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Pulmonary Arterial Hypertension

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Heart Failure in Adult Congenital Heart Disease

Part of the book series: Congenital Heart Disease in Adolescents and Adults ((CHDAA))

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Abstract

Pulmonary arterial hypertension (PAH) frequently complicates congenital heart disease (CHD). In the current era, life expectancy of patients with PAH-CHD has increased as a consequence of their advanced management in specialized centers and the widespread use of PAH-targeted therapy. Even though, progress has been made in this field, mortality still remains high, primarily due to right heart failure. Aggressive targeting of the underlying pulmonary hypertension and optimal management of fluid balance play a key role in preventing progressive heart failure due to right ventricle dysfunction.

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Abbreviations

ASD:

Atrial septal defect

CHD:

Congenital heart disease

CMR:

Cardiac magnetic resonance

CT:

Computed tomography

ES:

Eisenmenger syndrome

NT-proBNP:

N-terminal pro-B-type natriuretic peptide

NYHA:

New York Heart Association

PAH:

Pulmonary arterial hypertension

PDA:

Patent ductus arteriosus

PH:

Pulmonary hypertension

RV:

Right ventricle

TAPSE:

Tricuspid annular plane systolic excursion

VSD:

Ventricular septal defect

References

  1. Diller GP, Kempny A, Alonso-Gonzalez R, et al. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a Large Tertiary Centre. Circulation. 2015;132(22):2118–25.

    Article  PubMed  Google Scholar 

  2. Galie N, Humbert M, Vachiery JL, et al. ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint 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: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J. 2016;37(1):67–119.

    Article  PubMed  Google Scholar 

  3. Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D34–41.

    Article  PubMed  Google Scholar 

  4. Duffels MG, Engelfriet PM, Berger RM, et al. Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. Int J Cardiol. 2007;120(2):198–204.

    Article  CAS  PubMed  Google Scholar 

  5. Bouzas B, Gatzoulis MA. Pulmonary arterial hypertension in adults with congenital heart disease. Rev Esp Cardiol. 2005;58(5):465–9.

    Article  PubMed  Google Scholar 

  6. Gatzoulis MA, Alonso-Gonzalez R, Beghetti M. Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. Eur Respir Rev. 2009;18(113):154–61.

    Article  CAS  PubMed  Google Scholar 

  7. Van de Bruaene A, Delcroix M, Pasquet A, et al. The Belgian Eisenmenger syndrome registry: implications for treatment strategies? Acta Cardiol. 2009;64(4):447–53.

    Article  PubMed  Google Scholar 

  8. Engelfriet PM, Duffels MG, Moller T, et al. Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart. 2007;93(6):682–7.

    Article  PubMed  Google Scholar 

  9. Lange RA, Brickner ME. Improving survival in patients with Eisenmenger syndrome: are we any closer? Circulation. 2017;135(15):1441–3.

    Article  PubMed  Google Scholar 

  10. Moceri P, Kempny A, Liodakis E, et al. Physiological differences between various types of Eisenmenger syndrome and relation to outcome. Int J Cardiol. 2015;179:455–60.

    Article  PubMed  Google Scholar 

  11. Hopkins WE, Ochoa LL, Richardson GW, Trulock EP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996;15(1 Pt 1):100–5.

    PubMed  CAS  Google Scholar 

  12. Diller GP, Kempny A, Inuzuka R, et al. Survival prospects of treatment naive patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart. 2014;100(17):1366–72.

    Article  PubMed  Google Scholar 

  13. Manes A, Palazzini M, Leci E, et al. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. 2014;35(11):716–24.

    Article  PubMed  Google Scholar 

  14. Dimopoulos K, Wort SJ, Gatzoulis MA. Pulmonary hypertension related to congenital heart disease: a call for action. Eur Heart J. 2014;35(11):691–700.

    Article  PubMed  Google Scholar 

  15. Niedeggen A, Skobel E, Haager P, et al. Comparison of the 6-minute walk test with established parameters for assessment of cardiopulmonary capacity in adults with complex congenital cardiac disease. Cardiol Young. 2005;15(4):385–90.

    Article  PubMed  Google Scholar 

  16. Diller GP, Dimopoulos K, Okonko D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112(6):828–35.

    Article  PubMed  Google Scholar 

  17. Kempny A, Dimopoulos K, Uebing A, et al. Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life—single centre experience and review of published data. Eur Heart J. 2012;33(11):1386–96.

