Eisenmenger Syndrome Differential Diagnoses

1. Wood P. The Eisenmenger syndrome or pulmonary hypertension with reversed central shunt. Br Med J. 1958 Sep 27. 2(5099):755-62. [QxMD MEDLINE Link]. [Full Text].

2. Vongpatanasin W, Brickner ME, Hillis LD, Lange RA. The Eisenmenger syndrome in adults. Ann Intern Med. 1998 May 1. 128(9):745-55. [QxMD MEDLINE Link].

3. Diller GP, Gatzoulis MA. Pulmonary vascular disease in adults with congenital heart disease. Circulation. 2007 Feb 27. 115(8):1039-50. [QxMD MEDLINE Link].

4. Eisenmenger V. Die angeborenen Defecte der Kammerscheidewand des Herzens. Z Klin Med. 1897. 32 suppl:1-28.

5. Beghetti M, Galie N. Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J Am Coll Cardiol. 2009 Mar 3. 53(9):733-40. [QxMD MEDLINE Link].

6. [Guideline] Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24. 62(25 suppl):D34-41. [QxMD MEDLINE Link].

7. Celermajer DS, Cullen S, Deanfield JE. Impairment of endothelium-dependent pulmonary artery relaxation in children with congenital heart disease and abnormal pulmonary hemodynamics. Circulation. 1993 Feb. 87(2):440-6. [QxMD MEDLINE Link]. [Full Text].

8. Yoshibayashi M, Nishioka K, Nakao K, et al. Plasma endothelin concentrations in patients with pulmonary hypertension associated with congenital heart defects. Evidence for increased production of endothelin in pulmonary circulation. Circulation. 1991 Dec. 84(6):2280-5. [QxMD MEDLINE Link]. [Full Text].

9. Hassoun PM, Mouthon L, Barbera JA, et al. Inflammation, growth factors, and pulmonary vascular remodeling. J Am Coll Cardiol. 2009 Jun 30. 54(1 suppl):S10-9. [QxMD MEDLINE Link].

10. Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004 Sep 30. 351(14):1425-36. [QxMD MEDLINE Link].

11. Humbert M, Morrell NW, Archer SL, et al. Cellular and molecular pathobiology of pulmonary arterial hypertension. J Am Coll Cardiol. 2004 Jun 16. 43(12 suppl S):13S-24S. [QxMD MEDLINE Link].

12. 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 Oct. 18(4 part 1):533-47. [QxMD MEDLINE Link].

13. Kidd L, Driscoll DJ, Gersony WM, et al. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects. Circulation. 1993 Feb. 87(2 suppl):I38-51. [QxMD MEDLINE Link].

14. Onat T, Ahunbay G, Batmaz G, Celebi A. The natural course of isolated ventricular septal defect during adolescence. Pediatr Cardiol. 1998 May-Jun. 19(3):230-4. [QxMD MEDLINE Link].

15. Saha A, Balakrishnan KG, Jaiswal PK, et al. Prognosis for patients with Eisenmenger syndrome of various aetiology. Int J Cardiol. 1994 Jul. 45(3):199-207. [QxMD MEDLINE Link].

16. 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 Jan. 15(1 pt 1):100-5. [QxMD MEDLINE Link].

17. Daliento L, Somerville J, Presbitero P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J. 1998 Dec. 19(12):1845-55. [QxMD MEDLINE Link].

18. Mebus S, Schulze-Neick I, Oechslin E, et al. The adult patient with Eisenmenger syndrome: a medical update after Dana Point part II: medical treatment - study results. Curr Cardiol Rev. 2010 Nov. 6(4):356-62. [QxMD MEDLINE Link]. [Full Text].

19. Diller GP, Alonso-Gonzalez R, Kempny A, et al. B-type natriuretic peptide concentrations in contemporary Eisenmenger syndrome patients: predictive value and response to disease targeting therapy. Heart. 2012 May. 98(9):736-42. [QxMD MEDLINE Link].

20. 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 Sep. 100(17):1366-72. [QxMD MEDLINE Link].

