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Management of Eisenmenger syndrome

Heidi M Connolly, MD, FASE
Section Editor
John K Triedman, MD
Deputy Editor
Susan B Yeon, MD, JD, FACC


The triad of congenital systemic-to-pulmonary communication, pulmonary arterial disease, and cyanosis is called Eisenmenger syndrome. The diagnosis of Eisenmenger syndrome implies that the development of pulmonary arterial disease is a consequence of increased pulmonary blood flow, and requires exclusion of other causes of pulmonary arterial hypertension (PAH).

Management of Eisenmenger syndrome patients includes treatment pertinent to all patients with cyanotic heart disease as well as recommendations specific to CHD-PAH and Eisenmenger syndrome. Issues specific to CHD-PAH and Eisenmenger syndrome will be reviewed here.

Evaluation and prognosis of Eisenmenger syndrome and recommendations for patients with cyanotic heart disease (including those with Eisenmenger syndrome) are discussed separately. (See "Evaluation and prognosis of Eisenmenger syndrome" and "Medical management of cyanotic congenital heart disease in adults".)


Overview — Centers specializing in the management of adult patients with congenital heart disease (CHD) provide the optimal combination of clinical expertise, facilities for advanced diagnosis and therapy, structured care, and access to new and evolving therapies. Care of adults with CHD-related pulmonary arterial hypertension (PAH) including those with Eisenmenger syndrome should be performed in centers that have shared expertise and training in both adult CHD and PAH [1].

In the past, the management of patients with Eisenmenger syndrome was primarily conservative, with an emphasis on regular informed cardiovascular follow-up. Subsequent clinical research evaluated the use of therapies designed to improve patient survival and functional capacity. Pulmonary vasodilator therapy may improve hemodynamics and quality of life in some patients. Other important aspects of management include avoidance of high-risk situations outlined below, extreme caution when undertaking noncardiac surgery, and specific attention to hematologic issues. Heart and lung transplantation, or lung transplantation with repair of the cardiac defect, is appropriate in some patients with severe disease.

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Literature review current through: Dec 2017. | This topic last updated: Dec 27, 2016.
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  1. 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.
  2. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med 1998; 128:745.
  3. Niwa K, Perloff JK, Kaplan S, et al. Eisenmenger syndrome in adults: ventricular septal defect, truncus arteriosus, univentricular heart. J Am Coll Cardiol 1999; 34:223.
  4. Gleicher N, Midwall J, Hochberger D, Jaffin H. Eisenmenger's syndrome and pregnancy. Obstet Gynecol Surv 1979; 34:721.
  5. Yentis SM, Steer PJ, Plaat F. Eisenmenger's syndrome in pregnancy: maternal and fetal mortality in the 1990s. Br J Obstet Gynaecol 1998; 105:921.
  6. 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. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol 2007; 49:2303.
  8. Bédard E, Dimopoulos K, Gatzoulis MA. Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension? Eur Heart J 2009; 30:256.
  9. Miño M, Arjona JE, Cordón J, et al. Success rate and patient satisfaction with the Essure sterilisation in an outpatient setting: a prospective study of 857 women. BJOG 2007; 114:763.
  10. Famuyide AO, Hopkins MR, El-Nashar SA, et al. Hysteroscopic sterilization in women with severe cardiac disease: experience at a tertiary center. Mayo Clin Proc 2008; 83:431.
  11. European Society of Gynecology (ESG), Association for European Paediatric Cardiology (AEPC), German Society for Gender Medicine (DGesGM), 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; 32:3147.
  12. Elkayam U, Gleicher N. Cardiac problems in pregnancy. I. Maternal aspects: the approach to the pregnant patient with heart disease. JAMA 1984; 251:2838.
  13. Hankins GD, Berryman GK, Scott RT Jr, Hood D. Maternal arterial desaturation with 15-methyl prostaglandin F2 alpha for uterine atony. Obstet Gynecol 1988; 72:367.
  14. Kelion AD, Webb TP, Gardner MA, et al. The warm-up effect protects against ischemic left ventricular dysfunction in patients with angina. J Am Coll Cardiol 2001; 37:705.
  15. Avila WS, Grinberg M, Snitcowsky R, et al. Maternal and fetal outcome in pregnant women with Eisenmenger's syndrome. Eur Heart J 1995; 16:460.
  16. Pitts JA, Crosby WM, Basta LL. Eisenmenger's syndrome in pregnancy: does heparin prophylaxis improve the maternal mortality rate? Am Heart J 1977; 93:321.
  17. Kahn ML. Eisenmenger's syndrome in pregnancy. N Engl J Med 1993; 329:887.
  18. Robinson S. Pulmonary artery catheters in Eisenmenger's syndrome: many risks, few benefits. Anesthesiology 1983; 58:588.
  19. Midwall J, Jaffin H, Herman MV, Kupersmith J. Shunt flow and pulmonary hemodynamics during labor and delivery in the Eisenmenger syndrome. Am J Cardiol 1978; 42:299.
  20. Broberg CS, Uebing A, Cuomo L, et al. Adult patients with Eisenmenger syndrome report flying safely on commercial airlines. Heart 2007; 93:1599.
  21. Harinck E, Hutter PA, Hoorntje TM, et al. Air travel and adults with cyanotic congenital heart disease. Circulation 1996; 93:272.
  22. 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.
  23. Perloff JK, Hart EM, Greaves SM, et al. Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension. Am J Cardiol 2003; 92:182.
  24. Silversides CK, Granton JT, Konen E, et al. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol 2003; 42:1982.
  25. Altman R, Scazziota A, Rouvier J, et al. Coagulation and fibrinolytic parameters in patients with pulmonary hypertension. Clin Cardiol 1996; 19:549.
  26. Hassell KL. Altered hemostasis in pulmonary hypertension. Blood Coagul Fibrinolysis 1998; 9:107.
  27. Ammash NM, Connolly HM, Abel MD, Warnes CA. Noncardiac surgery in Eisenmenger syndrome. J Am Coll Cardiol 1999; 33:222.
  28. Graham TP Jr, Driscoll DJ, Gersony WM, et al. Task Force 2: congenital heart disease. J Am Coll Cardiol 2005; 45:1326.
  29. Galiè 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.
  30. Christensen DD, McConnell ME, Book WM, Mahle WT. Initial experience with bosentan therapy in patients with the Eisenmenger syndrome. Am J Cardiol 2004; 94:261.
  31. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation 1999; 99:1858.
  32. Fernandes SM, Newburger JW, Lang P, et al. Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology. Am J Cardiol 2003; 91:632.
  33. Chau EM, Fan KY, Chow WH. Effects of chronic sildenafil in patients with Eisenmenger syndrome versus idiopathic pulmonary arterial hypertension. Int J Cardiol 2007; 120:301.
  34. 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.
  35. Yang SI, Chung WJ, Jung SH, Choi DY. Effects of inhaled iloprost on congenital heart disease with Eisenmenger syndrome. Pediatr Cardiol 2012; 33:744.
  36. 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.
  37. 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.
  38. Waddell TK, Bennett L, Kennedy R, et al. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant 2002; 21:731.
  39. 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.