    Article  PubMed  Google Scholar 

  18. Inuzuka R, Diller GP, Borgia F, et al. Comprehensive use of cardiopulmonary exercise testing identifies adults with congenital heart disease at increased mortality risk in the medium term. Circulation. 2012;125(2):250–9.

    Article  PubMed  Google Scholar 

  19. Yasue H, Yoshimura M, Sumida H, et al. Localization and mechanism of secretion of B-type natriuretic peptide in comparison with those of A-type natriuretic peptide in normal subjects and patients with heart failure. Circulation. 1994;90(1):195–203.

    Article  CAS  PubMed  Google Scholar 

  20. Reardon LC, Williams RJ, Houser LS, et al. Usefulness of serum brain natriuretic peptide to predict adverse events in patients with the Eisenmenger syndrome. Am J Cardiol. 2012;110(10):1523–6.

    Article  CAS  PubMed  Google Scholar 

  21. Giannakoulas G, Mouratoglou SA, Gatzoulis MA, Karvounis H. Blood biomarkers and their potential role in pulmonary arterial hypertension associated with congenital heart disease. A systematic review. Int J Cardiol. 2014;174(3):618–23.

    Article  PubMed  Google Scholar 

  22. Schuijt MTU, Blok IM, Zwinderman AH, et al. Mortality in pulmonary arterial hypertension due to congenital heart disease: serial changes improve prognostication. Int J Cardiol. 2017.

    Google Scholar 

  23. D’Alto M, Romeo E, Argiento P, et al. Accuracy and precision of echocardiography versus right heart catheterization for the assessment of pulmonary hypertension. Int J Cardiol. 2013;168(4):4058–62.

    Article  PubMed  Google Scholar 

  24. D’Alto M, Dimopoulos K, Budts W, et al. Multimodality imaging in congenital heart disease-related pulmonary arterial hypertension. Heart. 2016;102(12):910–8.

    Article  CAS  PubMed  Google Scholar 

  25. D’Alto M, Romeo E, Argiento P, et al. Pulmonary arterial hypertension: the key role of echocardiography. Echocardiography. 2015;32 Suppl 1:S23–37.

    Article  PubMed  Google Scholar 

  26. Jensen AS, Broberg CS, Rydman R, et al. Impaired right, left, or biventricular function and resting oxygen saturation are associated with mortality in Eisenmenger syndrome: a clinical and cardiovascular magnetic resonance study. Circ Cardiovasc Imaging. 2015;8(12).

    Google Scholar 

  27. Devaraj A, Hansell DM. Computed tomography signs of pulmonary hypertension: old and new observations. Clin Radiol. 2009;64(8):751–60.

    Article  CAS  PubMed  Google Scholar 

  28. Babu-Narayan SV, Giannakoulas G, Valente AM, Li W, Gatzoulis MA. Imaging of congenital heart disease in adults. Eur Heart J. 2016;37(15):1182–95.

    Article  PubMed  Google Scholar 

  29. Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31(23):2915–57.

    Article  PubMed  Google Scholar 

  30. Mylotte D, Pilote L, Ionescu-Ittu R, et al. Specialized adult congenital heart disease care: the impact of policy on mortality. Circulation. 2014;129(18):1804–12.

    Article  PubMed  Google Scholar 

  31. Diller GP, Korten MA, Bauer UM, et al. Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects. Eur Heart J. 2016;37(18):1449–55.

    Article  PubMed  PubMed Central  Google Scholar 

  32. Regitz-Zagrosek V, Blomstrom Lundqvist C, Borghi C, et al. 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(24):3147–97.

    Article  PubMed  Google Scholar 

  33. Becker-Grunig T, Klose H, Ehlken N, et al. Efficacy of exercise training in pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol. 2013;168(1):375–81.

    Article  PubMed  Google Scholar 

  34. Budts W, Borjesson M, Chessa M, et al. Physical activity in adolescents and adults with congenital heart defects: individualized exercise prescription. Eur Heart J. 2013;34(47):3669–74.

    Article  PubMed  Google Scholar 

  35. Giannakoulas G, Dimopoulos K. Exercise training in congenital heart disease: should we follow the heart failure paradigm? Int J Cardiol. 2010;138(2):109–11.