21. Diller GP, Korten MA, Bauer UMM, et al, for the German Competence Network for Congenital Heart Defects Investigators. Current therapy and outcome of Eisenmenger syndrome: data of the German National Register for congenital heart defects. Eur Heart J. 2016 May 7. 37(18):1449-55. [QxMD MEDLINE Link]. [Full Text].

22. Salehian O, Schwerzmann M, Rambihar S, et al. Left ventricular dysfunction and mortality in adult patients with Eisenmenger syndrome. Congenit Heart Dis. 2007 May-Jun. 2(3):156-64. [QxMD MEDLINE Link].

23. Moceri P, Dimopoulos K, Liodakis E, et al. Echocardiographic predictors of outcome in eisenmenger syndrome. Circulation. 2012 Sep 18. 126(12):1461-8. [QxMD MEDLINE Link].

24. Attina D, Niro F, Garzillo G, et al. Thoracic manifestation of Eisenmenger's syndrome in adult patients: a MDCT review. Lung. 2015 Apr. 193(2):173-81. [QxMD MEDLINE Link].

25. Weiss BM, Hess OM. Analysis of pulmonary vascular disease in pregnant women. J Am Coll Cardiol. 1999 Nov 1. 34(5):1658. [QxMD MEDLINE Link].

26. Oechslin E, Mebus S, Schulze-Neick I, et al. The adult patient with Eisenmenger syndrome: a medical update after Dana Point part III: specific management and surgical aspects. Curr Cardiol Rev. 2010 Nov. 6(4):363-72. [QxMD MEDLINE Link]. [Full Text].

27. Kaemmerer H, Mebus S, Schulze-Neick I, et al. The adult patient with Eisenmenger syndrome: a medical update after Dana point part I: epidemiology, clinical aspects and diagnostic options. Curr Cardiol Rev. 2010 Nov. 6(4):343-55. [QxMD MEDLINE Link]. [Full Text].

28. Cantor WJ, Harrison DA, Moussadji JS, et al. Determinants of survival and length of survival in adults with Eisenmenger syndrome. Am J Cardiol. 1999 Sep 15. 84(6):677-81. [QxMD MEDLINE Link].

29. [Guideline] Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults with Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019 Apr 2. 73(12):e81-e192. [QxMD MEDLINE Link]. [Full Text].

30. Balzer DT, Kort HW, Day RW, et al. Inhaled nitric oxide as a preoperative test (INOP test I): the INOP Test Study Group. Circulation. 2002 Sep 24. 106(12 suppl 1):I76-81. [QxMD MEDLINE Link].

31. Bernus A, Wagner BD, Accurso F, Doran A, Kaess H, Ivy DD. Brain natriuretic peptide levels in managing pediatric patients with pulmonary arterial hypertension. Chest. 2009 Mar. 135(3):745-51. [QxMD MEDLINE Link]. [Full Text].

32. Babu-Narayan SV, Gatzoulis MA, Kilner PJ. Non-invasive imaging in adult congenital heart disease using cardiovascular magnetic resonance. J Cardiovasc Med (Hagerstown). 2007 Jan. 8(1):23-9. [QxMD MEDLINE Link].

33. Stojnic B, Pavlovic P, Ponomarev D, Aleksandrov R, Prcovic M. Bidirectional shunt flow across a ventricular septal defect: pulsed Doppler echocardiographic analysis. Pediatr Cardiol. 1995 Jan-Feb. 16(1):6-11. [QxMD MEDLINE Link].

34. Yock PG, Popp RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation. 1984 Oct. 70(4):657-62. [QxMD MEDLINE Link].

35. Dyer K, Lanning C, Das B, et al. Noninvasive Doppler tissue measurement of pulmonary artery compliance in children with pulmonary hypertension. J Am Soc Echocardiogr. 2006 Apr. 19(4):403-12. [QxMD MEDLINE Link]. [Full Text].

36. Lang RM, Bierig M, Devereux RB, et al, for the American Society of Echocardiography's Nomenclature and Standards Committee, Task Force on Chamber Quantification, et al. Recommendations for chamber quantification. Eur J Echocardiogr. 2006 Mar. 7(2):79-108. [QxMD MEDLINE Link].

