1887
Volume 2015, Issue 3
  • ISSN: 2305-7823
  • E-ISSN:

Abstract

Pulmonary arterial hypertension (PAH) is a serious complication of congenital heart disease, causing an increase in morbidity and mortality. The progressive and irreversible pulmonary vascular disease is more often the consequence of a significant, uncorrected, left-to-right shunt. The rise in pulmonary vascular resistance may lead to the reversal of the shunt and cyanosis, condition known as Eisenmenger syndrome. The management of this population is challenging and requires specific expertise both for diagnosis and follow-up. The progress in the understanding of the underlying pathophysiology of this condition has promoted recent pharmacological trials. New therapeutic options are now available that could improve the long-term prognosis and the quality of life of these patients, but several controversial points still remain and need to be addressed.

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2015-10-13
2019-12-15
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References

  1. Eisenmenger V. Die angeborenen defecte der kammerscheidewand des herzens. Z Klin Med. 1897; 32::128.
    [Google Scholar]
  2. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958; 2::701709.
    [Google Scholar]
  3. Rodriguez FH, Moodie DS, Parekh DR, Franklin WJ, Morales DLS, Zafar F, Graves DE, Friedman RA, Rossano JW. Outcomes of hospitalization in adults in the United States with atrial septal defect, ventricular septal defect, and atrioventricular septal defect. Am J Cardiol. 2011; 108::290293.
    [Google Scholar]
  4. Lowe BS, Therrien J, Ionescu-Ittu R, Pilote L, Martucci G, Marelli AJ. Diagnosis of pulmonary hypertension in the congenital heart disease adult population impact on outcomes. J Am Coll Cardiol. 2011; 58::538546.
    [Google Scholar]
  5. Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007; 115::163172.
    [Google Scholar]
  6. Adatia I, Kothari SS, Feinstein JA. Pulmonary hypertension associated with congenital heart disease: pulmonary vascular disease: the global perspective. Chest. 2010; 137:6 Suppl:52S61S.
    [Google Scholar]
  7. Duffels MGJ, Engelfriet PM, Berger RMF, van Loon RL, Hoendermis E, Vriend JW, van der Velde ET, Bresser P, Mulder BJ. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007; 120::198204.
    [Google Scholar]
  8. Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, Gomez Sanchez MA, Krishna Kumar R, Landzberg M, Machado RF, Olschewski H, Robbins IM, Souza R. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013; 62::D34D41.
    [Google Scholar]
  9. Heath D, Helmholz HF Jr, Burchell HB, Dushane JW, Edwards JE. Graded pulmonary vascular changes and hemodynamic findings in cases of atrial and ventricular septal defect and patent ductus arteriosus. Circulation. 1958; 18::11551166.
    [Google Scholar]
  10. Rabinovitch M, Haworth SG, Castaneda AR, Nadas AS, Reid LM. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation. 1978; 58::11071122.
    [Google Scholar]
  11. Frescura C, Thiene G, Giulia Gagliardi M, Mazzucco A, Pellegrino PA, Daliento L, Biscaglia S, Carminati M, Gallucci V. Is lung biopsy useful for surgical decision making in congenital heart disease? Eur J Cardio-Thorac Surg Off J Eur Assoc Cardio-Thorac Surg. 1991; 5::122123.
    [Google Scholar]
  12. Wagenvoort CA. Morphological substrate for the reversibility and irreversibility of pulmonary hypertension. Eur Heart J. 1988; 9::712.
    [Google Scholar]
  13. Wagenvoort CA, Wagenvoort N, Draulans-Noë Y. Reversibility of plexogenic pulmonary arteriopathy following banding of the pulmonary artery. J Thorac Cardiovasc Surg. 1984; 87::876886.
    [Google Scholar]
  14. Greenwood RD, Nadas AS. The clinical course of cardiac disease in Down's syndrome. Pediatrics. 1976; 58::893897.
    [Google Scholar]
  15. Saji T. Clinical characteristics of pulmonary arterial hypertension associated with Down syndrome. Pediatr Int Off J Jpn Pediatr Soc. 2014; 56::297303.
    [Google Scholar]
  16. Diller G-P, Dimopoulos K, Broberg CS, Kaya MG, Naghotra US, Uebing A, Harries C, Goktekin O, Gibbs JSR, Gatzoulis MA. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J. 2006; 27::17371742.