    Article  PubMed  Google Scholar 

  36. Rosove MH, Perloff JK, Hocking WG, et al. Chronic hypoxaemia and decompensated erythrocytosis in cyanotic congenital heart disease. Lancet. 1986;2(8502):313–5.

    Article  CAS  PubMed  Google Scholar 

  37. 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(3):307–12.

    Article  PubMed  Google Scholar 

  38. Sandoval J, Aguirre JS, Pulido T, et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med. 2001;164(9):1682–7.

    Article  CAS  PubMed  Google Scholar 

  39. Silversides CK, Granton JT, Konen E, et al. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol. 2003;42(11):1982–7.

    Article  PubMed  Google Scholar 

  40. Broberg C, Ujita M, Babu-Narayan S, et al. Massive pulmonary artery thrombosis with haemoptysis in adults with Eisenmenger’s syndrome: a clinical dilemma. Heart. 2004;90(11):e63.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Broberg CS, Jayaweera AR, Diller GP, et al. Seeking optimal relation between oxygen saturation and hemoglobin concentration in adults with cyanosis from congenital heart disease. Am J Cardiol. 2011;107(4):595–9.

    Article  CAS  PubMed  Google Scholar 

  42. Ammash NM, Connolly HM, Abel MD, Warnes CA. Noncardiac surgery in Eisenmenger syndrome. J Am Coll Cardiol. 1999;33(1):222–7.

    Article  CAS  PubMed  Google Scholar 

  43. Bennett JM, Ehrenfeld JM, Markham L, Eagle SS. Anesthetic management and outcomes for patients with pulmonary hypertension and intracardiac shunts and Eisenmenger syndrome: a review of institutional experience. J Clin Anesth. 2014;26(4):286–93.

    Article  PubMed  Google Scholar 

  44. Granton J, Mercier O, De Perrot M. Management of severe pulmonary arterial hypertension. Semin Respir Crit Care Med. 2013;34(5):700–13.

    Article  PubMed  Google Scholar 

  45. Hoeper MM, Granton J. Intensive care unit management of patients with severe pulmonary hypertension and right heart failure. Am J Respir Crit Care Med. 2011;184(10):1114–24.

    Article  CAS  PubMed  Google Scholar 

  46. Price LC, Wort SJ, Finney SJ, Marino PS, Brett SJ. Pulmonary vascular and right ventricular dysfunction in adult critical care: current and emerging options for management: a systematic literature review. Crit Care. 2010;14(5):R169.

    Article  PubMed  PubMed Central  Google Scholar 

  47. Bansal S, Lindenfeld J, Schrier RW. Sodium retention in heart failure and cirrhosis: potential role of natriuretic doses of mineralocorticoid antagonist? Circ Heart Fail. 2009;2(4):370–6.

    Article  CAS  PubMed  Google Scholar 

  48. Trojnarska O. Heart failure in the adult patient with congenital heart disease. Cardiol J. 2007;14(2):127–36.

    PubMed  Google Scholar 

  49. Hopkins WE, Kelly DP. Angiotensin-converting enzyme inhibitors in adults with cyanotic congenital heart disease. Am J Cardiol. 1996;77(5):439–40.

    Article  CAS  PubMed  Google Scholar 

  50. Peacock A, Ross K. Pulmonary hypertension: a contraindication to the use of {beta}-adrenoceptor blocking agents. Thorax. 2010;65(5):454–5.

    Article  CAS  PubMed  Google Scholar 

  51. Bogaard HJ, Natarajan R, Mizuno S, et al. Adrenergic receptor blockade reverses right heart remodeling and dysfunction in pulmonary hypertensive rats. Am J Respir Crit Care Med. 2010;182(5):652–60.

    Article  CAS  PubMed  Google Scholar 

  52. Grinnan D, Bogaard HJ, Grizzard J, et al. Treatment of group I pulmonary arterial hypertension with carvedilol is safe. Am J Respir Crit Care Med. 2014;189(12):1562–4.

    Article  PubMed  Google Scholar 

  53. van Riel AC, Schuuring MJ, van Hessen ID, et al. Treatment of pulmonary arterial hypertension in congenital heart disease in Singapore versus the Netherlands: age exceeds ethnicity in influencing clinical outcome. Neth Heart J. 2016;24(6):410–6.

    Article  PubMed  PubMed Central  Google Scholar 

  54. Dimopoulos K, Inuzuka R, Goletto S, et al. Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension. Circulation. 2010;121(1):20–5.

