eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Eisenmenger Syndrome: Multimedia

Author: Mikhael F El-Chami, MD, Fellow, Department of Cardiology, Emory University School of Medicine
Coauthor(s): Charles D Searles Jr, MD, Assistant Professor of Medicine, Division of Cardiology, Emory University School of Medicine; Consulting Staff, Division of Cardiology, Director of Stress Echo Laboratory, Grady Memorial Hospital
Contributor Information and Disclosures

Updated: Jun 5, 2008

Multimedia

Apical 4-chamber transthoracic view demonstrating...Media file 1: Apical 4-chamber transthoracic view demonstrating an ostium primum atrial septal defect (ASD) with enlarged right-side chambers. RA = right atrium, RV = right ventricle, LA = left atrium, LV = left ventricle.
Apical 4-chamber transthoracic view demonstrating...

Apical 4-chamber transthoracic view demonstrating an ostium primum atrial septal defect (ASD) with enlarged right-side chambers. RA = right atrium, RV = right ventricle, LA = left atrium, LV = left ventricle.

This radiograph reveals an enlarged right heart a...Media file 2: This radiograph reveals an enlarged right heart and pulmonary artery dilatation in a 24-year-old woman with an unrestricted patent ductus arteriosus (PDA) and Eisenmenger syndrome.
This radiograph reveals an enlarged right heart a...

This radiograph reveals an enlarged right heart and pulmonary artery dilatation in a 24-year-old woman with an unrestricted patent ductus arteriosus (PDA) and Eisenmenger syndrome.

This CT chest scan shows a large, unrestricted pa...Media file 3: This CT chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome. Asc Ao = ascending aorta.
This CT chest scan shows a large, unrestricted pa...

This CT chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome. Asc Ao = ascending aorta.

This transthoracic apical 4-chamber segment shows...Media file 4: This transthoracic apical 4-chamber segment shows color Doppler flow across the interatrial septum at the site of a large ostium primum atrial septal defect (ASD). RA = right atrium, LA = left atrium.
This transthoracic apical 4-chamber segment shows...

This transthoracic apical 4-chamber segment shows color Doppler flow across the interatrial septum at the site of a large ostium primum atrial septal defect (ASD). RA = right atrium, LA = left atrium.

This transesophageal image is from the mid esopha...Media file 5: This transesophageal image is from the mid esophagus of a patient with Eisenmenger syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely dilated pulmonary artery. Pulm a = pulmonary artery, Asc Ao = ascending aorta.
This transesophageal image is from the mid esopha...

This transesophageal image is from the mid esophagus of a patient with Eisenmenger syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely dilated pulmonary artery. Pulm a = pulmonary artery, Asc Ao = ascending aorta.

This is a color Doppler interrogation of the tric...Media file 6: This is a color Doppler interrogation of the tricuspid valve in a case of Eisenmenger syndrome. It demonstrates an elevated estimated right ventricular systolic pressure of 106 mm Hg + right atrial pressure, reflecting pulmonary hypertension. TR = tricuspid regurgitation.
This is a color Doppler interrogation of the tric...

This is a color Doppler interrogation of the tricuspid valve in a case of Eisenmenger syndrome. It demonstrates an elevated estimated right ventricular systolic pressure of 106 mm Hg + right atrial pressure, reflecting pulmonary hypertension. TR = tricuspid regurgitation.

This is the transthoracic Doppler examination of ...Media file 7: This is the transthoracic Doppler examination of the pulmonic valve in a 24-year-old woman with Eisenmenger syndrome secondary to an uncorrected ostium primum atrial septal defect (ASD). This reveals an elevated estimated pulmonary artery diastolic pressure of 51 mm Hg + right atrial pressure. PR = pulmonic regurgitation.
This is the transthoracic Doppler examination of ...

This is the transthoracic Doppler examination of the pulmonic valve in a 24-year-old woman with Eisenmenger syndrome secondary to an uncorrected ostium primum atrial septal defect (ASD). This reveals an elevated estimated pulmonary artery diastolic pressure of 51 mm Hg + right atrial pressure. PR = pulmonic regurgitation.