    [Google Scholar]
  17. Hopkins WE. The remarkable right ventricle of patients with Eisenmenger syndrome. Coron Artery Dis. 2005; 16::1925.
    [Google Scholar]
  18. Diller G-P, Lammers AE, Haworth SG, Dimopoulos K, Derrick G, Bonhoeffer P, Gatzoulis MA, Francis DP. A modelling study of atrial septostomy for pulmonary arterial hypertension, and its effect on the state of tissue oxygenation and systemic blood flow. Cardiol Young. 2010; 20::2532.
    [Google Scholar]
  19. Blanc J, Vouhé P, Bonnet D. Potts shunt in patients with pulmonary hypertension. N Engl J Med. 2004; 350::623.
    [Google Scholar]
  20. Baruteau AE, Belli E, Boudjemline Y, Laux D, Lévy M, Simonneau G, Carotti A, Humbert M, Bonnet D. Palliative Potts shunt for the treatment of children with drug-refractory pulmonary arterial hypertension: updated data from the first 24 patients. Eur J Cardiothorac Surg. 2015; 47:3:e105e110.
    [Google Scholar]
  21. Boudjemline Y, Patel M, Malekzadeh-Milani S, Szezpanski I, Lévy M, Bonnet D. Patent ductus arteriosus stenting (Transcatheter Potts shunt) for palliation of suprasystemic pulmonary arterial hypertension: a case series. Circ Cardiovasc Interv. 2013; 6::e18e20.
    [Google Scholar]
  22. D'Alto M, Santoro G, Palladino MT, Parisi F, Russo MG. Patent ductus arteriosus stenting for palliation of severe pulmonary arterial hypertension in childhood. Cardiol Young. 2014; Jul 7::15, [Epub ahead of print].
    [Google Scholar]
  23. DeFilippis AP, Law K, Curtin S, Eckman JR. Blood is thicker than water: the management of hyperviscosity in adults with cyanotic heart disease. Cardiol Rev. 2007; 15::3134.
    [Google Scholar]
  24. Somerville J. How to manage the Eisenmenger syndrome. Int J Cardiol. 1998; 63::18.
    [Google Scholar]
  25. Broberg CS, Ujita M, Prasad S, Li W, Rubens M, Bax BE, Davidson SJ, Bouzas B, Gibbs JSR, Burman J, Gatzoulis MA. Pulmonary arterial thrombosis in eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol. 2007; 50::634642.
    [Google Scholar]
  26. Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, Stone S. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J. 1998; 19::18451855.
    [Google Scholar]
  27. Perloff JK, Rosove MH, Child JS, Wright GB. Adults with cyanotic congenital heart disease: hematologic management. Ann Intern Med. 1988; 109::406413.
    [Google Scholar]
  28. Waldow HC, Westhoff-Bleck M, Widera C, Templin C, von Depka M. Acquired von Willebrand syndrome in adult patients with congenital heart disease. Int J Cardiol. 2014; 176::739745.
    [Google Scholar]
  29. Olsson KM, Delcroix M, Ghofrani HA, Tiede H, Huscher D, Speich R, Grünig E, Staehler G, Rosenkranz S, Halank M, Held M, Lange TJ, Behr J, Klose H, Claussen M, Ewert R, Opitz CF, Vizza CD, Scelsi L, Vonk-Noordegraaf A, Kaemmerer H, Gibbs JS, Coghlan G, Pepke-Zaba J, Schulz U, Gorenflo M, Pittrow D, Hoeper MM. Anticoagulation and survival in pulmonary arterial hypertension: results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA). Circulation. 2014; 129:1:5765.
    [Google Scholar]
  30. Dimopoulos K, Wort SJ, Gatzoulis MA. Pulmonary hypertension related to congenital heart disease: a call for action. Eur Heart J. 2014; 35::691700.
    [Google Scholar]
  31. Perloff JK. Systemic complications of cyanosis in adults with congenital heart disease. Hematologic derangements, renal function, and urate metabolism. Cardiol Clin. 1993; 11::689699.
    [Google Scholar]
  32. Habib G, Hoen B, Tornos P, Thuny F, Prendergast B, Vilacosta I, Moreillon P, de Jesus Antunes M, Thilen U, Lekakis J, Lengyel M, Müller L, Naber CK, Nihoyannopoulos P, Moritz A, Zamorano JL. ESC Committee for Practice Guidelines. Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009): the Task Force on the Prevention, Diagnosis, and Treatment of Infective Endocarditis of the European Society of Cardiology (ESC). Endorsed by the European Society of Clinical Microbiology and Infectious Diseases (ESCMID) and the International Society of Chemotherapy (ISC) for Infection and Cancer. Eur Heart J. 2009; 30::23692413.
