Int J Angiol 2021; 30(04): 305-309
DOI: 10.1055/s-0039-1696979
Case Report

Medical Therapy for Eisenmenger Syndrome: A Case Report and Review of Literature

1   Department of Cardiology, King George's Medical University, Lucknow, Uttar Pradesh, India
,
1   Department of Cardiology, King George's Medical University, Lucknow, Uttar Pradesh, India
,
Pravesh Vishwakarma
1   Department of Cardiology, King George's Medical University, Lucknow, Uttar Pradesh, India
,
Rishi Sethi
1   Department of Cardiology, King George's Medical University, Lucknow, Uttar Pradesh, India
› Author Affiliations

Abstract

Eisenmenger syndrome (ES) is defined as the process in which a long-standing left-to-right cardiac shunt caused by a congenital heart defect is eventually reversed into a cyanotic right-to-left shunt. It is crucial to recognize this grave pathology at the earliest because once it develops, treatment by medical or surgical means becomes even more challenging. In past decades, various therapeutic options have been developed that address the specific pathophysiological aspects of the disease and have shown to improve functional capacity and quality of life. There are three major therapeutic pathways in pulmonary hypertension treatment - endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, and prostacyclin derivatives. These therapies not only improve hemodynamic parameters and exercise capacity but they also improve prognosis with various form of Pulmonary hypertension including ES. We report a case of a 35-year-old female of ostium secundum atrial septal defect with ES, started on upfront combination therapy of ambrisentan and tadalafil who demonstrated marked improvement after 3 months of medical therapy.



Publication History

Article published online:
20 September 2019

© 2019. International College of Angiology. This article is published by Thieme.

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  • References

  • 1 Baumgartner H, Bonhoeffer P, De Groot NM. et al; Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC); Association for European Paediatric Cardiology (AEPC); ESC Committee for Practice Guidelines (CPG). ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31 (23) 2915-2957
  • 2 Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation 2007; 115 (08) 1039-1050
  • 3 Galiè N, Manes A, Negro L, Palazzini M, Bacchi-Reggiani ML, Branzi A. A meta-analysis of randomized controlled trials in pulmonary arterial hypertension. Eur Heart J 2009; 30 (04) 394-403
  • 4 Humbert M, Barst RJ, Robbins IM. et al. Combination of bosentan with epoprostenol in pulmonary arterial hypertension: BREATHE-2. Eur Respir J 2004; 24 (03) 353-359
  • 5 Hoeper MM, Faulenbach C, Golpon H, Winkler J, Welte T, Niedermeyer J. Combination therapy with bosentan and sildenafil in idiopathic pulmonary arterial hypertension. Eur Respir J 2004; 24 (06) 1007-1010
  • 6 Hoeper MM, Markevych I, Spiekerkoetter E, Welte T, Niedermeyer J. Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. Eur Respir J 2005; 26 (05) 858-863
  • 7 McLaughlin VV, Benza RL, Rubin LJ. et al. Addition of inhaled treprostinil to oral therapy for pulmonary arterial hypertension: a randomized controlled clinical trial. J Am Coll Cardiol 2010; 55 (18) 1915-1922
  • 8 McLaughlin VV, Oudiz RJ, Frost A. et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006; 174 (11) 1257-1263
  • 9 Simonneau G, Rubin LJ, Galiè N. et al; PACES Study Group. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008; 149 (08) 521-530
  • 10 Galie N, Barbera JA, Frost AE. et al. Initial use of ambrisentan plus tadalafil in pulmonary arterial hypertension. N Engl J Med 2015; 373: 834-844
  • 11 Tay EL, Peset A, Papaphylactou M. et al. Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome. Int J Cardiol 2011; 151 (03) 307-312
  • 12 Bharani A, Mathew V, Sahu A, Lunia B. The efficacy and tolerability of sildenafil in patients with moderate-to-severe pulmonary hypertension. Indian Heart J 2003; 55 (01) 55-59
  • 13 Garg N, Sharma MK, Sinha N. Role of oral sildenafil in severe pulmonary arterial hypertension: clinical efficacy and dose response relationship. Int J Cardiol 2007; 120 (03) 306-313
  • 14 Gupta A, Kerkar P. Sildenafil for pulmonary hypertension secondary to congenital heart diseases. Indian Heart J 2007; 59 (04) 342-345
  • 15 Tay EL, Papaphylactou M, Diller GP. et al. Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy. Int J Cardiol 2011; 149 (03) 372-376
  • 16 Sun YJ, Yang T, Zeng WJ. et al. Impact of sildenafil on survival of patients with Eisenmenger syndrome. J Clin Pharmacol 2013; 53 (06) 611-618
  • 17 Mukhopadhyay S, Nathani S, Yusuf J, Shrimal D, Tyagi S. Clinical efficacy of phosphodiesterase-5 inhibitor tadalafil in Eisenmenger syndrome--a randomized, placebo-controlled, double-blind crossover study. Congenit Heart Dis 2011; 6 (05) 424-431
  • 18 Galiè N, Beghetti M, Gatzoulis MA. et al; Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation 2006; 114 (01) 48-54
  • 19 Diller GP, Dimopoulos K, Kaya MG. et al. Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease. Heart 2007; 93 (08) 974-976
  • 20 Cha KS, Cho KI, Seo JS. et al. Effects of inhaled iloprost on exercise capacity, quality of life, and cardiac function in patients with pulmonary arterial hypertension secondary to congenital heart disease (the Eisenmenger syndrome) (from the EIGER Study). Am J Cardiol 2013; 112 (11) 1834-1839
  • 21 Sitbon O, Channick R, Chin KM. et al; GRIPHON Investigators. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med 2015; 373 (26) 2522-2533
  • 22 Zuckerman WA, Leaderer D, Rowan CA, Mituniewicz JD, Rosenzweig EB. Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. Am J Cardiol 2011; 107 (09) 1381-1385
  • 23 Gatzoulis MA, Landzberg M, Beghetti M. et al; MAESTRO Study Investigators. Evaluation of macitentan in patients with Eisenmenger syndrome. Circulation 2019; 139 (01) 51-63
  • 24 Monfredi O, Heward E, Griffiths L, Condliffe R, Mahadevan VS. Effect of dual pulmonary vasodilator therapy in pulmonary arterial hypertension associated with congenital heart disease: a retrospective analysis. Open Heart 2016; 3 (01) e000399
  • 25 Iversen K, Jensen AS, Jensen TV, Vejlstrup NG, Søndergaard L. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial. Eur Heart J 2010; 31 (09) 1124-1131
  • 26 D'Alto M, Romeo E, Argiento P. et al. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol 2012; 155 (03) 378-382