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References

1. Senzaki, H, et al. Ventricular afterload and ventricular work in fontan circulation: comparison with normal two-ventricle circulation and single-ventricle circulation with blalock-taussig shunts. Circulation 2002; 105: 2885–2892.CrossRefGoogle ScholarPubMed
2. Cheung, YF, Penny, DJ, Redington, AN. Serial assessment of left ventricular diastolic function after Fontan procedure. Heart 2000; 83: 420–424.CrossRefGoogle ScholarPubMed
3. Garofalo, CA, et al. Ventricular diastolic stiffness predicts perioperative morbidity and duration of pleural effusions after the Fontan operation. Circulation 2006; 114 (Suppl): I56–I61.CrossRefGoogle ScholarPubMed
4. Graham, TP Jr., Johns, JA. Pre-operative assessment of ventricular function in patients considered for Fontan procedure. Herz 1992; 17: 213–219.Google ScholarPubMed
5. The SOLVD Investigators. Effect of enalapril on survival in patients with reduced left ventricular ejection fractions and congestive heart failure. N Engl J Med 1991; 325: 293–302.CrossRefGoogle Scholar
6. The CONSENSUS Trial Study Group. Effects of enalapril on mortality in severe congestive heart failure. Results of the Cooperative North Scandinavian Enalapril Survival Study (CONSENSUS). N Engl J Med 1987; 316: 1429–1435.CrossRefGoogle Scholar
7. Pouleur, H, et al. Effects of long-term enalapril therapy on left ventricular diastolic properties in patients with depressed ejection fraction. SOLVD Investigators. Circulation 1993; 88: 481–491.CrossRefGoogle Scholar
8. Bristow, MR, et al. Carvedilol produces dose-related improvements in left ventricular function and survival in subjects with chronic heart failure. MOCHA Investigators. Circulation 1996; 94: 2807–2816.CrossRefGoogle ScholarPubMed
9. Packer, M, et al. Effect of carvedilol on the morbidity of patients with severe chronic heart failure: results of the carvedilol prospective randomized cumulative survival (COPERNICUS) study. Circulation 2002; 106: 2194–2199.CrossRefGoogle ScholarPubMed
10. Packer, M, et al. Double-blind, placebo-controlled study of the effects of carvedilol in patients with moderate to severe heart failure. The PRECISE trial. Prospective Randomized Evaluation of Carvedilol on Symptoms and Exercise. Circulation 1996; 94: 2793–2799.CrossRefGoogle ScholarPubMed
11. Kim, MH, et al. Effects of beta-adrenergic blocking therapy on left ventricular diastolic relaxation properties in patients with dilated cardiomyopathy. Circulation 1999; 100: 729–735.CrossRefGoogle Scholar
12. Mori, Y, et al. Long-term effect of angiotensin-converting enzyme inhibitor in volume overloaded heart during growth: a controlled pilot study. J Am Coll Cardiol 2000; 36: 270–275.CrossRefGoogle ScholarPubMed
13. Calabro, R, et al. Hemodynamic effects of a single oral dose of enalapril among children with asymptomatic chronic mitral regurgitation. Am Heart J 1999; 138 (Pt 1): 955–961.CrossRefGoogle ScholarPubMed
14. Gisler, F, et al. Effectiveness of angiotensin-converting enzyme inhibitors in pediatric patients with mid to severe aortic valve regurgitation. Pediatr Cardiol 2008; 29: 906–909.CrossRefGoogle ScholarPubMed
15. Hsu, DT, et al. Enalapril in infants with single ventricle: results of a multicenter randomized trial. Circulation 2010; 122: 333–340.CrossRefGoogle ScholarPubMed
16. Kouatli, AA, et al. Enalapril does not enhance exercise capacity in patients after fontan procedure. Circulation 1997; 96: 1507–1512.CrossRefGoogle Scholar
17. WHO. Child Growth Standards based on length/height, weight and age. Acta Paediatr 2006; 450 (Suppl): 76–85.Google Scholar
18. d’Udekem, Y, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 2007; 116 (Suppl): I157–I164.CrossRefGoogle ScholarPubMed
19. Iyengar, AJ, et al. The fontan procedure in Australia: a population-based study. J Thorac Cardiovasc Surg 2007; 134: 1353–1354.CrossRefGoogle ScholarPubMed
20. Iyengar, AJ, et al. The extracardiac conduit fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes dagger. Eur J Cardiothorac Surg 2014; 46: 465–473.CrossRefGoogle Scholar
21. Mair, DD, et al. Fontan operation in 176 patients with tricuspid atresia. Results and a proposed new index for patient selection. Circulation 1990; 82 (Suppl): IV164–IV169.Google Scholar
22. Ommen, SR, et al. Clinical utility of Doppler echocardiography and tissue Doppler imaging in the estimation of left ventricular filling pressures: a comparative simultaneous Doppler-catheterization study. Circulation 2000; 102: 1788–1794.CrossRefGoogle ScholarPubMed
23. Menon, SC, Gray, R, Tani, LY. Evaluation of ventricular filling pressures and ventricular function by Doppler echocardiography in patients with functional single ventricle: correlation with simultaneous cardiac catheterization. J Am Soc Echocardiogr 2011; 24: 1220–1225.CrossRefGoogle ScholarPubMed