    [Google Scholar]
  33. Nishimura RA, Carabello BA, Faxon DP, Freed MD, Lytle BW, O'Gara PT, O'Rourke RA, Shah PM, Bonow RO, Carabello BA, Chatterjee K, de Leon AC, Faxon DP, Freed MD, Gaasch WH, Lytle BW, Nishimura RA, O'Gara PT, O'Rourke RA, Otto CM, Shah PM, Shanewise JS, Smith SC, Jacobs AK, Buller CE, Creager MA, Ettinger SM, Krumholz HM, Kushner FG, Lytle BW, Nishimura RA, Page RL, Tarkington LG, Yancy CW. American College of Cardiology/American Heart Association Task Force. ACC/AHA 2008 guideline update on valvular heart disease: focused update on infective endocarditis: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines: endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation. 2008; 118::887896.
    [Google Scholar]
  34. Sadeghpour A, Kyavar M, Alizadehasl A. Comprehensive Approach to Adult Congenital Heart Disease. Springer 2014.
  35. Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery J-L, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G, ESC Committee for Practice Guidelines (CPG) , Vahanian A, Auricchio A, Bax J, Ceconi C, Dean V, Filippatos G, Funck-Brentano C, Hobbs R, Kearney P, McDonagh T, McGregor K, Popescu BA, Reiner Z, Sechtem U, Sirnes PA, Tendera M, Vardas P, Widimsky P, Document Reviewers: , Sechtem U, Al Attar N, Andreotti F, Aschermann M, Asteggiano R, Benza R, Berger R, Bonnet D, Delcroix M, Howard L, Kitsiou AN, Lang I, Maggioni A, Nielsen-Kudsk JE, Park M, Perrone-Filardi P, Price S, Domenech MTS, Vonk-Noordegraaf A, Zamorano JL. Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2009; 30::24932537.
    [Google Scholar]
  36. Hoeper MM, Bogaard HJ, Condliffe R, Frantz R, Khanna D, Kurzyna M, Langleben D, Manes A, Satoh T, Torres F, Wilkins MR, Badesch DB. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013; 62::D42D50.
    [Google Scholar]
  37. Moceri P, Dimopoulos K, Liodakis E, Germanakis I, Kempny A, Diller G-P, Swan L, Wort SJ, Marino PS, Gatzoulis MA, Li W. Echocardiographic predictors of outcome in Eisenmenger syndrome. Circulation. 2012; 126::14611468.
    [Google Scholar]
  38. Budts W, Van Pelt N, Gillyns H, Gewillig M, Van De Werf F, Janssens S. Residual pulmonary vasoreactivity to inhaled nitric oxide in patients with severe obstructive pulmonary hypertension and Eisenmenger syndrome. Heart. 2001; 86:5:553558.
    [Google Scholar]
  39. D'Alto M, Romeo E, Argiento P, Santoro G, Sarubbi B, Gaio G, Mélot C, Russo MG, Naeije R, Calabrò R. Pulmonary vasoreactivity predicts long-term outcome in patients with Eisenmenger syndrome receiving bosentan therapy. Heart. 2010; 96:18:14751479.
    [Google Scholar]
  40. Tay ELW, Papaphylactou M, Diller GP, Alonso-Gonzalez R, Inuzuka R, Giannakoulas G, Harries C, Wort SJ, Swan L, Dimopoulos K, Gatzoulis MA. Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy. Int J Cardiol. 2011; 149::372376.
    [Google Scholar]
  41. Spence MS, Balaratnam MS, Gatzoulis MA. Clinical update: cyanotic adult congenital heart disease. Lancet. 2007; 370::15301532.
    [Google Scholar]
  42. Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol. 1996; 28::768772.
    [Google Scholar]
  43. Maurer HM, McCue CM, Robertson LW, Haggins JC. Correction of platelet dysfunction and bleeding in cyanotic congenital heart disease by simple red cell volume reduction. Am J Cardiol. 1975; 35::831835.
    [Google Scholar]
  44. Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RMF, Lauer A, Chiossi E, Landzberg M. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006; 114::4854.
    [Google Scholar]
  45. D'Alto M, Vizza CD, Romeo E, Badagliacca R, Santoro G, Poscia R, Sarubbi B, Mancone M, Argiento P, Ferrante F, Russo MG, Fedele F, Calabrò R. Long term effects of bosentan treatment in adult patients with pulmonary arterial hypertension related to congenital heart disease (Eisenmenger physiology): safety, tolerability, clinical, and haemodynamic effect. Heart. 2007; 93:5:621625.
