Eisenmenger’s syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2], Priyamavada Singh, MMBS [[3]]Assistant Editor-In-Chief: Kristin Feeney, B.S. [[4]]

Medical Therapy

If surgical intervention is not available, treatment is mostly palliative, using anticoagulants, pulmonary vasodilators such as bosentan, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance. These measures can prolong lifespan and improve quality of life.

ACC / AHA Guidelines- Recommendations for Medical Therapy of Eisenmenger Physiology (DO NOT EDIT)

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Class I 1. It is recommended that patients with Eisenmenger syndrome avoid the following activities or exposures, which carry increased risks: a. Pregnancy. (Level of Evidence: B) b. Dehydration. (Level of Evidence: C) c. Moderate and severe strenuous exercise, particularly isometric exercise. (Level of Evidence: C) d. Acute exposure to excessive heat (eg, hot tub or sauna). (Level of Evidence: C) e. Chronic high-altitude exposure, because this causes further reduction in oxygen saturation and increased risk of altitude-related cardiopulmonary complications (particularly at an elevation greater than 5000 feet above sea level). (Level of Evidence: C) f. Iron deficiency. (Level of Evidence: B) 2. Patients with Eisenmenger syndrome should seek prompt therapy for arrhythmias and infections. (Level of Evidence: C) 3. Patients with Eisenmenger syndrome should have hemoglobin, platelet count, iron stores, creatinine, and uric acid assessed at least yearly. (Level of Evidence: C) 4. Patients with Eisenmenger syndrome should have assessment of digital oximetry, both with and without supplemental oxygen therapy, at least yearly. The presence of oxygen-responsive hypoxemia should be investigated further. (Level of Evidence: C) 5. Exclusion of air bubbles in intravenous tubing is recommended as essential during treatment of adults with Eisenmenger syndrome. (Level of Evidence: C) 6. Patients with Eisenmenger syndrome should undergo noncardiac surgery and cardiac catheterization only in centers with expertise in the care of such patients. In emergent or urgent situations in which transportation is not feasible, consultation with designated caregivers in centers with expertise in the care of patients with Eisenmenger syndrome should be performed and sustained throughout care. (Level of Evidence: C) Class IIa 1. All medications given to patients with Eisenmenger physiology should undergo rigorous review for the potential to change systemic blood pressure, loading conditions, intravascular shunting, and renal or hepatic flow or function. (Level of Evidence: C) 2. Pulmonary vasodilator therapy can be beneficial for patients with Eisenmenger physiology because of the potential for improved quality of life. (Level of Evidence: C)

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