Eisenmenger’s syndrome medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief: Cafer Zorkun, M.D., Ph.D. [2], Priyamavada Singh, MMBS [3], Kristin Feeney, B.S. [4], Abdelrahman Ibrahim Abushouk, MD[5]

Overview

If surgical intervention is not available, treatment is mostly palliative. It includes anticoagulants, pulmonary vasodilators such as bosentan, PGE5 inhibitor, prostacyclin, antibiotic prophylaxis to prevent endocarditis, phlebotomy to treat polycythemia, and maintaining proper fluid balance.

Medical Therapy

If surgical intervention is not available, treatment is mostly palliative. These measures can prolong lifespan and improve quality of life. They include[1][2][3]:

2018 ACC/AHA Guidelines for the Management of Adults With Congenital Heart Disease (DO NOT EDIT)

Medical Therapy - Eisenmenger Physiology (DO NOT EDIT)[4]

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)"

Follow-Up - Eisenmenger's Syndrome (DO NOT EDIT)[5]

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).[6] (Level of Evidence: B)"

References

  1. Gatzoulis MA, Beghetti M, Galiè N, Granton J, Berger RM, Lauer A; et al. (2008). "Longer-term bosentan therapy improves functional capacity in Eisenmenger syndrome: results of the BREATHE-5 open-label extension study". Int J Cardiol. 127 (1): 27–32. doi:10.1016/j.ijcard.2007.04.078. PMID 17658633.
  2. Galie N, Manes A, Palazzini M, Negro L, Marinelli A, Gambetti S; et al. (2008). "Management of pulmonary arterial hypertension associated with congenital systemic-to-pulmonary shunts and Eisenmenger's syndrome". Drugs. 68 (8): 1049–66. doi:10.2165/00003495-200868080-00004. PMID 18484798.
  3. Sun YJ, Yang T, Zeng WJ, Gu Q, Ni XH, Zhao ZH; et al. (2013). "Impact of sildenafil on survival of patients with Eisenmenger syndrome". J Clin Pharmacol. 53 (6): 611–8. doi:10.1002/jcph.78. PMID 23553708.
  4. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM; et al. (2019). "2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines". Circulation. 139 (14): e698–e800. doi:10.1161/CIR.0000000000000603. PMID 30586767.
  5. 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.
  6. Khairy P, Landzberg MJ, Gatzoulis MA, Mercier LA, Fernandes SM, Côté JM, Lavoie JP, Fournier A, Guerra PG, Frogoudaki A, Walsh EP, Dore A (2006). "Transvenous pacing leads and systemic thromboemboli in patients with intracardiac shunts: a multicenter study". Circulation. 113 (20): 2391–7. doi:10.1161/CIRCULATIONAHA.106.622076. PMID 16702467. Retrieved 2012-11-15. Unknown parameter |month= ignored (help)



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