37. Lammers AE, Diller GP, Odendaal D, Tailor S, Derrick G, Haworth SG. Comparison of 6-min walk test distance and cardiopulmonary exercise test performance in children with pulmonary hypertension. Arch Dis Child. 2011 Feb. 96(2):141-7. [QxMD MEDLINE Link].

38. Mocellin R. [Sports fitness in pulmonary hypertension] [German]. Wien Klin Wochenschr. 1986 Nov 7. 98(21):735-9. [QxMD MEDLINE Link].

39. Ikawa S, Shimazaki Y, Nakano S, Kobayashi J, Matsuda H, Kawashima Y. Pulmonary vascular resistance during exercise late after repair of large ventricular septal defects. Relation to age at the time of repair. J Thorac Cardiovasc Surg. 1995 Jun. 109 (6):1218-24. [QxMD MEDLINE Link].

40. Hascoet S, Baruteau A, Humbert M. 0425: Prognostic value of invasive hemodynamic parameters in Eisenmenger syndrome [abstract]. Arch Cardiovasc Dis Suppl. Jan 2016. 8(1):101-2. [Full Text].

41. [Guideline] Badesch DB, Abman SH, Simonneau G, Rubin LJ, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: updated ACCP evidence-based clinical practice guidelines. Chest. 2007 Jun. 131(6):1917-28. [QxMD MEDLINE Link].

42. Maron BA, Galie N. Diagnosis, treatment, and clinical management of pulmonary arterial hypertension in the contemporary era: a review. JAMA Cardiol. 2016 Dec 1. 1(9):1056-65. [QxMD MEDLINE Link].

43. Matsubara H, Ogawa A. Treatment of idiopathic/hereditary pulmonary arterial hypertension. J Cardiol. 2014 Oct. 64(4):243-9. [QxMD MEDLINE Link].

44. Kelion AD, Webb TP, Gardner MA, Ormerod OJ, Banning AP. The warm-up effect protects against ischemic left ventricular dysfunction in patients with angina. J Am Coll Cardiol. 2001 Mar 1. 37(3):705-10. [QxMD MEDLINE Link]. [Full Text].

45. [Guideline] 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 Dec 2. 118(23):e714-833. [QxMD MEDLINE Link]. [Full Text].

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

47. Castaneda AR, Jonas RA, Mayer JE, Hanley FL, eds. Surgery for infants with congenital heart defects. Cardiac Surgery of the Neonate and Infant. Philadelphia, PA: WB Saunders Co; 1994.

48. Esmore DS, Brown R, Buckland M, et al. Techniques and results in bilateral sequential single lung transplantation. The National Heart & Lung Replacement Service. J Card Surg. 1994 Jan. 9(1):1-14. [QxMD MEDLINE Link].

49. Noyes BE, Kurland G, Orenstein DM, Fricker FJ, Armitage JM. Experience with pediatric lung transplantation. J Pediatr. 1994 Feb. 124(2):261-8. [QxMD MEDLINE Link].

50. Noyes BE, Kurland G, Orenstein DM. Lung and heart-lung transplantation in children. Pediatr Pulmonol. 1997 Jan. 23(1):39-48. [QxMD MEDLINE Link].

51. Ueno T, Smith JA, Snell GI, et al. Bilateral sequential single lung transplantation for pulmonary hypertension and Eisenmenger's syndrome. Ann Thorac Surg. 2000 Feb. 69(2):381-7. [QxMD MEDLINE Link].

52. Das BB, Wolfe RR, Chan KC, Larsen GL, Reeves JT, Ivy D. High-altitude pulmonary edema in children with underlying cardiopulmonary disorders and pulmonary hypertension living at altitude. Arch Pediatr Adolesc Med. 2004 Dec. 158(12):1170-6. [QxMD MEDLINE Link].

53. Broberg CS, Uebing A, Cuomo L, et al. Adult patients with Eisenmenger syndrome report flying safely on commercial airlines. Heart. 2007 Dec. 93(12):1599-603. [QxMD MEDLINE Link]. [Full Text].

