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]]

Overview

Medical Therapy

If surgical intervention is not available, treatment is mostly palliative

  • Anticoagulants
  • Pulmonary vasodilators such as bosentan
  • PGE 5 inhibitor
  • Prostacyclin may improve pulmonary artery pressure and may improve length of life.
  • Antibiotic prophylaxis to prevent endocarditis
  • Phlebotomy to treat polycythemia
  • Maintaining proper fluid balance.
  • These measures can prolong lifespan and improve quality of life.

ACC / AHA 2008 Guidelines- Recommendations for Medical Therapy- Eisenmenger's syndrome(DO NOT EDIT)[1]

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) "

ACC / AHA Guidelines- Recommendations for Follow-Up - Eisenmenger's Syndrome(DO NOT EDIT)[1]

Class I
"1. Patients with CHD-related PAH should:

a. Have coordinated care under the supervision of a trained CHD and PAH provider and be seen by such individuals at least yearly. (Level of Evidence: C) "

b. Have yearly comprehensive evaluation of functional capacity and assessment of secondary complications.(Level of Evidence: C) "

c. Discuss all medication changes or planned interventions with their CHD-related PAH caregiver. (Level of Evidence: C) "

Class III
"1. Endocardial pacing is not recommended in patients with CHD-PAH with persistent intravascular shunting, and alternative access for pacing leads should be sought (the risks should be individualized).136 (Level of Evidence: B) "

References

  1. 1.0 1.1 Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA; et al. (2008). "ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons". J Am Coll Cardiol. 52 (23): e1–121. doi:10.1016/j.jacc.2008.10.001. PMID 19038677.

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