Eisenmenger Syndrome Medication

  • Author: Mikhael F El-Chami, MD; Chief Editor: Park W Willis IV, MD   more...
 
Updated: Dec 12, 2011
 

Medication Summary

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

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Diuretics

Class Summary

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

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Furosemide (Lasix)

 

Increases excretion of water by interfering with chloride-binding cotransport system, which in turn results inhibits sodium and chloride reabsorption in the ascending loop of Henle and distal renal tubule.

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

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Cardiac Glycosides

Class Summary

Positive inotropic and negative chronotropic effects are useful in the setting of left or right heart failure.

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Digoxin (Lanoxin, Lanoxicaps)

 

Cardiac glycoside with direct inotropic effects in addition to indirect effects on the cardiovascular system.

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.

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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.

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Epoprostenol (Flolan)

 

Strong vasodilator of all vascular beds. May decrease thrombogenesis and platelet clumping in the lungs by inhibiting platelet aggregation.

Continuous chronic infusion should be administered through a central venous catheter.

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Vitamins

Class Summary

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

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Iron sulfate (Feosol, Ferrous sulfate)

 

A nutritionally essential inorganic substance.

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Anti-inflammatory Medications

Class Summary

Indicated for symptomatic secondary gout.

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Colchicine

 

Decreases leukocyte motility and phagocytosis in inflammatory responses.

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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.

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Bosentan (Tracleer)

 

Endothelin receptor antagonist indicated for the treatment of PAH in patients with WHO class III or IV symptoms to improve exercise ability and decrease rate of clinical worsening. Inhibits vessel constriction and elevation of blood pressure by competitively binding to ET-1 receptors EtA and EtB in endothelium and vascular smooth muscle. This leads to significant increase in cardiac index (CI) associated with significant reduction in pulmonary artery pressure, PVR, and mean right atrial pressure. Due to teratogenic potential, can only be prescribed through the Tracleer Access Program (1-866-228-3546).

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Ambrisentan (Letairis)

 

Endothelin receptor antagonist indicated for pulmonary arterial hypertension in patients with WHO class II or III symptoms. Improves exercise ability and decreases progression of clinical symptoms. Inhibits vessel constriction and elevation of blood pressure by competitively binding to endothelin-1 receptors ETA and ETB in endothelium and vascular smooth muscle. This leads to significant increase in cardiac index associated with significant reduction in pulmonary artery pressure, pulmonary vascular resistance, and mean right atrial pressure. Because of the risks of hepatic injury and teratogenic potential, only available through the Letairis Education and Access Program (LEAP). Prescribers and pharmacies must register with LEAP in order to prescribe and dispense. For more information, see http://www.letairis.com or call (866) 664-LEAP (5327).

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Phosphodiesterase (Type 5) Enzyme Inhibitors

Class Summary

The antiproliferative effects of the phosphodiesterase type 5 pathway (PDE5), which regulates cyclic guanosine monophosphate hydrolysis, may be significant in the chronic treatment of pulmonary hypertension with PDE5 inhibitors such as sildenafil.

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Sildenafil (Revatio)

 

Promotes selective smooth muscle relaxation in lung vasculature possibly by inhibiting PDE5. This results in subsequent reduction of blood pressure in pulmonary arteries and increase in cardiac output.

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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, and Heart Rhythm Society

Disclosure: Medtronic Inc Grant/research funds 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 and Sigma Xi

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Marschall S Runge, MD, PhD  Charles and Anne Sanders Distinguished Professor of Medicine, Chairman, Department of Medicine, Vice Dean for Clinical Affairs, University of North Carolina at Chapel Hill School of Medicine

Marschall S Runge, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians-American Society of Internal Medicine, American Federation for Clinical Research, American Federation for Medical Research, American Heart Association, American Physiological Society, American Society for Clinical Investigation, American Society for Investigative Pathology, Association of American Physicians, Association of Professors of Cardiology, Association of Professors of Medicine, Southern Society for Clinical Investigation, and Texas Medical Association

Disclosure: Pfizer Honoraria Speaking and teaching; Merck Honoraria Speaking and teaching; Orthoclinica Diagnostica Consulting fee Consulting

Amer Suleman, MD  Private Practice

Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD  Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Lisa A Hourigan, MBBS and Elyse Foster, MD to the development and writing of this article.

References

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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 CT chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome. Asc Ao = ascending aorta.
This transthoracic apical 4-chamber segment shows color Doppler flow across the interatrial septum at the site of a large ostium primum atrial septal defect (ASD). RA = right atrium, LA = left atrium.
This transesophageal image is from the mid esophagus of a patient with Eisenmenger syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely dilated pulmonary artery. Pulm a = pulmonary artery, Asc Ao = ascending aorta.
This is a color Doppler interrogation of the tricuspid valve in a case of Eisenmenger syndrome. It demonstrates an elevated estimated right ventricular systolic pressure of 106 mm Hg + right atrial pressure, reflecting pulmonary hypertension. TR = tricuspid regurgitation.
This is the transthoracic Doppler examination of 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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