More on Eisenmenger Syndrome

Overview: Eisenmenger Syndrome
Differential Diagnoses & Workup: Eisenmenger Syndrome
Treatment & Medication: Eisenmenger Syndrome
Follow-up: Eisenmenger Syndrome
Multimedia: Eisenmenger Syndrome
References

References

  1. Salehian O, Schwerzmann M, Rambihar S, Silver D, Siu S, Webb G, et al. Left ventricular dysfunction and mortality in adult patients with Eisenmenger syndrome. Congenit Heart Dis. May 2007;2(3):156-64. [Medline].

  2. Bowyer JJ, Busst CM, Denison DM, Shinebourne EA. Effect of long term oxygen treatment at home in children with pulmonary vascular disease. Br Heart J. Apr 1986;55(4):385-90. [Medline].

  3. Sandoval J, Aguirre JS, Pulido T, Martinez-Guerra ML, Santos E, Alvarado P. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med. Nov 1 2001;164(9):1682-7. [Medline].

  4. Harinck E, Hutter PA, Hoorntje TM. Air travel and adults with cyanotic congenital heart disease. Circulation. Jan 15 1996;93(2):272-6. [Medline].

  5. Rosenzweig EB, Kerstein D, Barst RJ. Long-term prostacyclin for pulmonary hypertension with associated congenital heart defects. Circulation. Apr 13 1999;99(14):1858-65. [Medline].

  6. Fernandes SM, Newburger JW, Lang P, Pearson DD, Feinstein JA, Gauvreau K. Usefulness of epoprostenol therapy in the severely ill adolescent/adult with Eisenmenger physiology. Am J Cardiol. Mar 1 2003;91(5):632-5. [Medline].

  7. Christensen DD, McConnell ME, Book WM, Mahle WT. Initial experience with bosentan therapy in patients with the Eisenmenger syndrome. Am J Cardiol. Jul 15 2004;94(2):261-3. [Medline].

  8. Schulze-Neick I, Gilbert N, Ewert R, Witt C, Gruenig E, Enke B. Adult patients with congenital heart disease and pulmonary arterial hypertension: first open prospective multicenter study of bosentan therapy. Am Heart J. Oct 2005;150(4):716. [Medline].

  9. Galiè N, Beghetti M, Gatzoulis MA, Granton J, Berger RM, Lauer A. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. Jul 4 2006;114(1):48-54. [Medline].

  10. Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RM, Lauer A, et al. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: Results of the BREATHE-5 open-label extension study. Int J Cardiol. Jul 19 2007;[Medline].

  11. 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. Jun 2006;27(12):1472-7. [Medline].

  12. 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. Apr 2006;151(4):851.e1-5. [Medline].

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

  14. Mukhopadhyay S, Sharma M, Ramakrishnan S, Yusuf J, Gupta MD, Bhamri N. Phosphodiesterase-5 inhibitor in Eisenmenger syndrome: a preliminary observational study. Circulation. Oct 24 2006;114(17):1807-10. [Medline].

  15. Silversides CK, Granton JT, Konen E, Hart MA, Webb GD, Therrien J. Pulmonary thrombosis in adults with Eisenmenger syndrome. J Am Coll Cardiol. Dec 3 2003;42(11):1982-7. [Medline].

  16. Dajani AS, Taubert KA, Wilson W, et al. Prevention of bacterial endocarditis. Recommendations by the American Heart Association. Circulation. Jul 1 1997;96(1):358-66. [Medline].

  17. Linderkamp O, Klose HJ, Betke K, Brodherr-Heberlein S, Bühlmeyer K, Kelson S. Increased blood viscosity in patients with cyanotic congenital heart disease and iron deficiency. J Pediatr. Oct 1979;95(4):567-9. [Medline].

  18. Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. Second of two parts. N Engl J Med. Feb 3 2000;342(5):334-42. [Medline].