54. 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. 1986 Apr. 55(4):385-90. [QxMD MEDLINE Link]. [Full Text].

55. Sandoval J, Aguirre JS, Pulido T, et al. Nocturnal oxygen therapy in patients with the Eisenmenger syndrome. Am J Respir Crit Care Med. 2001 Nov 1. 164(9):1682-7. [QxMD MEDLINE Link].

56. Gonzaga LR, Matos-Garcia BC, Rocco IS, et al. Effects of acute oxygen supplementation on functional capacity and heart rate recovery in Eisenmenger syndrome. Int J Cardiol. 2017 Mar 15. 231:110-4. [QxMD MEDLINE Link].

57. Harinck E, Hutter PA, Hoorntje TM, et al. Air travel and adults with cyanotic congenital heart disease. Circulation. 1996 Jan 15. 93(2):272-6. [QxMD MEDLINE Link].

58. Hascoet S, Baruteau AE, Humbert M, et al. Long-term outcomes of pulmonary arterial hypertension under specific drug therapy in Eisenmenger syndrome. J Heart Lung Transplant. 2017 Apr. 36(4):386-98. [QxMD MEDLINE Link].

59. Lange RA, Brickner ME. Improving survival in patients with Eisenmenger syndrome: are we any closer?. Circulation. 2017 Apr 11. 135(15):1441-3. [QxMD MEDLINE Link]. [Full Text].

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

61. 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 Mar 1. 91(5):632-5. [QxMD MEDLINE Link].

62. Ivy DD, Doran A, Claussen L, Bingaman D, Yetman A. Weaning and discontinuation of epoprostenol in children with idiopathic pulmonary arterial hypertension receiving concomitant bosentan. Am J Cardiol. 2004 Apr 1. 93(7):943-6. [QxMD MEDLINE Link]. [Full Text].

63. El Yafawi R, Wirth JA. What is the role of oral prostacyclin pathway medications in pulmonary arterial hypertension management?. Curr Hypertens Rep. 2017 Oct 25. 19(12):97. [QxMD MEDLINE Link].

64. Feldman J, Habib N, Radosevich J, Dutt M. Oral treprostinil in the treatment of pulmonary arterial hypertension. Expert Opin Pharmacother. 2017 Oct. 18(15):1661-7. [QxMD MEDLINE Link].

65. Ivy DD, Claussen L, Doran A. Transition of stable pediatric patients with pulmonary arterial hypertension from intravenous epoprostenol to intravenous treprostinil. Am J Cardiol. 2007 Mar 1. 99(5):696-8. [QxMD MEDLINE Link]. [Full Text].

66. Carpentier E, Mur S, Aubry E, et al. Safety and tolerability of subcutaneous treprostinil in newborns with congenital diaphragmatic hernia and life-threatening pulmonary hypertension. J Pediatr Surg. 2017 Sep. 52(9):1480-3. [QxMD MEDLINE Link].

67. Ivy DD, Doran AK, Parker DK, et al. Acute and chronic effects of inhaled iloprost therapy in children with pulmonary arterial hypertension [abstract]. Chest. Oct 2006. 130(4 suppl):156S. [Full Text].

68. Ivy DD, Doran AK, Smith KJ, et al. Short- and long-term effects of inhaled iloprost therapy in children with pulmonary arterial hypertension. J Am Coll Cardiol. 2008 Jan 15. 51(2):161-9. [QxMD MEDLINE Link]. [Full Text].

69. Barst RJ, Ivy D, Dingemanse J, et al. Pharmacokinetics, safety, and efficacy of bosentan in pediatric patients with pulmonary arterial hypertension. Clin Pharmacol Ther. 2003 Apr. 73(4):372-82. [QxMD MEDLINE Link].

70. Maiya S, Hislop AA, Flynn Y, Haworth SG. Response to bosentan in children with pulmonary hypertension. Heart. 2006 May. 92(5):664-70. [QxMD MEDLINE Link]. [Full Text].