    Article  PubMed  Google Scholar 

  55. Diller GP, Alonso-Gonzalez R, Dimopoulos K, et al. Disease targeting therapies in patients with Eisenmenger syndrome: response to treatment and long-term efficiency. Int J Cardiol. 2013;167(3):840–7.

    Article  PubMed  Google Scholar 

  56. Adriaenssens T, Delcroix M, Van Deyk K, Budts W. Advanced therapy may delay the need for transplantation in patients with the Eisenmenger syndrome. Eur Heart J. 2006;27(12):1472–7.

    Article  CAS  PubMed  Google Scholar 

  57. Cacoub P, Dorent R, Maistre G, et al. Endothelin-1 in primary pulmonary hypertension and the Eisenmenger syndrome. Am J Cardiol. 1993;71(5):448–50.

    Article  CAS  PubMed  Google Scholar 

  58. Galie N, Manes A, Branzi A. The endothelin system in pulmonary arterial hypertension. Cardiovasc Res. 2004;61(2):227–37.

    Article  CAS  PubMed  Google Scholar 

  59. Galie N, Beghetti M, Gatzoulis MA, et al. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006;114(1):48–54.

    Article  CAS  PubMed  Google Scholar 

  60. Vis JC, Duffels MG, Mulder P, et al. Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease. Int J Cardiol. 2013;164(1):64–9.

    Article  PubMed  Google Scholar 

  61. 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(9):1381–5.

    Article  CAS  PubMed  Google Scholar 

  62. Pulido T, Adzerikho I, Channick RN, et al. Macitentan and morbidity and mortality in pulmonary arterial hypertension. N Engl J Med. 2013;369(9):809–18.

    Article  CAS  PubMed  Google Scholar 

  63. Ltd AP. Actelion announces results of the MAESTRO study with macitentan in patients with pulmonary arterial hypertension due to Eisenmenger syndrome. 2017.

    Google Scholar 

  64. Herbert S, Gin-Sing W, Howard L, Tulloh RM. Early experience of Macitentan for pulmonary arterial hypertension in adult congenital heart disease. Heart Lung Circ. 2017.

    Google Scholar 

  65. Blok IM, van Riel A, van Dijk APJ, Mulder BJM, Bouma BJ. From bosentan to macitentan for pulmonary arterial hypertension and adult congenital heart disease: further improvement? Int J Cardiol. 2017;227:51–2.

    Article  PubMed  Google Scholar 

  66. Galie N, Ghofrani HA, Torbicki A, et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med. 2005;353(20):2148–57.

    Article  CAS  PubMed  Google Scholar 

  67. 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(4):851.e851–5.

    Article  Google Scholar 

  68. 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(3):372–6.

    Article  PubMed  Google Scholar 

  69. Chau EM, Fan KY, Chow WH. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol. 2007;120(3):301–5.

    Article  PubMed  Google Scholar 

  70. Zhang ZN, Jiang X, Zhang R, et al. Oral sildenafil treatment for Eisenmenger syndrome: a prospective, open-label, multicentre study. Heart. 2011;97(22):1876–81.

    Article  CAS  PubMed  Google Scholar 

  71. Galie N, Brundage BH, Ghofrani HA, et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation. 2009;119(22):2894–903.

    Article  CAS  PubMed  Google Scholar 

  72. Mukhopadhyay S, Sharma M, Ramakrishnan S, et al. Phosphodiesterase-5 inhibitor in Eisenmenger syndrome: a preliminary observational study. Circulation. 2006;114(17):1807–10.

    Article  CAS  PubMed  Google Scholar 

  73. 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(5):424–31.

    Article  PubMed  Google Scholar 

  74. Ghofrani HA, Galie N, Grimminger F, et al. Riociguat for the treatment of pulmonary arterial hypertension. N Engl J Med. 2013;369(4):330–40.

    Article  CAS  PubMed  Google Scholar 

  75. Rosenkranz S, Ghofrani HA, Beghetti M, et al. Riociguat for pulmonary arterial hypertension associated with congenital heart disease. Heart. 2015;101(22):1792–9.

    Article  CAS  PubMed  Google Scholar 

  76. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. 1999;99(14):1858–65.