  19. Broberg CS, Uebing A, Cuomo L, Thein SL, Papadopoulos MG, Gatzoulis MA. Adult patients with Eisenmenger syndrome report flying safely on commercial airlines. Heart. Dec 2007;93(12):1599-603. [Medline].

  20. Avila WS, Grinberg M, Snitcowsky R, Faccioli R, Da Luz PL, Bellotti G, et al. Maternal and fetal outcome in pregnant women with Eisenmenger's syndrome. Eur Heart J. Apr 1995;16(4):460-4. [Medline].

  21. Bando K, Armitage JM, Paradis IL, et al. Indications for and results of single, bilateral, and heart-lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg. Dec 1994;108(6):1056-65. [Medline].

  22. Besterman E. Atrial Septal Defect with pulmonary hypertension. Br Heart J. 1961;23:587-598.

  23. Bethesda Conference. Congenital heart disease after childhood: an expanding patient population. 22nd Bethesda Conference, Maryland, October 18-19, 1990. J Am Coll Cardiol. Aug 1991;18(2):311-42. [Medline].

  24. Cooper JD, Patterson GA, Trulock EP. Results of single and bilateral lung transplantation in 131 consecutive recipients. Washington University Lung Transplant Group. J Thorac Cardiovasc Surg. Feb 1994;107(2):460-70; discussion 470-1. [Medline].

  25. Eisenmenger V. Die angeboren en Defecte der Kammer sheidewand des Herzen. Z Klin Med Suppl. 1897;132:1.

  26. Elkayam U. Pregnancy and cardiovascular disease. In: Braunwald E, ed. Heart Disease. 4th ed. Philadelphia, Pa: WB Saunders Co; 1992:1790-1809.

  27. Elkayam U, Cobb T, Gleicher N. Congenital heart disease in pregnancy. In: Elkayam U, Gleicher N, eds. Cardiac Problems in Pregnancy: Diagnosis and Management of Maternal and Fetal Disease. 2nd ed. New York, NY: Alan R. Liss, Inc; 1990:73-98.

  28. Gleicher N, Midwall J, Hochberger D, Jaffin H. Eisenmenger's syndrome and pregnancy. Obstet Gynecol Surv. Oct 1979;34(10):721-41. [Medline].

  29. Graham TP Jr, Bricker JT, James FW, Strong WB. 26th Bethesda conference: recommendations for determining eligibility for competition in athletes with cardiovascular abnormalities. Task Force 1: congenital heart disease. J Am Coll Cardiol. Oct 1994;24(4):867-73. [Medline].

  30. Harino S, Motokura M, Nishikawa N, Fukuda M, Sasaoka A, Grunwald JE. Chronic ocular ischemia associated with the Eisenmenger's syndrome. Am J Ophthalmol. Mar 15 1994;117(3):302-7. [Medline].

  31. Heytens L, Alexander JP. Maternal and neonatal death associated with Eisenmenger's syndrome. Acta Anaesthesiol Belg. 1986;37(1):45-51. [Medline].

  32. Hoffman JI, Rudolph AM. The natural history of ventricular septal defects in infancy. Am J Cardiol. Nov 1965;16(5):634-53. [Medline].

  33. Juaneda E, Haworth SG. Pulmonary vascular disease in children with truncus arteriosus. Am J Cardiol. Dec 1 1984;54(10):1314-20. [Medline].

  34. McManus BM, Hahn PF, Smith JA, et al. Eisenmenger ductus arteriosus with prolonged survival. Am J Cardiol. Aug 1 1984;54(3):462-4. [Medline].

  35. Paul MH, Miller RA, Potts WJ. Long-term results of aortic-pulmonary anastomosis for tetralogy of Fallot. An analysis of the first 100 cases eleven to thirteen years after operation. Circulation. Apr 1961;23:525-33. [Medline].

