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Medication

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.

Class Summary

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

Furosemide (Lasix)

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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.

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)

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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.

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)

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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)

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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.

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)

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Iron is a nutritionally essential inorganic substance.

Class Summary

These agents are indicated for symptomatic secondary gout.

Colchicine

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Colchicine decreases leukocyte motility and phagocytosis in inflammatory responses.

Class Summary

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

Bosentan (Tracleer)

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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)

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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 https://www.letairis.com or call (866) 664-LEAP (5327).

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)

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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)

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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.

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Media Gallery

Eisenmenger Syndrome. 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. RA = right atrium.
Eisenmenger Syndrome. Apical, 4-chamber, transthoracic echocardiographic 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.
Eisenmenger Syndrome. This computed tomography (CT) chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome. Desc. aorta = descending aorta.
Eisenmenger Syndrome. This apical, 4-chamber, transthoracic echocardiographic segment shows color Doppler flow across the interatrial septum at the site of a large ostium primum atrial septal defect (ASD). RA = right atrium.
Eisenmenger Syndrome. This transesophageal echocardiographic 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). Asc. Ao. = ascending aorta.
Eisenmenger Syndrome. 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 and right atrial pressure, reflecting pulmonary hypertension. TR = tricuspid regurgitation.
Eisenmenger Syndrome. This is a 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). It reveals an elevated estimated pulmonary artery diastolic pressure of 51 mm Hg and right atrial pressure. PR = pulmonic regurgitation.

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Author

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

Disclosure: Nothing to disclose.

Coauthor(s)

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.

Chief Editor

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;Verywell Health; MedStudy;WellnessVerge;Prose Media; AKH, Inc.; LearnRoll, LLC; Eradigm; RxCe.com; M3 USA; M3 EU; Future US Inc.; Edanz Group; ChesterPA511<br/>Serve(d) as a speaker or a member of a speakers bureau for: RxCe.com.

Additional Contributors

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.

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