TY - JOUR
T1 - Review of evidence for bosentan therapy for treatment of Eisenmenger syndrome
AU - Hartwig, Benjamin J.
AU - Schultze, Benjamin
N1 - Copyright:
This record is sourced from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
PY - 2019/1/1
Y1 - 2019/1/1
N2 - BACKGROUND AND PURPOSE: Eisenmenger syndrome (ES) is a rare condition caused by a right-to-left cyanotic shunt. To date, only heart-lung transplant has been shown to be curative. Bosentan is the only medication studied with a double-blind placebo-controlled randomized trial for management of this condition. The intent of this article is to explore the literature surrounding bosentan in ES and assess its efficacy. METHODS: A literature review was conducted with no limitation on date. Titles were scanned for applicability, and abstracts of those articles found to be pertinent were reviewed. Those articles considered relevant based on the abstract were read in entirety. CONCLUSIONS: Eisenmenger syndrome remains incurable except through heart-lung transplant. Although no specific medical treatment or algorithm exists, three pharmacological classes show promise in disease management: endothelin receptor antagonists, phosphodiesterase inhibitors, and prostacyclins. Combined therapy with these agents may improve cardiopulmonary function. Bosentan has not been proven as a monotherapy for ES and is not appropriate in all patients as side effects are commonly reported. IMPLICATIONS FOR PRACTICE: Further study is required to assess efficacy of combination therapy and utilization as a bridge to transplant or surgical correction of the underlying defect.
AB - BACKGROUND AND PURPOSE: Eisenmenger syndrome (ES) is a rare condition caused by a right-to-left cyanotic shunt. To date, only heart-lung transplant has been shown to be curative. Bosentan is the only medication studied with a double-blind placebo-controlled randomized trial for management of this condition. The intent of this article is to explore the literature surrounding bosentan in ES and assess its efficacy. METHODS: A literature review was conducted with no limitation on date. Titles were scanned for applicability, and abstracts of those articles found to be pertinent were reviewed. Those articles considered relevant based on the abstract were read in entirety. CONCLUSIONS: Eisenmenger syndrome remains incurable except through heart-lung transplant. Although no specific medical treatment or algorithm exists, three pharmacological classes show promise in disease management: endothelin receptor antagonists, phosphodiesterase inhibitors, and prostacyclins. Combined therapy with these agents may improve cardiopulmonary function. Bosentan has not been proven as a monotherapy for ES and is not appropriate in all patients as side effects are commonly reported. IMPLICATIONS FOR PRACTICE: Further study is required to assess efficacy of combination therapy and utilization as a bridge to transplant or surgical correction of the underlying defect.
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U2 - 10.1097/JXX.0000000000000104
DO - 10.1097/JXX.0000000000000104
M3 - Review article
C2 - 30475252
AN - SCOPUS:85059796160
VL - 31
SP - 72
EP - 77
JO - Journal of the American Association of Nurse Practitioners
JF - Journal of the American Association of Nurse Practitioners
SN - 2327-6886
IS - 1
ER -