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Eisenmenger Syndrome

  • Author: Mikhael F El-Chami, MD; Chief Editor: Park W Willis IV, MD  more...
 
Updated: Nov 23, 2014
 

Background

Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis.[1, 2, 3] The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease. (See Etiology, Treatment, and Medication.)

Lesions in Eisenmenger syndrome, such as large septal defects, are characterized by high pulmonary pressure and/or a high pulmonary flow state. Development of the syndrome represents a point at which pulmonary hypertension is irreversible and is an indication that the cardiac lesion is likely inoperable (see the image below). (See Etiology, Workup, and Treatment.)

This radiograph reveals an enlarged right heart an 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.

Eisenmenger syndrome was initially described in 1897, when Victor Eisenmenger reported on a patient with symptoms of dyspnea and cyanosis from infancy who subsequently developed heart failure and succumbed to massive hemoptysis.[4] An autopsy revealed a large ventricular septal defect (VSD) and an overriding aorta. This was the first description of a link between a large congenital cardiac shunt defect and the development of pulmonary hypertension. (See Presentation and Workup.)

Advances in the medical treatment of patients with severe pulmonary hypertension may improve survival in patients with Eisenmenger syndrome and may potentially reverse the process in selected patients to a point at which they again become candidates for surgical repair. (See Treatment and Medication.)[5]

Pulmonary hypertension

Pulmonary hypertension is defined as a mean pulmonary artery pressure of more than 25 mm Hg at rest or more than 30 mm Hg during exercise. The World Health Organization (WHO) has published a classification system of various etiologies of pulmonary hypertension; the most recent update was published in 2013.[6] Eisenmenger syndrome is considered part of the group 1 causes of pulmonary hypertension, according to the Venice classification.

Intracardiac communication

An intracardiac communication allows high pulmonary artery pressures to develop and produces right-to-left intracardiac blood flow. Originally described in association with a large VSD, Eisenmenger syndrome can also manifest with a patent ductus arteriosus (PDA) or, less frequently, with other congenital cardiac anomalies. (See the images below.)

This transesophageal image is from the midesophagu This transesophageal image is from the midesophagus of a patient with Eisenmenger syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely dilated pulmonary artery. PA = pulmonary artery, Asc Ao = ascending aorta.
This computed tomography (CT) chest scan shows a l This computed tomography (CT) chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome.

Examples of congenital heart disease subtypes that may cause pulmonary vascular disease and proceed to Eisenmenger syndrome include the following:

  • Increased pulmonary arterial flow - Atrial septal defect (ASD), systemic arteriovenous fistulae, total anomalous pulmonary venous return
  • Increased pulmonary arterial pressure and flow - Large VSD, large PDA, truncus arteriosus, single ventricle with unobstructed pulmonary blood flow
  • Elevated pulmonary venous pressure - Mitral stenosis, cor triatriatum, obstructed pulmonary venous return
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Epidemiology

Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood.

Patients in underdeveloped countries are more likely to present late with uncorrected congenital cardiac lesions and a markedly elevated pulmonary vascular resistance (PVR). They are more likely to be inoperable secondary to Eisenmenger physiology.

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Etiology

Eisenmenger syndrome occurs in patients with large, congenital cardiac or surgically created extracardiac left-to-right shunts. These shunts initially cause increased pulmonary blood flow.

If left unchecked, increased pulmonary blood flow and/or elevated pulmonary arterial pressure can result in remodeling of the pulmonary microvasculature, with subsequent obstruction to pulmonary blood flow. This is commonly referred to as pulmonary vascular obstructive disease (PVOD).

According to Ohms law, flow (Q) is inversely related to resistance (R) and is directly proportional to pressure (P), as represented by the equation Q = P/R. Any increase in flow, as is observed in patients with intracardiac defects and initial left-to-right shunts, results in increased pulmonary artery pressures. Additionally, any increase in resistance, as occurs in PVOD, results in a decrease in effective flow at the same pressure.