    [Google Scholar]
  46. Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RMF, Lauer A, Chiossi E, Landzberg M. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008; 127::2732.
    [Google Scholar]
  47. Berger RMF, Beghetti M, Galiè N, Gatzoulis MA, Granton J, Lauer A, Chiossi E, Landzberg M. Atrial septal defects versus ventricular septal defects in BREATHE-5, a placebo-controlled study of pulmonary arterial hypertension related to Eisenmenger's syndrome: A subgroup analysis. Int J Cardiol [Internet]. 2009;, [cited 2010 Apr 29]; Available from: http://www.ncbi.nlm.nih.gov/pubmed/19464064 .
    [Google Scholar]
  48. 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::13811385.
    [Google Scholar]
  49. MAESTRO (Macitentan in Eisenmenger Syndrome To Restore Exercise Capacity), ClinicalTrials.gov [Internet]. ddad;, [cited 2014 Nov 9]; Available from: http://clinicaltrials.gov/show/NCT01743001 .
    [Google Scholar]
  50. Chau EMC, Fan KYY, Chow WH. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol. 2007; 120::301305.
    [Google Scholar]
  51. 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::306313.
    [Google Scholar]
  52. Singh TP, Rohit M, Grover A, Malhotra S, Vijayvergiya R. A randomized, placebo-controlled, double-blind, crossover study to evaluate the efficacy of oral sildenafil therapy in severe pulmonary artery hypertension. Am Heart J. 2006; 151::851e1851e5.
    [Google Scholar]
  53. 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::424431.
    [Google Scholar]
  54. Cha KS, Cho KI, Seo JS, Choi JH, Park YH, Yang DH, Hong GR, Kim DS. 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::18341839.
    [Google Scholar]
  55. D'Alto M, Romeo E, Argiento P, Sarubbi B, Santoro G, Grimaldi N, Correra A, Scognamiglio G, Russo MG, Calabrò R. Bosentan-sildenafil association in patients with congenital heart disease-related pulmonary arterial hypertension and Eisenmenger physiology. Int J Cardiol. 2012; 155::378382.
    [Google Scholar]
  56. 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::11241131.
    [Google Scholar]
  57. Dimopoulos K, Inuzuka R, Goletto S, Giannakoulas G, Swan L, Wort SJ, Gatzoulis MA. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010; 121::2025.
    [Google Scholar]
  58. Hertz MI, Aurora P, Christie JD, Dobbels F, Edwards LB, Kirk R, Kucheryavaya AY, Rahmel AO, Rowe AW, Taylor DO. Registry of the International Society for Heart and Lung Transplantation: a quarter century of thoracic transplantation. J Heart Lung Transplant Off Publ Int Soc Heart Transplant. 2008; 27::937942.
    [Google Scholar]
  59. Gatzoulis MA, Beghetti M, Landzberg MJ, Galiè N. Pulmonary arterial hypertension associated with congenital heart disease: recent advances and future directions. Int J Cardiol. 2014Dec 15; 177:2:340347.
    [Google Scholar]
  60. Cantor WJ, Harrison DA, Moussadji JS, Connelly MS, Webb GD, Liu P, McLaughlin PR, Siu SC. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol. 1999; 84::677e81.
    [Google Scholar]
  61. Diller G-P, Kempny A, Inuzuka R, Radke R, Wort SJ, Baumgartner H, Gatzoulis MA, Dimopoulos K. Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart Br Card Soc. 2014; 100::13661372.
    [Google Scholar]
  62. D'Alto M, Romeo E, Argiento P, Correra A, Santoro G, Gaio G, Sarubbi B, Calabrò R, Russo MG. Hemodynamics of patients developing pulmonary arterial hypertension after shunt closure. Int J Cardiol. 2013; 168.4::37973801.
    [Google Scholar]
  63. Van Loon RLE, Roofthooft MTR, Hillege HL, ten Harkel ADJ, van Osch-Gevers M, Delhaas T, Kapusta L, Strengers JLM, Rammeloo L, Clur S-AB, Mulder BJM, Berger RMF. Pediatric pulmonary hypertension in the Netherlands: epidemiology and characterization during the period 1991 to 2005. Circulation. 2011; 124::17551764.
    [Google Scholar]
  64. Talwar S1, Choudhary SK, Saxena A, Kothari SS, Juneja R, Airan B. Unidirectional valved patches for closure of septal defects in patients with severe pulmonary hypertension. Ann Pediatr Cardiol. 2008; 1:2:114119.