71. Rosenzweig EB, Ivy DD, Widlitz A, et al. Effects of long-term bosentan in children with pulmonary arterial hypertension. J Am Coll Cardiol. 2005 Aug 16. 46(4):697-704. [QxMD MEDLINE Link].

72. Kaya MG, Lam YY, Erer B, et al. Long-term effect of bosentan therapy on cardiac function and symptomatic benefits in adult patients with Eisenmenger syndrome. J Card Fail. 2012 May. 18(5):379-84. [QxMD MEDLINE Link].

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

74. Schulze-Neick I, Gilbert N, Ewert R, et al. Adult patients with congenital heart disease and pulmonary arterial hypertension: first open prospective multicenter study of bosentan therapy. Am Heart J. 2005 Oct. 150(4):716. [QxMD MEDLINE Link].

75. Galie N, Beghetti M, Gatzoulis MA, et al, for the Bosentan Randomized Trial of Endothelin Antagonist Therapy-5 (BREATHE-5) Investigators. Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation. 2006 Jul 4. 114 (1):48-54. [QxMD MEDLINE Link].

76. Gatzoulis MA, Beghetti M, Galie N, et al, for the BREATHE-5 Investigators. Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study. Int J Cardiol. 2008 Jun 23. 127(1):27-32. [QxMD MEDLINE Link].

77. Elshafay A, Truong DH, AboElnas MM, et al. The effect of endothelin receptor antagonists in patients with Eisenmenger syndrome: a systematic review. Am J Cardiovasc Drugs. 2018 Apr. 18(2):93-102. [QxMD MEDLINE Link].

78. Ereminiene E, Kinderyte M, Miliauskas S. Impact of advanced medical therapy for the outcome of an adult patient with Eisenmenger syndrome. Respir Med Case Rep. 2017. 21:16-20. [QxMD MEDLINE Link].

79. 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 Jun. 27(12):1472-7. [QxMD MEDLINE Link].

80. Arnott C, Strange G, Bullock A, et al. Pulmonary vasodilator therapy is associated with greater survival in Eisenmenger syndrome. Heart. 2017 Aug 9. 104:732-7. [QxMD MEDLINE Link].

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

82. Humpl T, Reyes JT, Holtby H, Stephens D, Adatia I. Beneficial effect of oral sildenafil therapy on childhood pulmonary arterial hypertension: twelve-month clinical trial of a single-drug, open-label, pilot study. Circulation. 2005 Jun 21. 111(24):3274-80. [QxMD MEDLINE Link].

83. Raja SG, Danton MD, MacArthur KJ, Pollock JC. Effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension and congenital cardiac defects. J Cardiothorac Vasc Anesth. 2007 Apr. 21(2):203-7. [QxMD MEDLINE Link].

84. 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 Apr. 151(4):851.e1-5. [QxMD MEDLINE Link].

85. Mukhopadhyay S, Sharma M, Ramakrishnan S, et al. Phosphodiesterase-5 inhibitor in Eisenmenger syndrome: a preliminary observational study. Circulation. 2006 Oct 24. 114(17):1807-10. [QxMD MEDLINE Link].

86. Ivy DD, Griebel JL, Kinsella JP, Abman SH. Acute hemodynamic effects of pulsed delivery of low flow nasal nitric oxide in children with pulmonary hypertension. J Pediatr. 1998 Sep. 133 (3):453-6. [QxMD MEDLINE Link].

87. Ivy DD, Parker D, Doran A, Parker D, Kinsella JP, Abman SH. Acute hemodynamic effects and home therapy using a novel pulsed nasal nitric oxide delivery system in children and young adults with pulmonary hypertension. Am J Cardiol. 2003 Oct 1. 92(7):886-90. [QxMD MEDLINE Link].

88. Kinsella JP, Parker TA, Ivy DD, Abman SH. Noninvasive delivery of inhaled nitric oxide therapy for late pulmonary hypertension in newborn infants with congenital diaphragmatic hernia. J Pediatr. 2003 Apr. 142(4):397-401. [QxMD MEDLINE Link].

89. US Food and Drug Administration. FDA approves new orphan drug to treat pulmonary arterial hypertension [press release]. Available at https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm478599.htm. December 22, 2015; Accessed: November 20, 2017.

