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Evaluation and prognosis 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 systemic-to-pulmonary congenital cardiovascular communication, pulmonary arterial disease, and cyanosis is called Eisenmenger syndrome. The diagnosis of Eisenmenger syndrome implies that pulmonary arterial disease has developed as a consequence of increased pulmonary blood flow, and requires exclusion of other causes of pulmonary hypertension.

In 1897, Eisenmenger described a syndrome in which pulmonary vascular disease developed in patients with a nonrestrictive ventricular septal defect [1]. It was subsequently shown that pulmonary vascular disease could also occur with other congenital cardiac defects in which a systemic-to-pulmonary communication is present such as atrial shunts, other ventricular shunts, and aortic shunts (table 1).

Such conditions are often associated with an initial left-to-right shunt. Increased pulmonary blood flow as a result of the shunt leads to the development of pulmonary vascular disease with increased pulmonary vascular resistance (PVR). The shunt may then reverse and become right-to-left (figure 1A-B and algorithm 1) [2,3]. At this stage, the patient typically becomes cyanotic.

With the advent of surgical correction of congenital heart disease (CHD), the prevalence of Eisenmenger syndrome has declined but is not negligible. This topic will review the general features, evaluation, and prognosis of CHD-related pulmonary arterial hypertension and Eisenmenger syndrome. The pathology and pathophysiology of pulmonary hypertension in Eisenmenger syndrome are discussed in detail separately. (See "The epidemiology and pathogenesis of pulmonary arterial hypertension (Group 1)".)

The medical management of patients with Eisenmenger syndrome is discussed separately. (See "Management of Eisenmenger syndrome".)

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Literature review current through: Nov 2017. | This topic last updated: Jan 05, 2017.
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  1. Eisenmenger, V. Die Angeboren Defect der Kammer scheiderwand des Herzens. Z Klin Med Suppl 1897; 32:1.
  2. WOOD P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. I. Br Med J 1958; 2:701.
  3. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med 1998; 128:745.
  4. Saha A, Balakrishnan KG, Jaiswal PK, et al. Prognosis for patients with Eisenmenger syndrome of various aetiology. Int J Cardiol 1994; 45:199.
  5. Granton JT, Rabinovitch M. Pulmonary arterial hypertension in congenital heart disease. Cardiol Clin 2002; 20:441.
  6. Blieden LC, Moller JH. Small ventricular septal defect associated with severe pulmonary hypertension. Br Heart J 1984; 52:117.
  7. Bisset GS 3rd, Hirschfeld SS. Severe pulmonary hypertension associated with a small ventricular septal defect. Circulation 1983; 67:470.
  8. Bolger AP, Sharma R, Li W, et al. Neurohormonal activation and the chronic heart failure syndrome in adults with congenital heart disease. Circulation 2002; 106:92.
  9. Ohuchi H, Takasugi H, Ohashi H, et al. Stratification of pediatric heart failure on the basis of neurohormonal and cardiac autonomic nervous activities in patients with congenital heart disease. Circulation 2003; 108:2368.
  10. Mikami T, Kudo T, Sakurai N, et al. Mechanisms for development of functional tricuspid regurgitation determined by pulsed Doppler and two-dimensional echocardiography. Am J Cardiol 1984; 53:160.
  11. 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.
  12. Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19:1845.
  13. Warnes CA, Boger JE, Roberts WC. Eisenmenger ventricular septal defect with prolonged survival. Am J Cardiol 1984; 54:460.
  14. Diller GP, Dimopoulos K, Broberg CS, et al. Presentation, survival prospects, and predictors of death in Eisenmenger syndrome: a combined retrospective and case-control study. Eur Heart J 2006; 27:1737.
  15. Kempny A, Hjortshøj CS, Gu H, et al. Predictors of Death in Contemporary Adult Patients With Eisenmenger Syndrome: A Multicenter Study. Circulation 2017; 135:1432.
  16. Diller GP, Kempny A, Inuzuka R, et al. Survival prospects of treatment naïve patients with Eisenmenger: a systematic review of the literature and report of own experience. Heart 2014; 100:1366.
  17. 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.
  18. Hayden AM, Robert RC, Kriett JM, et al. Primary diagnosis predicts prognosis of lung transplant candidates. Transplantation 1993; 55:1048.
  19. Hopkins WE, Waggoner AD. Severe pulmonary hypertension without right ventricular failure: the unique hearts of patients with Eisenmenger syndrome. Am J Cardiol 2002; 89:34.
  20. 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.
  21. Sheehan R, Perloff JK, Fishbein MC, et al. Pulmonary neovascularity: a distinctive radiographic finding in Eisenmenger syndrome. Circulation 2005; 112:2778.
  22. 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.
  23. Silversides CK, Granton JT, Konen E, et al. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol 2003; 42:1982.
  24. HEATH D, EDWARDS JE. The pathology of hypertensive pulmonary vascular disease; a description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 1958; 18:533.
  25. Rabinovitch M, Haworth SG, Castaneda AR, et al. Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease. Circulation 1978; 58:1107.