    [Google Scholar]
  65. Althoff TF, Knebel F, Panda A, McArdle J, Gliech V, Franke I, Witt C, Baumann G, Borges AC. Long-term follow-up of a fenestrated Amplatzer atrial septal occluder in pulmonary arterial hypertension. Chest. 2008; 133:1:283285.
    [Google Scholar]
  66. Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, Cifkova R, Ferreira R, Foidart J-M, Gibbs JSR, Gohlke-Baerwolf C, Gorenek B, Iung B, Kirby M, Maas AHEM, Morais J, Nihoyannopoulos P, Pieper PG, Presbitero P, Roos-Hesselink JW, Schaufelberger M, Seeland U, Torracca L, European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM) . ESC Committee for Practice Guidelines. ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Eur Heart J. 2011; 32::31473197.
    [Google Scholar]
  67. Presbitero P, Somerville J, Stone S, Aruta E, Spiegelhalter D, Rabajoli F. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation. 1994; 89::26732676.
    [Google Scholar]
  68. Morris JK, Alberman E. Trends in Down's syndrome live births and antenatal diagnoses in England and Wales from 1989 to 2008: analysis of data from the National Down Syndrome Cytogenetic Register. BMJ. 2009; 339::b3794.
    [Google Scholar]
  69. Freeman SB, Bean LH, Allen EG, Tinker SW, Locke AE, Druschel C, Hobbs CA, Romitti PA, Royle MH, Torfs CP, Dooley KJ, Sherman SL. Ethnicity, sex, and the incidence of congenital heart defects: a report from the National Down Syndrome Project. Genet Med Off J Am Coll Med Genet. 2008; 10::173180.
    [Google Scholar]
  70. Cappelli-Bigazzi M, Santoro G, Battaglia C, Palladino MT, Carrozza M, Russo MG, Pacileo G, Calabrò R. Endothelial cell function in patients with Down's syndrome. Am J Cardiol. 2004; 94::392395.
    [Google Scholar]
  71. D'Alto M, Mahadevan VS. Pulmonary arterial hypertension associated with congenital heart disease. Eur Respir Rev Off J Eur Respir Soc. 2012; 21::328337.
    [Google Scholar]
  72. Duffels MGJ, Vis JC, van Loon RLE, Berger RMF, Hoendermis ES, van Dijk APJ, Bouma BJ, Mulder BJM. Down patients with Eisenmenger syndrome: is bosentan treatment an option? Int J Cardiol. 2009; 134::378383.
    [Google Scholar]
  73. Duffels MGJ, Vis JC, van Loon RLE, Nieuwkerk PT, van Dijk APJ, Hoendermis ES, de Bruin-Bon RHACM, Bouma BJ, Bresser P, Berger RMF, Mulder BJM. Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down's syndrome. Am J Cardiol. 2009; 103::13091315.
    [Google Scholar]
  74. D'Alto M, Romeo E, Argiento P, D'Andrea A, Sarubbi B, Correra A, Scognamiglio G, Papa S, Bossone E, Calabrò R, Vizza CD, Russo MG. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down's syndrome. Int J Cardiol. 2013; 164::323326.
    [Google Scholar]
  75. Schuuring MJ, Bouma BJ, Cordina R, Gatzoulis MA, Budts W, Mullen MP, Vis JC, Celermajer D, Mulder BJM. Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int J Cardiol. 2013; 164::106110.
    [Google Scholar]
  76. 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::16811687.
    [Google Scholar]
  77. Derk G, Houser L, Miner P, Williams R, Moriarty J, Finn P, Alejos J, Aboulhosn J. Efficacy of Endothelin Blockade in Adults with Fontan Physiology. Congenit Heart Dis. 2014; 10:1:E11E16.
    [Google Scholar]
  78. Goldberg DJ, French B, Szwast AL, McBride MG, Marino BS, Mirarchi N, Hanna BD, Wernovsky G, Paridon SM, Rychik J. Impact of Sildenafil on Echocardiographic Indices of Myocardial Performance After the Fontan Operation. Pediatr Cardiol. 2012; 33::689696.
    [Google Scholar]
  79. Schuuring MJ, Vis JC, van Dijk APJ, van Melle JP, Vliegen HW, Pieper PG, Sieswerda GT, de Bruin-Bon RHACM, Mulder BJM, Bouma BJ. Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail. 2013; 15::690698.
    [Google Scholar]
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  • Article Type: Research Article
Keyword(s): congenital heart disease , Eisenmenger syndrome and pulmonary hypertension
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