90. Honorato Perez J. Selexipag, a selective prostacyclin receptor agonist in pulmonary arterial hypertension: a pharmacology review. Expert Rev Clin Pharmacol. 2017 Jul. 10(7):753-62. [QxMD MEDLINE Link].

91. Sorensen LM, Wehland M, Kruger M, et al. A special focus on selexipag - treatment of pulmonary arterial hypertension. Curr Pharm Des. 2017. 23(34):5191-9. [QxMD MEDLINE Link].

92. Gallotti R, Drogalis-Kim DE, Satou G, Alejos J. Single-center experience using selexipag in a pediatric population. Pediatr Cardiol. 2017 Oct. 38(7):1405-9. [QxMD MEDLINE Link].

93. [Guideline] Wilson W, Taubert KA, Gewitz M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Circulation. 2007 Oct 9. 116(15):1736-54. [QxMD MEDLINE Link]. [Full Text].

94. [Guideline] Nishimura RA, Carabello BA, Faxon DP, et al. ACC/AHA 2008 guideline update on valvular heart disease: focused update on infective endocarditis: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines: endorsed by the Society of Cardiovascular Anesthesiologists, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. Circulation. 2008 Aug 19. 118(8):887-96. [QxMD MEDLINE Link]. [Full Text].

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

96. [Guideline] American Heart Association. Infective endocarditis. Available at https://www.heart.org/HEARTORG/Conditions/CongenitalHeartDefects/TheImpactofCongenitalHeartDefects/Infective-Endocarditis_UCM_307108_Article.jsp#.WhM5tVtSyHs. Updated: September 29, 2017; Accessed: November 20, 2017.

97. Linderkamp O, Klose HJ, Betke K, et al. Increased blood viscosity in patients with cyanotic congenital heart disease and iron deficiency. J Pediatr. 1979 Oct. 95(4):567-9. [QxMD MEDLINE Link].

98. Van De Bruaene A, Delcroix M, Pasquet A, et al. Iron deficiency is associated with adverse outcome in Eisenmenger patients. Eur Heart J. 2011 Nov. 32(22):2790-9. [QxMD MEDLINE Link].

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

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

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

102. Avila WS, Grinberg M, Snitcowsky R, et al. Maternal and fetal outcome in pregnant women with Eisenmenger's syndrome. Eur Heart J. 1995 Apr. 16(4):460-4. [QxMD MEDLINE Link].

103. Benoit L, Nizard J, Radojevic J, et al. Pregnancy outcomes in Eisenmenger syndrome: a French multicentric cohort study [abstract]. Eur J Obstet Gynecol Reprod Biol. Nov 2016. 206:e135-6. [Full Text].

104. Rosenzweig EB, Abrams D, Biscotti M, et al. Eisenmenger syndrome and pregnancy: novel ECMO configuration as a bridge to delivery and recovery utilizing a multidisciplinary team. ASAIO J. 2018 Jan/Feb. 64(1):e8-e10. [QxMD MEDLINE Link].

105. Berman EB, Barst RJ. Eisenmenger's syndrome: current management. Prog Cardiovasc Dis. 2002 Sep-Oct. 45 (2):129-38. [QxMD MEDLINE Link].

106. Stoica SC, McNeil KD, Perreas K, et al. Heart-lung transplantation for Eisenmenger syndrome: early and long-term results. Ann Thorac Surg. 2001 Dec. 72(6):1887-91. [QxMD MEDLINE Link].

107. Waddell TK, Bennett L, Kennedy R, Todd TR, Keshavjee SH. Heart-lung or lung transplantation for Eisenmenger syndrome. J Heart Lung Transplant. 2002 Jul. 21(7):731-7. [QxMD MEDLINE Link].

108. Nagy E, Edwards LB, Kucheryavaya AY, Stehlik J, Yusen RD, Lund LH. Orthotopic heart-lung transplantation: outcomes and predictors of outcomes in recipients with Eisenmenger syndrome, other congenital etiologies, and idiopathic pulmonary arterial hypertension [abstract]. J Heart Lung Transpl. Apr 2017. 36(4) suppl:S21-2. [Full Text].

