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Pulmonary Hypertension and Eisenmenger Physiology

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Adult Congenital Heart Disease in Clinical Practice

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References

  1. 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;93:682–7.

    Article  Google Scholar 

  2. Eisenmenger V. Die angeborenen Defekte der Kammerscheidewände des Herzens. Zeitschr Klin Med. 1897;32:1–28.

    Google Scholar 

  3. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958;2:755–62.

    Article  CAS  Google Scholar 

  4. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice guidelines (writing committee to develop guidelines on the management of adults with congenital heart disease). Circulation. 2008;118:e714–833.

    PubMed  Google Scholar 

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

    Article  Google Scholar 

  6. Manes A, Palazzini M, Leci E, Bacchi Reggiani ML, Branzi A, Galie N. Current era survival of patients with pulmonary arterial hypertension associated with congenital heart disease: a comparison between clinical subgroups. Eur Heart J. 2014;35:716–24.

    Article  Google Scholar 

  7. 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;120:198–204.

    Article  CAS  Google Scholar 

  8. 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  Google Scholar 

  9. Alonso-Gonzalez R, Lopez-Guarch CJ, Subirana-Domenech MT, et al. Pulmonary hypertension and congenital heart disease: an insight from the REHAP National Registry. Int J Cardiol. 2015;184:717–23.

    Article  Google Scholar 

  10. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med. 1998;128:745–55.

    Article  CAS  Google Scholar 

  11. Tuder RM, Cool CD, Yeager M, Taraseviciene-Stewart L, Bull TM, Voelkel NF. The pathobiology of pulmonary hypertension. Endothelium Clin Chest Med. 2001;22:405–18.

    Article  CAS  Google Scholar 

  12. Post MC, Janssens S, Van de Werf F, Budts W. Responsiveness to inhaled nitric oxide is a predictor for mid-term survival in adult patients with congenital heart defects and pulmonary arterial hypertension. Eur Heart J. 2004;25:1651–6.

    Article  CAS  Google Scholar 

  13. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation. 2007;115:1039–50.

    Article  Google Scholar 

  14. 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;15:100–5.

    CAS  PubMed  Google Scholar 

  15. Cantor WJ, Harrison DA, Moussadji JS, et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol. 1999;84:677–81.

    Article  CAS  Google Scholar 

  16. Galie N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. Rev Esp Cardiol (Engl Ed). 2016;69:177.

    Article  Google Scholar 

  17. Roth TS, Aboulhosn JA. Pulmonary hypertension and congenital heart disease. Cardiol Clin. 2016;34:391–400.

    Article  Google Scholar 

  18. Frost AE, Quinones MA, Zoghbi WA, Noon GP. Reversal of pulmonary hypertension and subsequent repair of atrial septal defect after treatment with continuous intravenous epoprostenol. J Heart Lung Transplant. 2005;24:501–3.

    Article  Google Scholar 

  19. Schwerzmann M, Zafar M, McLaughlin PR, Chamberlain DW, Webb G, Granton J. Atrial septal defect closure in a patient with “irreversible” pulmonary hypertensive arteriopathy. Int J Cardiol. 2006;110:104–7.

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

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

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  23. Sun YJ, Yang T, Zeng WJ, et al. Impact of sildenafil on survival of patients with Eisenmenger syndrome. J Clin Pharmacol. 2013;53:611–8.

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  25. 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;6:424–31.

    Article  Google Scholar 

  26. 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;114:48–54.

    Article  CAS  Google Scholar 

  27. Gatzoulis MA, Beghetti M, Galie N, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008;127:27–32.

    Article  Google Scholar 

  28. Zuckerman WA, Leaderer D, Rowan CA, Mituniewicz JD, Rosenzweig EB. Ambrisentan for pulmonary arterial hypertension due to congenital heart disease. Am J Cardiol. 2011;107:1381–5.

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  31. 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;112:1834–9.

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  33. 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;155:378–82.

    Article  Google Scholar 

  34. 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;31:1124–31.

    Article  CAS  Google Scholar 

  35. Broberg CS, Ujita M, Prasad S, et al. Pulmonary arterial thrombosis in Eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity. J Am Coll Cardiol. 2007;50:634–42.

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  37. Ammash N, Warnes CA. Cerebrovascular events in adult patients with cyanotic congenital heart disease. J Am Coll Cardiol. 1996;28:768–72.

    Article  CAS  Google Scholar 

  38. Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J. 1998;19:1845–55.

    Article  CAS  Google Scholar 

  39. Oya H, Nagaya N, Satoh T, et al. Haemodynamic correlates and prognostic significance of serum uric acid in adult patients with Eisenmenger syndrome. Heart. 2000;84:53–8.

    Article  CAS  Google Scholar 

  40. Weiss BM, Zemp L, Seifert B, Hess OM. Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996. J Am Coll Cardiol. 1998;31:1650–7.

    Article  CAS  Google Scholar 

  41. Canobbio MM, Warnes CA, Aboulhosn J, et al. Management of pregnancy in patients with complex congenital heart disease: a scientific statement for healthcare professionals from the American Heart Association. Circulation. 2017;135(8):e50–87.

    Article  Google Scholar 

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Authors and Affiliations

  1. Massachusetts General Hospital, Boston, MA, USA

    Jonathan Kochav M.D.

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  1. Jonathan Kochav M.D.
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Editors and Affiliations

  1. Massachusetts General Hospital Heart Center, Boston, MA, USA

    Doreen DeFaria Yeh

  2. Massachusetts General Hospital Heart Center, Boston, MA, USA

    Ami Bhatt

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Kochav, J. (2018). Pulmonary Hypertension and Eisenmenger Physiology. In: DeFaria Yeh, D., Bhatt, A. (eds) Adult Congenital Heart Disease in Clinical Practice. In Clinical Practice. Springer, Cham. https://doi.org/10.1007/978-3-319-67420-9_9

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  • DOI: https://doi.org/10.1007/978-3-319-67420-9_9

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