Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
권호 표지
DOI QR코드

DOI QR Code

Inhaled iloprost for the treatment of patient with Fontan circulation

  • Kim, Yong Hyun (Department of Pediatrics, Gachon University Gil Medical Center) ;
  • Chae, Moon Hee (Department of Pediatrics, Gachon University Gil Medical Center) ;
  • Choi, Deok Young (Department of Pediatrics, Gachon University Gil Medical Center)
  • Received : 2013.09.09
  • Accepted : 2013.10.20
  • Published : 2014.10.10
Download PDF
⟨ Previous Next ⟩

Abstract

Decreased exercise capacity after Fontan surgery is relatively common and the failure of the Fontan state gradually increases with age. However, there is no further treatment for patients with Fontan circulation. Pulmonary vasodilation therapy is an effective method to solve this problem because pulmonary vascular resistance is a major factor of the Fontan problem. Inhaled iloprost is a chemically stable prostacyclin analogue and a potent pulmonary vasodilator. We experienced two cases of Fontan patients treated with inhaled iloprost for 12 weeks. The first patient was an 18-year-old female with pulmonary atresia with an intact ventricular septum, and the second patient was a 22-year-old male with a double outlet right ventricle. Fifteen years have passed since both patients received Fontan surgery. While the pulmonary pressure was not decreased significantly, improved exercise capacity and cardiac output were observed without any major side effects in both patients. The iloprost inhalation therapy was well tolerated and effective for the symptomatic treatment of Fontan patients.

Keywords

References

  1. Mondesert B, Marcotte F, Mongeon FP, Dore A, Mercier LA, Ibrahim R, et al. Fontan circulation: success or failure? Can J Cardiol 2013;29:811-20. https://doi.org/10.1016/j.cjca.2012.12.009
  2. Dasi LP, Krishnankuttyrema R, Kitajima HD, Pekkan K, Sundareswaran KS, Fogel M, et al. Fontan hemodynamics: importance of pulmonary artery diameter. J Thorac Cardiovasc Surg 2009; 137:560-4. https://doi.org/10.1016/j.jtcvs.2008.04.036
  3. La Gerche A, Gewillig M. What limits cardiac performance during exercise in normal subjects and in healthy fontan patients? Int J Pediatr 2010;2010. Article ID 791291.
  4. Gewillig M, Brown SC, Eyskens B, Heying R, Ganame J, Budts W, et al. The Fontan circulation: who controls cardiac output? Interact Cardiovasc Thorac Surg 2010;10:428-33. https://doi.org/10.1510/icvts.2009.218594
  5. Busse R, Fleming I. Pulsatile stretch and shear stress: physical stimuli determining the production of endothelium-derived relaxing factors. J Vasc Res 1998;35:73-84. https://doi.org/10.1159/000025568
  6. Tatum GH, Sigfusson G, Ettedgui JA, Myers JL, Cyran SE, Weber HS, et al. Pulmonary artery growth fails to match the increase in body surface area after the Fontan operation. Heart 2006;92:511-4.
  7. Marrone C, Galasso G, Piccolo R, de Leva F, Paladini R, Piscione F, et al. Antiplatelet versus anticoagulation therapy after extracardiac conduit Fontan: a systematic review and meta-analysis. Pediatr Cardiol 2011;32:32-9. https://doi.org/10.1007/s00246-010-9808-4
  8. Hebert A, Jensen AS, Idorn L, Sorensen KE, Sondergaard L. The effect of bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study. BMC Cardiovasc Disord 2013;13:36. https://doi.org/10.1186/1471-2261-13-36
  9. Bowater SE, Weaver RA, Thorne SA, Clift PF. The safety and effects of bosentan in patients with a Fontan circulation. Congenit Heart Dis 2012;7:243-9. https://doi.org/10.1111/j.1747-0803.2012.00635.x
  10. Schuuring MJ, Vis JC, Bouma BJ, van Dijk AP, van Melle JP, Pieper PG, et al. Rationale and design of a trial on the role of bosentan in Fontan patients: improvement of exercise capacity? Contemp Clin Trials 2011;32:586-91. https://doi.org/10.1016/j.cct.2011.04.001
  11. Shabanian R, Shahbaznejad L, Razaghian A, Kiani A, Rahimzadeh M, Seifirad S, et al. Sildenafil and ventriculo-arterial coupling in Fontan-palliated patients: a noninvasive echocardiographic assessment. Pediatr Cardiol 2013;34:129-34. https://doi.org/10.1007/s00246-012-0400-y
  12. Ciliberti P, Schulze-Neick I, Giardini A. Modulation of pulmonary vascular resistance as a target for therapeutic interventions in Fontan patients: focus on phosphodiesterase inhibitors. Future Cardiol 2012;8:271-84. https://doi.org/10.2217/fca.12.16
  13. Mitchell MB, Campbell DN, Ivy D, Boucek MM, Sondheimer HM, Pietra B, et al. Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure. J Thorac Cardiovasc Surg 2004;128:693-702. https://doi.org/10.1016/S0022-5223(04)00991-2
  14. Yamagishi M, Kurosawa H, Hashimoto K, Nomura K, Kitamura N. The role of plasma endothelin in the Fontan circulation. J Cardiovasc Surg (Torino) 2002;43:793-7.
  15. Rhodes J, Ubeda-Tikkanen A, Clair M, Fernandes SM, Graham DA, Milliren CE, et al. Effect of inhaled iloprost on the exercise function of Fontan patients: a demonstration of concept. Int J Cardiol 2013;168:2435-40. https://doi.org/10.1016/j.ijcard.2013.03.014

Cited by

  1. Managing adult Fontan patients: where do we stand? vol.25, pp.142, 2014, https://doi.org/10.1183/16000617.0091-2016
  2. The use of Macitentan in Fontan circulation: a case report vol.17, pp.None, 2014, https://doi.org/10.1186/s12872-017-0567-5
  3. Heart failure in single right ventricle congenital heart disease: physiological and molecular considerations vol.318, pp.4, 2014, https://doi.org/10.1152/ajpheart.00518.2019
  4. Focused Update on Pulmonary Hypertension in Children—Selected Topics of Interest for the Adult Cardiologist vol.56, pp.9, 2014, https://doi.org/10.3390/medicina56090420