Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Eisenmenger Syndrome Medication

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

Medication Summary

The medical treatment of Eisenmenger syndrome is directed toward the improvement of symptoms related to heart failure and pulmonary hypertension and the prevention and management of complications related to cyanotic congenital heart disease.

A partial list of medications used in the management of Eisenmenger syndrome includes aspirin, to prevent thrombotic complications; allopurinol, for gout; iron supplementation, for microcytosis; and digitalis and diuretics, for symptoms of heart failure.

Next

Diuretics

Class Summary

These agents are useful to remove fluid and reduce preload and afterload in the treatment of heart failure.

Furosemide (Lasix)

 

Furosemide increases the excretion of water by interfering with the chloride-binding cotransport system; this, in turn, inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule.

The dose of furosemide must be individualized to the patient. Depending on response, administer the medication at increments of 20-40 mg, with each dose provided no sooner than 6-8 hours after the previous dose, until the desired diuresis occurs. When treating infants, titrate with 1-mg/kg/dose increments until a satisfactory effect is achieved.

Previous
Next

Cardiac Glycosides

Class Summary

The positive inotropic and negative chronotropic effects of these agents are useful in the setting of left or right heart failure. Cardiac glycosides are used to enhance cardiac contractility as an adjunct to treating congestive heart failure. They are used to augment the function of the failing right ventricle.

Digoxin (Lanoxin)

 

Digoxin is a cardiac glycoside with direct inotropic effects, as well as indirect effects, on the cardiovascular system. It acts directly on cardiac muscle, increasing myocardial systolic contractions. Its indirect actions result in increased carotid sinus nerve activity and enhanced sympathetic withdrawal for any given increase in mean arterial pressure.

Previous
Next

Prostaglandins

Class Summary

These drugs can be effective in reversing reactive pulmonary vasoconstriction and can, therefore, lower pulmonary vascular resistance, decrease afterload, reduce the right ventricle, and reduce right-to-left shunting. In some patients, chronic prostacyclin analogue therapy (epoprostenol) can be of benefit, particularly as a bridge to heart-lung transplantation.

Epoprostenol (Flolan, Veletri)

 

Epoprostenol is a strong vasodilator of all vascular beds. It may decrease thrombogenesis and platelet clumping in the lungs by inhibiting platelet aggregation.

Continuous intravenous infusion of the drug can be carried out via a permanent, indwelling central venous catheter, using a small, battery-powered infusion pump worn at the hip or carried in a backpack.

Initiate administration of epoprostenol under close observation in the intensive care unit (ICU), with a right heart flotation catheter in place

Iloprost (Ventavis)

 

Iloprost is a synthetic analogue of prostacyclin PGI2 that dilates systemic and pulmonary arterial vascular beds. It is indicated for pulmonary arterial hypertension (WHO Class I) in patients with NYHA Class III or IV symptoms to improve exercise tolerance and symptoms and to delay deterioration.

Previous
Next

Iron Products

Class Summary

Recurrent phlebotomy for erythrocytosis can lead to microcytic anemia. Iron stores should be replaced if the deficiency is symptomatic and the hematocrit is below 65%.

Ferrous sulfate (Feosol, Fer-iron)

 

Iron is a nutritionally essential inorganic substance.

Previous
Next

Rheumatologics, Other

Class Summary

These agents are indicated for symptomatic secondary gout.

Colchicine

 

Colchicine decreases leukocyte motility and phagocytosis in inflammatory responses.

Previous
Next

Endothelin Antagonists

Class Summary

These agents competitively bind to endothelin-1 (ET-1) receptors ETA and ETB in endothelium and vascular smooth muscle, inhibiting vessel constriction and elevation of blood pressure.

Bosentan (Tracleer)

 

Bosentan is an endothelin receptor antagonist indicated for the treatment of pulmonary arterial hypertension (PAH) in patients with WHO class III or IV symptoms. It is used to improve exercise ability and decrease the rate of clinical worsening.

Bosentan inhibits vessel constriction and elevation of blood pressure by competitively binding to ET-1 receptors ETA and ETB in the endothelium and vascular smooth muscle. This leads to a significant increase in the cardiac index associated with a significant reduction in pulmonary artery pressure, pulmonary vascular resistance (PVR), and mean right atrial pressure. Due to its teratogenic potential, bosentan can be prescribed only through the Tracleer Access Program (1-866-228-3546).

Ambrisentan (Letairis)

 

Ambrisentan is an endothelin receptor antagonist indicated for PAH in patients with WHO class II or III symptoms. It improves exercise ability and decreases the progression of clinical symptoms.

Ambrisentan inhibits vessel constriction and blood pressure elevation by competitively binding to endothelin-1 receptors ETA and ETB in the endothelium and vascular smooth muscle. This leads to a significant increase in the cardiac index associated with a significant reduction in pulmonary artery pressure, PVR, and mean right atrial pressure.

Because of the risks of hepatic injury and the drug's teratogenic potential, ambrisentan is available only through the Letairis Education and Access Program (LEAP). Prescribers and pharmacies must register with LEAP in order to prescribe and dispense this drug. For more information, see http://www.letairis.com or call (866) 664-LEAP (5327).

Previous
Next

Phosphodiesterase-5 Enzyme Inhibitors

Class Summary

The antiproliferative effects of the phosphodiesterase type-5 (PDE5) pathway, which regulates cyclic guanosine monophosphate hydrolysis, may be significant in the chronic treatment of pulmonary hypertension with PDE5 inhibitors such as sildenafil. These agents act synergistically with nitric oxide to promote smooth muscle relaxation.

Sildenafil (Revatio)

 

Sildenafil promotes selective smooth muscle relaxation in the lung vasculature, possibly by inhibiting PDE5. The inhibition of PDE5 increases cyclic guanosine monophosphate (cGMP) activity, which increases the vasodilatory effects of nitric oxide. Nitric oxide is a powerful, naturally produced vasodilator used clinically as an inhaled agent.

Tadalafil (Adcirca)

 

Tadalafil is a PDE5 selective inhibitor. Inhibition of PDE5 increases cGMP activity, which increases the vasodilatory effects of nitric oxide. Tadalafil promotes selective smooth muscle relaxation in the lung vasculature, possibly by inhibiting PDE5.

Previous
 
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].

 
Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.