The progression to Eisenmenger physiology is represented by a spectrum of morphologic changes in the capillary bed that progress from reversible lesions to irreversible ones. Endothelial dysfunction and smooth muscle proliferation result from the changes in flow and pressure, increasing the PVR.[7]

The cellular and molecular mechanisms remain fully uncharacterized, representing pathways of inflammation, cell proliferation, vasoconstriction, and fibrosis.[8] The mechanism of pulmonary hypertension in congenital heart disease may share characteristics with other mechanisms of pulmonary hypertension, but the pathways remain complex.

In 1958, Heath and Edwards proposed a histologic classification to describe the changes in Eisenmenger syndrome.[9] Stages I and II represent disease that is most likely reversible. Stage III disease may still be reversible, but in progressing to stages IV-VI, the disease is thought to become irreversible. Pulmonary biopsies are rarely performed today for this condition.

Natural history

Failure to reduce pulmonary pressures in the first 2 years of life may result in the failure of the normal regression of the intimal smooth muscle. This is followed by the progressive changes described by Heath and Edwards.[9] The condition then advances to irreversible pulmonary hypertension, defined as unresponsiveness to inhalation of 100% oxygen or nitric oxide. This point usually correlates to a PVR of more than 12 Woods units.

Clinically, patients gradually develop the following complications of advanced pulmonary vascular disease:

  • Dyspnea upon exertion
  • Syncope
  • Chest pain
  • Stroke
  • Brain abscess
  • Cyanosis
  • Congestive heart failure
  • Dysrhythmia
  • Hyperviscosity complications
  • Pulmonary hemorrhage/hemoptysis
  • Endocarditis

The time frame for this process depends on the anatomic nature of the lesion and whether conditions, such as trisomy 21 (Down syndrome), that are known to accelerate the development of PVOD are present.[10] Without intervention, reversal of flow may happen in early childhood or around puberty, and progression of symptoms may lead to death by the second or third decade of life.[11, 12] Interestingly, adult patients with Eisenmenger syndrome may have a better prognosis compared with those with other causes of pulmonary hypertension.[13]

Causes

Causes of Eisenmenger syndrome include the following:

  • Large, uncorrected cardiac shunt or palliative, surgically created systemic-to-pulmonary shunt for congenital heart disease
  • Large, nonrestrictive VSD
  • Nonrestrictive PDA
  • Atrioventricular septal defect, including a large ostium primum ASD without a ventricular component
  • Aortopulmonary window
  • Palliative, surgically created systemic-to-pulmonary anastomosis for treatment of congenital heart disease
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Prognosis

Eisenmenger syndrome is uniformly fatal; however, some patients survive into the sixth decade of life. The usual life expectancy of a patient with Eisenmenger syndrome is 20-50 years if the syndrome is diagnosed promptly and treated with vigilance. The onset of pulmonary hemorrhage is usually the hallmark of a rapid progression of the disease.[14]

The complications of chronic cyanotic heart disease affect multiple organ systems, including the hematologic, skeletal, renal, and neurologic systems, causing significant morbidity and mortality.

The quality of life is poor in patients with Eisenmenger syndrome because exercise tolerance is extremely limited (due to limited oxygen uptake resulting from an inability to increase pulmonary blood flow) and complications are profound. Poor prognosis is predicted by syncope, elevated-right sided pressures, and hypoxemia.

A study by Salehian et al reported that left ventricular dysfunction (defined as left ventricular ejection fraction [LVEF] < 50%), right ventricular hypertrophy, and signs and symptoms of heart failure predict mortality in patients with Eisenmenger syndrome.[15] A simple echocardiographic score relying on right ventricular and right atrial characteristics was found to predict adverse outcomes in patients with Eisenmenger syndrome that is not associated with complex congenital heart disease.[16]

Uncorrected congenital heart disease with development of Eisenmenger complex portends an insidious progression to near complete physical disability.