109. Moceri P, Kempny A, Liodakis E, et al. Physiological differences between various types of Eisenmenger syndrome and relation to outcome. Int J Cardiol. 2015 Jan 20. 179:455-60. [QxMD MEDLINE Link].

Jorge L Penalver, MD Resident Physician, Department of Internal Medicine, Einstein Medical Center

Disclosure: Nothing to disclose.

Aman M Amanullah, MD, PhD, FACC, FASE, FASNC, FAHA Section Chief of Non-Invasive Cardiology, Division of Cardiology, Department of Medicine, Albert Einstein Medical Center; Clinical Professor of Medicine, Jefferson Medical College of Thomas Jefferson University

Aman M Amanullah, MD, PhD, FACC, FASE, FASNC, FAHA is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, American Society of Nuclear Cardiology, North American Society for Cardiovascular Imaging, Swedish Society of Medicine, Society of Cardiovascular Computed Tomography

Disclosure: Nothing to disclose.

Yasmine S Ali, MD, MSCI, FACC, FACP Assistant Clinical Professor of Medicine, Vanderbilt University School of Medicine; President, LastSky Writing, LLC

Yasmine S Ali, MD, MSCI, FACC, FACP is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, American Medical Association, American Medical Writers Association, National Lipid Association, Tennessee Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: LastSky Writing;Philips Healthcare;M Health;Athena Health;PeerView Institute;Verywell Health; HealthCentral;MedEdicus;Pharmaceutical Training Institute;Elsevier;MedStudy;North American Thrombosis Forum;WellnessVerge;Prose Media; AKH, Inc.<br/>Serve(d) as a speaker or a member of a speakers bureau for: North American Thrombosis Forum.

Mikhael F El-Chami, MD Associate Professor, Department of Medicine, Division of Cardiology, Section of Electrophysiology, Emory University School of Medicine

Mikhael F El-Chami, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Cardiology, American Heart Association, Heart Rhythm Society

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Medtronic; Boston Scientific, Biotronik<br/>Received research grants (as part of Multicenter studies) from Medtronic and Boston Scientific.

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, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Stuart Berger, MD Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin

Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose

Brian M Cummings, MD Pediatric Critical Care; Director Pediatric Transport, Medical Director PALS, MassGeneral Hospital for Children, Instructor in Pediatrics, Harvard Medical School

Brian M Cummings, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Elyse Foster, MD Director of Adult Echocardiography Laboratory and Adult Congenital Heart Disease Service, Department of Internal Medicine, Division of Cardiology, Moffitt Hospital; Assistant Professor of Cardiology, University of California, San Francisco, School of Medicine

Elyse Foster, MD is a member of the following medical societies American College of Cardiology, American College of Physicians, American Heart Association, and American Society of Echocardiography

Disclosure: Nothing to disclose.

Lisa A Hourigan, MBBS, FRACP Consulting Staff, Department of Cardiology, University of California, San Francisco School of Medicine

Disclosure: Nothing to disclose.

Christopher Johnsrude, MD, MS Chief, Division of Pediatric Cardiology, University of Louisville School of Medicine; Director, Congenital Heart Center, Kosair Children's Hospital

Christopher Johnsrude, MD, MS is a member of the following medical societies: American Academy of Pediatrics and American College of Cardiology

Disclosure: St Jude Medical Honoraria Speaking and teaching

John W Moore, MD, MPH Professor of Clinical Pediatrics, Section of Pediatric Cardiology, Department of Pediatrics, University of California San Diego School of Medicine; Director of Cardiology, Rady Children's Hospital

John W Moore, MD, MPH is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Jeff L Myers, MD, PhD Chief, Pediatric and Congenital Cardiac Surgery, Department of Surgery, Massachusetts General Hospital; Associate Professor of Surgery, Harvard Medical School

Jeff L Myers, MD, PhD is a member of the following medical societies: American College of Surgeons, American Heart Association, and International Society for Heart and Lung Transplantation

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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