  36. Perloff JK, Rosove MH, Sietsema KE. Cyanotic congenital heart disease: a multisystem disorder. In: Perloff JK, Child JS, eds. Congenital Heart Disease in Adults. 2nd ed. Philadelphia, Pa: WB Saunders Co; 1998:199-225.

  37. Pitts JA, Crosby WM, Basta LL. Eisenmenger's syndrome in pregnancy: does heparin prophylaxis improve the maternal mortality rate?. Am Heart J. Mar 1977;93(3):321-6. [Medline].

  38. Rabinovich M, Moss AJ, Adams FH. Pulmonary hypertension. In: Adams FH, Emmanouilides GC, Riemenschneider TA, eds. Heart Disease in Infants, Children and Adolescents. 4th ed. Baltimore, Md: Williams & Wilkins; 1989:856-86.

  39. Reid JM, Stevenson JG, Coleman EN, et al. Moderate to severe pulmonary hypertension accompanying patent ductus arteriosus. Br Heart J. Sep 1964;26:600-5. [Medline].

  40. Reitz BA, Wallwork JL, Hunt SA, et al. Heart-lung transplantation: successful therapy for patients with pulmonary vascular disease. N Engl J Med. Mar 11 1982;306(10):557-64. [Medline].

  41. Sarris GE, Smith JA, Shumway NE, et al. Long-term results of combined heart-lung transplantation: the Stanford experience. J Heart Lung Transplant. Nov-Dec 1994;13(6):940-9. [Medline].

  42. Stoddart P, O'Sullivan G. Eisenmenger's Syndrome in Pregnancy. A case report and review. Online J Obstet Anesth[serial online]. 1993;2:159-168.

  43. Waldman JD, Paul MH, Newfeld EA, et al. Transposition of the great arteries with intact ventricular septum and patent ductus arteriosus. Am J Cardiol. Feb 1977;39(2):232-8. [Medline].

  44. 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. Jun 1998;31(7):1650-7. [Medline].

  45. Whittemore R. Congenital heart disease: its impact on pregnancy. Hosp Pract (Off Ed). Dec 1983;18(12):65-74. [Medline].

  46. Yentis SM, Steer PJ, Plaat F. Eisenmenger's syndrome in pregnancy: maternal and fetal mortality in the 1990s. Br J Obstet Gynaecol. Aug 1998;105(8):921-2. [Medline].

Further Reading

Keywords

Eisenmenger complex, Eisenmenger defect, Eisenmenger disease, Eisenmenger tetralogy, exercise intolerance, cyanosis, heart failure, hemoptysis, ventricular septal defect, VSD, overriding aorta, congenital cardiac shunt defect, pulmonary hypertension, patent ductus arteriosus, PDA, large congenital cardiac left-to-right shunts, surgically created extracardiac left-to-right shunts, increased pulmonary blood flow, transposition of the great arteries, atrial septal defect, persistent truncus arteriosus, unrestricted pulmonary blood flow, common atrioventricular canal, Blalock-Taussig anastomosis, Waterston shunt, Potts shunt, chronic cyanotic heart disease, large nonrestrictive ventricular septal defect, nonrestrictive patent ductus arteriosus

Contributor Information and Disclosures

Author

Mikhael F El-Chami, MD, Fellow, Department of Cardiology, Emory University School of Medicine
Mikhael F El-Chami, MD is a member of the following medical societies: Alpha Omega Alpha and American College of Cardiology
Disclosure: Nothing to disclose.

Coauthor(s)

Charles D Searles Jr, MD, Assistant Professor of Medicine, Division of Cardiology, Emory University School of Medicine; Consulting Staff, Division of Cardiology, Director of Stress Echo Laboratory, Grady Memorial Hospital
Charles D Searles Jr, MD is a member of the following medical societies: American Heart Association and Sigma Xi
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Marschall S Runge, MD, PhD, Charles and Anne Sanders Distinguished Professor of Medicine, Chairman of Medicine, Vice Dean for Clinical Affairs, Chairman, Department of Medicine, University of North Carolina at Chapel Hill School of Medicine
Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association
Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.