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Occurrence

The frequency of pulmonary hypertension and the subsequent development of reversed shunting vary depending on the specific heart defect and operative intervention. Such variations include the following:

  • Large, nonrestrictive VSD or PDA - Approximately 50% of infants with one of these defects develop pulmonary hypertension by early childhood
  • VSD or PDA and transposition of the great arteries - Forty percent of patients develop pulmonary hypertension within the first year of life
  • Large secundum ASD - The natural history of a large secundum ASD differs in that the 10% of cases that progress to pulmonary hypertension do so more slowly and usually not until after the third decade of life
  • Persistent truncus arteriosus and unrestricted pulmonary blood flow - All patients develop severe pulmonary hypertension by the second year of life
  • Common atrioventricular canal - Almost all patients develop severe pulmonary hypertension by the second year of life
  • Surgically created systemic-to-pulmonary shunt - The frequency of pulmonary hypertension varies depending on size and anatomy
  • Blalock-Taussig anastomosis (subclavian artery to pulmonary artery) - Ten percent of patients develop pulmonary hypertension
  • Waterston (ascending aorta to pulmonary artery) or Potts (descending aorta to pulmonary artery) shunt - Thirty percent of patients develop pulmonary hypertension.
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Morbidity

Complications in Eisenmenger syndrome include the following:

  • Hematologic complications - These include hyperviscosity syndromes related to secondary erythrocytosis and bleeding diatheses
  • Nervous system complications – These include brain abscess, transient cerebral ischemia, thrombotic stroke, and intracerebral hemorrhage
  • Hyperbilirubinemia - Increases the risk of gallstones
  • Hyperuricemia - Can cause nephrolithiasis and secondary gout
  • Hypertrophic osteoarthropathy - Causes bone pain and tenderness
  • Vision loss - Reports document transient vision loss related to peripheral retinal microvascular abnormalities
  • Congestive heart failure
  • Dysrhythmia
  • Pulmonary infarction and hemorrhage
  • Infective endocarditis
  • Syncope - The systemic vascular bed is prone to vasodilation and subsequent systemic arterial hypotension, which can cause syncope
  • Sudden death
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Mortality

Patients with Eisenmenger syndrome usually do not survive beyond the second or third decade. Long-term survival depends on the patient’s age at the onset of pulmonary hypertension and the coexistence of additional adverse features, such as Down syndrome. Survival predominantly depends on right ventricular function. The mortality rate in pregnant patients with Eisenmenger syndrome is reported to be approximately 50%, although it may be higher.

The most frequent terminal event in this syndrome is a combination of hypoxemia and arrhythmia in the setting of rapid increases in pulmonary vascular resistance or decreases in systemic vascular resistance (SVR). Death also commonly results from congestive heart failure, massive hemoptysis, or thromboembolism.[11]

A study by Diller et al indicated that survival rates for untreated patients with Eisenmenger syndrome may have been overestimated in previous studies and that these rates have not improved since the 1970s. The report involved a literature review of 12 studies published between 1971 and 2013 (1131 patients total), along with an analysis of 219 contemporary, treatment-naïve patients at the investigators’ own institution.[17]

The investigators stated that almost none of the studies appropriately accounted for immortal time bias and therefore overestimated patient’s survival chances by as much as 20 years. When Diller and colleagues took immortal time bias into account, they determined that the 10-year mortality rate among untreated patients approached 30-40%. They also found no indication that the chances of survival for these patients were better than they would have been in the 1970s, 1980s, or 1990s, although survival prospects were found to be better than for patients in the 1950s and 1960s.[17]

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Patient Education

The following points should be considered in patient education:

  • Inform patients that diet and weight control are essential
  • Educate patients to avoid smoking
  • Provide an exercise prescription
  • Advise abstinence from or only moderate intake of alcohol
  • Educate patients about contraception options and pregnancy risk (the mortality rate in pregnant patients with Eisenmenger syndrome is approximately 50%) [18]
  • Contraception by means of tubal ligation (with subacute bacterial endocarditis [SBE] prophylaxis) may be recommended
  • Oral or implantable contraceptives may promote pulmonary infarction through activation of the coagulation cascade
  • Educate patients about the signs and symptoms of polycythemia and hyperviscosity
  • Inform patients about the importance of dental hygiene

Additional resources for patients with pulmonary hypertension can be found at the Pulmonary Hypertension Association Web site.

