Hostname: page-component-cd9895bd7-gxg78 Total loading time: 0 Render date: 2024-12-18T16:20:57.611Z Has data issue: false hasContentIssue false
  • English
  • Français

Acute vasodilator testing following Fontan palliation: an opportunity to guide precision care?

Published online by Cambridge University Press:  22 May 2020

Ronald W. Day Open the ORCID record for Ronald W. Day [Opens in a new window]
Ronald W. Day*
Affiliation:
Department of Pediatrics, University of Utah, Salt Lake City, UT, USA
*
Author for correspondence: Ronald W. Day, MD, Primary Children’s Hospital, 81 North Mario Capecchi Drive, Salt Lake City, UT84113, USA. Tel: +1 801 213 7635; Fax: +1 801 213 7778. E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background:

Pulmonary vasodilators improve the functional capacity of some patients with pulmonary arterial hypertension. However, pulmonary vasodilators frequently fail to improve unequivocal endpoints of efficacy in patients with lower pulmonary arterial pressures who have been palliated with a Fontan procedure.

Objective:

Haemodynamic measurements and the results of acute vasodilator testing in a subset of patients were reviewed to determine whether some patients acutely respond more favourably to sildenafil and might be candidates for precision care with a phosphodiesterase V inhibitor long term.

Materials and Methods:

Heart catheterisation was performed in 11 patients with a Fontan procedure. Haemodynamic measurements were performed before and after treatment with intravenous sildenafil (mean 0.14, range 0.05–0.20 mg/kg). Results (mean ± standard deviation) were compared by paired and unpaired t-tests to identify statistically significant changes.

Results:

Sildenafil was acutely associated with changes in mean pulmonary arterial pressure, transpulmonary gradient, indexed blood flow, and indexed vascular resistance. Changes in mean pulmonary arterial pressure were greater for patients with a mean pulmonary arterial pressure greater than 14 mmHg compared to patients with a lower mean pulmonary arterial pressure. Changes in transpulmonary gradient were greater for patients with a transpulmonary gradient greater than 5 mmHg compared to patients with a lower transpulmonary gradient.

Conclusion:

Sildenafil acutely decreases mean pulmonary arterial pressure and transpulmonary gradient and causes greater acute changes in patients with higher mean pulmonary arterial pressures and transpulmonary gradients. Haemodynamic measurements and vasodilator testing might help to guide precision care following Fontan palliation.

Keywords

Acute vasodilator testingFontan procedureheart catheterisationphosphodiesterase V inhibition
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 6 , June 2020 , pp. 829 - 833
Copyright
© The Author(s), 2020. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Choussat, A, Fontan, F, Besse, P. Selection criteria for the Fontan procedure. In: Anderson, RH, Shinebourne, EA (eds). Paediatric Cardiology. Churchill Livingstone, Edinburgh, 1977: 559–566.Google Scholar
Driscoll, DJ, Offord, KP, Feldt, RH, Schaff, HV, Puga, FJ, Danielson, GK.Five- to fifteen-year follow-up after Fontan operation. Circulation 1992; 85: 201–210.CrossRefGoogle ScholarPubMed
Day, RW, Etheridge, SP, Veasy, LG, et al.Single ventricle palliation: Greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone. Int J Cardiol 2006; 106: 201–210.CrossRefGoogle ScholarPubMed
Ridderbos, FLS, Wolff, D, Timmer, A, et al.Adverse pulmonary vascular remodeling in the Fontan circulation. J Heart Lung Transplant 2015; 34: 404–413.CrossRefGoogle ScholarPubMed
Ridderbos, FJS, Bonenkamp, BE, Meyer, SL, et al.Pulmonary artery size is associated with functional clinical status in the Fontan circulation. Heart 2020; 106: 233–239. doi:10.1136/heartjnl-2019-314972Google ScholarPubMed
Beghetti, M.Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies. Heart 2010; 96: 911–916.CrossRefGoogle ScholarPubMed
Gewillig, M, Brown, SC, van der Bruaene, A, Rychik, J.Providing a framework of principles for conceptualising the Fontan circulation. Acta Pediatr 2020; 109: 651–658.Google ScholarPubMed
Rhodes, J, Ubeda-Tikkanen, A, Claire, M, et al.Effect of inhaled iloprost on the exercise function of Fontan patients: a demonstration of concept. Int J Cardiol 2013; 168: 2435–2440.CrossRefGoogle ScholarPubMed
Giardini, A, Balducci, A, Specchia, S, Gargiulo, G, Bonvicini, M, Picchio, FM.Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 2008; 29: 1681–1687.CrossRefGoogle ScholarPubMed
Van de Bruaene, A, La Gerche, A, Claessen, G, et al.Sildenafil improves exercise hemodynamics in Fontan patients. Circ Cardiovasc Imag 2014; 7: 265–273.Google ScholarPubMed
Goldberg, DJ, French, B, McBride, MG, et al.Impact of oral sildenafil on exercise performance in children and young adults after the Fontan operation: a randomized, double-blind, placebo-controlled, crossover trial. Circulation 2011; 123: 1185–1193.CrossRefGoogle Scholar
Hebert, A, Mikkelsen, UR, Thilen, U, et al.Bosentan improves exercise capacity in adolescents and adults after Fontan operation: the TEMPO (treatment with endothelin receptor antagonist in Fontan patients: a randomized, placebo controlled, double-blind study measuring peak oxygen consumption) study. Circulation 2014; 130: 2021–2030.CrossRefGoogle ScholarPubMed
Schuuring, MJ, Vis, JC, van Dijk, AP, et al.Impact of Bosentan on exercise capacity in adults after the Fontan operation: a randomized study. Eur J Heart Fail 2013; 15: 690–698.Google Scholar
Ovaert, C, Thijs, D, Dewolf, D, et al.The effect of Bosentan in patients with a failing Fontan circulation. Cardiol Young 2009; 19: 331–339.CrossRefGoogle ScholarPubMed
Collins, JLG, Law, MA, Borasino, S, Erwin, WC, Cleveland, DC, Alten, JA.Routine sildenafil does not improve clinical outcomes after Fontan operation. Pediatr Cardiol 2017; 38: 1703–1708.CrossRefGoogle Scholar
Mori, H, Park, IS, Yamagishi, H, et al.Sildenafil reduces pulmonary vascular resistance in single ventricle physiology. Int J Cardiol 2016; 221: 122–127.CrossRefGoogle Scholar
Oldenburger, NJ, Mank, A, Etnel, J, Takkenberg, JJ, Helbing, WA.Drug therapy in the prevention of failure of the Fontan circulation: a systematic review. Cardiol Young 2016; 26: 842–850.CrossRefGoogle ScholarPubMed
Hager, A, Weber, R, Müller, J, Hess, J.Predictors of sildenafil effects on exercise capacity in adolescents and adults with Fontan circulation. Clin Res Cardiol 2014; 103: 641–646.CrossRefGoogle ScholarPubMed
Wang, W, Hu, X, Liao, W, et al.The efficacy and safety of pulmonary vasodilators in patients with Fontan circulation: a meta-analysis of randomized controlled trials. Pulm Circ 2019; 9: 1–9.CrossRefGoogle ScholarPubMed
Goldberg, DJ, Zak, V, Goldstein, BH, et al.Results of the Fontan udenafil exercise longitudinal (FUEL) trial. Circulation, 2019 [Epub ahead of print].Google ScholarPubMed