Sildenafil for pulmonary hypertension due to left-toright shunt after corrective procedure

  • Dyahris Kuntartiwi Department of Child Health, Brawijaya University Medical School/Dr. Saiful Anwar Hospital, Malang, East Java, Indonesia.
  • Piprim Basarah Yanuarso Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
  • Sudigdo Sastroasmoro Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
Keywords: pulmonary arterial hypertension, left-to-right CHD, corrective procedure, sildenafil

Abstract

Background Pulmonary arterial hypertension (PAH) is a common complication seen in those with a left-to-right shunt congenital heart defect (CHD). Corrective procedures by surgery or catheterization are the therapies of choice for reversible PAH. Since morbidity and mortality due to PAH after correction is high, sildenafil has been used as a selective vasodilator of the pulmonary artery, in order to decrease pulmonary arterial pressure. Objectives To evaluate the effect of sildenafil on pulmonary arterial pressure and clinical outcomes after left-to-right shunt CHD corrective procedures. Methods Left-to-right shunt patients aged < 18 years scheduled for corrective treatment were randomized in a double-blind fashion, to receive either oral sildenafil or placebo, given on days 3 to 30 after the corrective procedure. Clinical and pulmonary arterial pressures were evaluated by echocardiography before, 3 days after, and 30 days after the corrective procedure. Results From July 2013 to June 2014, 36 patients were included in the study: 17 in the placebo and 19 in the sildenafil groups. There were no differences in pulmonary arterial pressure or in clinical outcomes after corrective procedure between the two groups. There were no adverse events during the treatment. Conclusion Sildenafil has little effect on decreasing pulmonary arterial pressure, as most of our subjects seem to have hyperkinetic PAH. As such, pulmonary arterial pressure returns to normal soon after corrective procedures.

References

McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573–619.

Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev. 2009;18:154–61.

Galie N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, et al. Guidelines for the diagnosis and treatment of pulmonary hypertension: the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J. 2009;30:2493–537.

Palma G, Giordano R, Russolillo V, Cioffi S, Palumbo S, Mucerino M, et al. Sildenafil therapy for pulmonary hypertension before and after pediatric congenital heart surgery. Tex Heart Inst J. 2011;38:238-42.

Otto CM, Wong SP. Echocardiographic findings in acute and chronic pulmonary disease. In: Otto CM, editor. The practice of clinical echocardiography. London: WB Saunders Co; 2002. p. 748-50.

Duffels MG, Engelfriet PM, Berger RM, van Loon RL, Hoendermis E, Vriend JW, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiological perspective from a Dutch registry. Int J Cardiol. 2007;120: 198-204.

Kiefer TL, Bashore T. Anatomy of congenital heart disease lesions associated with pulmonary arterial hypertension. Adv Pulm Hypertension. 2013;11:166-170.

Gorenflo M, Gu H, Xu Z. Peri-operative pulmonary hypertension in paediatric patients: current strategies in children with congenital heart disease. Cardiology. 2010;116:10–7.

Landzberg MJ. Congenital heart disease associated pulmonary arterial hypertension. Clin Chest Med. 2007;28:243-53.

Haworth SG. Pathobiology of pulmonary hypertension in infants and children. Progress Ped Cardiol. 2001;12:249-69.

Haworth SG. Pulmonary hypertension in the young. Heart. 2002;88;658-64.

Barst RJ, Ivy DD, Gaitan G, Szatmari A, Rudzinski A, Garcia AE, et al. A randomized, double-blind, placebo controlled, dose-ranging study of oral sildenafil citrate in treatment-naive children with pulmonary arterial hypertension. Circulation. 2012;125:324-34.

Barst RJ, McGoon MD, Elliott CG, Foreman AJ, Miller DP, Ivy DD. Survival in childhood pulmonary arterial hypertension: insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management. Circulation. 2012;125:113–22.

Kothari SS. Assessment of operability in left to right shunt. In: Kumar RK, Haworth SG, editors. Pulmonary hypertension. 2nd ed. New Delhi: Elsevier; 2010. p. 103-13.

Chaumais MC, Perrin S, Sitbon O, Simonneau G, Humbert M, Montani D. Pharmacokinetic evaluation of sildenafil as a pulmonary hypertension treatment. Expert Opin Drug Metab Toxicol. 2013;9:1193-205.

Published
2015-09-01
How to Cite
1.
Kuntartiwi D, Yanuarso P, Sastroasmoro S. Sildenafil for pulmonary hypertension due to left-toright shunt after corrective procedure. PI [Internet]. 1Sep.2015 [cited 26Apr.2024];55(5):257-2. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/46
Received 2016-02-01
Accepted 2016-02-01
Published 2015-09-01