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Contributor Information and Disclosures
Author

Mikhael F El-Chami, MD Assistant Professor, Department of Cardiology, Division 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, Heart Rhythm Society

Disclosure: Received grant/research funds from Medtronic Inc for principle investigator.

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, Sigma Xi

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.

Acknowledgements

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.

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

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

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

  5. Beghetti M, Galiè 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. [Medline].

  6. [Guideline] Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013 Dec 24. 62(25 Suppl):D34-41. [Medline].

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

  8. 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. [Medline].

  9. 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. [Medline].

  10. 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. [Medline].

  11. Saha A, Balakrishnan KG, Jaiswal PK, Venkitachalam CG, Tharakan J, Titus T, et al. Prognosis for patients with Eisenmenger syndrome of various aetiology. Int J Cardiol. 1994 Jul. 45(3):199-207. [Medline].

  12. 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. [Medline].

  13. 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. [Medline].

  14. 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. May 2012. 98(9):736-42.

  15. 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. 2007 May-Jun. 2(3):156-64. [Medline].

  16. Moceri P, Dimopoulos K, Liodakis E, Germanakis I, Kempny A, Diller GP, et al. Echocardiographic Predictors of Outcome in Eisenmenger Syndrome. Circulation. 2012 Aug 16. [Medline].

  17. 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 Sep. 100(17):1366-72. [Medline].

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

  19. 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. [Medline]. [Full Text].

  20. 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. [Medline]. [Full Text].

  21. 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. [Medline].

  22. 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. [Medline]. [Full Text].

  23. 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. [Medline].

  24. 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. [Medline].

  25. Dyer K, Lanning C, Das B, Lee PF, Ivy DD, Valdes-Cruz L, 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. [Medline]. [Full Text].

  26. 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. [Medline].

  27. 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. [Medline].

  28. 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. [Medline].

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

  30. 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. [Medline].

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

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

  33. Ueno T, Smith JA, Snell GI, Williams TJ, Kotsimbos TC, Rabinov M, et al. Bilateral sequential single lung transplantation for pulmonary hypertension and Eisenmenger's syndrome. Ann Thorac Surg. 2000 Feb. 69(2):381-7. [Medline].

  34. 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. [Medline].

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

  36. 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. [Medline]. [Full Text].

  37. 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. [Medline].

  38. 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. [Medline].

  39. 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. [Medline].

  40. Fernandes SM, Newburger JW, Lang P, Pearson DD, Feinstein JA, Gauvreau K, 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. [Medline].

  41. 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. [Medline]. [Full Text].

  42. 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. [Medline]. [Full Text].

  43. Ivy DD, Doran AK, Parker DK, et al. Acute and Chronic Effects of Inhaled Iloprost Therapy in Children with Pulmonary Arterial Hypertension. Chest. 2006. 130 (4) Meeting abstracts:156S.

  44. 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. [Medline]. [Full Text].

  45. 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. [Medline].

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

  47. 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. [Medline].

  48. 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. [Medline].

  49. 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. [Medline].

  50. 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. [Medline].

  51. 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 Jul 4. 114(1):48-54. [Medline].

  52. 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. 2008 Jun 23. 127(1):27-32. [Medline].

  53. 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. [Medline].

  54. 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. [Medline].

  55. 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. [Medline].

  56. 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. [Medline].

  57. 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. [Medline].

  58. 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. [Medline].

  59. 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. [Medline].

  60. 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. [Medline].

  61. 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. [Medline].

  62. [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. [Medline].

  63. [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. [Medline].

  64. 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. [Medline].

  65. 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. [Medline].

  66. 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. [Medline].

  67. 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. [Medline].

 
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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.
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 computed tomography (CT) chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome.
This apical, 4-chamber, transthoracic 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 midesophagus of a patient with Eisenmenger syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely dilated pulmonary artery. PA = pulmonary artery, Asc Ao = ascending aorta.
This is a color Doppler interrogation of the tricuspid valve in a patient with 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 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.
 
 
 
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