    Article  CAS  PubMed  Google Scholar 

  77. Thomas IC, Glassner-Kolmin C, Gomberg-Maitland M. Long-term effects of continuous prostacyclin therapy in adults with pulmonary hypertension associated with congenital heart disease. Int J Cardiol. 2013;168(4):4117–21.

    Article  PubMed  Google Scholar 

  78. Simonneau G, Barst RJ, Galie N, et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med. 2002;165(6):800–4.

    Article  PubMed  Google Scholar 

  79. 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):1834–9.

    Article  CAS  PubMed  Google Scholar 

  80. Sitbon O, Channick R, Chin KM, et al. Selexipag for the treatment of pulmonary arterial hypertension. N Engl J Med. 2015;373(26):2522–33.

    Article  CAS  PubMed  Google Scholar 

  81. Fox BD, Shtraichman O, Langleben D, Shimony A, Kramer MR. Combination therapy for pulmonary arterial hypertension: a systematic review and meta-analysis. Can J Cardiol. 2016;32(12):1520–30.

    Article  PubMed  Google Scholar 

  82. Iversen K, Jensen AS, Jensen TV, Vejlstrup NG, Sondergaard L. Combination therapy with bosentan and sildenafil in Eisenmenger syndrome: a randomized, placebo-controlled, double-blinded trial. Eur Heart J. 2010;31(9):1124–31.

    Article  CAS  PubMed  Google Scholar 

  83. 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(3):378–82.

    Article  PubMed  Google Scholar 

  84. Schuuring MJ, Bouma BJ, Cordina R, et al. Treatment of segmental pulmonary artery hypertension in adults with congenital heart disease. Int J Cardiol. 2013;164(1):106–10.

    Article  PubMed  Google Scholar 

  85. Duffels MG, Vis JC, van Loon RL, et al. Down patients with Eisenmenger syndrome: is bosentan treatment an option? Int J Cardiol. 2009;134(3):378–83.

    Article  PubMed  Google Scholar 

  86. Duffels MG, Vis JC, van Loon RL, et al. 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(9):1309–15.

    Article  CAS  PubMed  Google Scholar 

  87. D’Alto M, Romeo E, Argiento P, et al. Therapy for pulmonary arterial hypertension due to congenital heart disease and Down’s syndrome. Int J Cardiol. 2013;164(3):323–6.

    Article  PubMed  Google Scholar 

  88. Stout KK, Broberg CS, Book WM, et al. Chronic heart failure in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133(8):770–801.

    PubMed  Google Scholar 

  89. Ross HJ, Law Y, Book WM, et al. Transplantation and mechanical circulatory support in congenital heart disease: a scientific statement from the American Heart Association. Circulation. 2016;133(8):802–20.

    PubMed  Google Scholar 

  90. Budts W, Roos-Hesselink J, Radle-Hurst T, et al. Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology. Eur Heart J. 2016;37(18):1419–27.

    Article  PubMed  PubMed Central  Google Scholar 

  91. Patel ND, Weiss ES, Allen JG, et al. Heart transplantation for adults with congenital heart disease: analysis of the United Network for organ sharing database. Ann Thorac Surg. 2009;88(3):814–21. discussion 821-812.

    Article  PubMed  Google Scholar 

  92. Stoica SC, McNeil KD, Perreas K, et al. Heart-lung transplantation for Eisenmenger syndrome: early and long-term results. Ann Thorac Surg. 2001;72(6):1887–91.

    Article  CAS  PubMed  Google Scholar 

  93. Waddell TK, Bennett L, Kennedy R, Todd TR, Keshavjee SH. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant. 2002;21(7):731–7.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Cardiology Department, AHEPA University Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece

    Despina Ntiloudi M.D. & George Giannakoulas M.D., Ph.D.

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  1. Despina Ntiloudi M.D.
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  2. George Giannakoulas M.D., Ph.D.
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Correspondence to George Giannakoulas M.D., Ph.D. .

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  1. Royal Brompton Hospital, London, United Kingdom

    Lorna Swan

  2. ATTIKON University Hospital, Athens University, Athens, Greece

    Alexandra A. Frogoudaki

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Ntiloudi, D., Giannakoulas, G. (2018). Pulmonary Arterial Hypertension. In: Swan, L., Frogoudaki, A. (eds) Heart Failure in Adult Congenital Heart Disease. Congenital Heart Disease in Adolescents and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-77803-7_8

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