Vol 16, No 6 (2009)
Review Article
Submitted: 2013-01-14
Published online: 2009-11-19
Therapeutic methods used in patients with Eisenmenger syndrome
Olga Trojnarska, Karolina Plaskota
DOI: 10.5603/cj.21432
·
Cardiol J 2009;16(6):500-506.
Vol 16, No 6 (2009)
Review articles
Submitted: 2013-01-14
Published online: 2009-11-19
Abstract
Patients with Eisenmenger syndrome form a small percentage of congenital heart disease
patients. The rarity of this syndrome, combined with its complex pathophysiology, account for
the insufficient understanding of the principles underlying its proper treatment. The main
clinical symptoms are: cyanosis due to secondary erythrocytosis, resulting in increased blood
viscosity, iron deficiency anemia (enhanced by unnecessary phlebotomies), blood clotting disturbances,
heart failure and serious supraventricular and ventricular arrhythmias. Recent
decades have seen developments in pulmonary hypertension pathophysiology which have led to
the introduction of new groups of drugs: prostacycline analogs (Epoprostenol, Treprostinil,
Beraprost, Illoprost), phosphodiesterase inhibitors (Sildenafil, Tadalafil), endothelin receptor
antagonists (Bosentan, Sitaxantan, Ambrisentan) and nitric oxide. These drugs should be
administered to patients in III-IV NYHA class. Despite successful early results, the therapeutic
effect on patients with Eisenmenger syndrome has not been conclusively established. Our
therapeutic efforts should be directed mainly towards preventing complications. As a rule, we
should avoid agents with no established therapeutic efficacy and try to alleviate symptoms
without any additional risk, so as not to disrupt the existing clinical balance.
Abstract
Patients with Eisenmenger syndrome form a small percentage of congenital heart disease
patients. The rarity of this syndrome, combined with its complex pathophysiology, account for
the insufficient understanding of the principles underlying its proper treatment. The main
clinical symptoms are: cyanosis due to secondary erythrocytosis, resulting in increased blood
viscosity, iron deficiency anemia (enhanced by unnecessary phlebotomies), blood clotting disturbances,
heart failure and serious supraventricular and ventricular arrhythmias. Recent
decades have seen developments in pulmonary hypertension pathophysiology which have led to
the introduction of new groups of drugs: prostacycline analogs (Epoprostenol, Treprostinil,
Beraprost, Illoprost), phosphodiesterase inhibitors (Sildenafil, Tadalafil), endothelin receptor
antagonists (Bosentan, Sitaxantan, Ambrisentan) and nitric oxide. These drugs should be
administered to patients in III-IV NYHA class. Despite successful early results, the therapeutic
effect on patients with Eisenmenger syndrome has not been conclusively established. Our
therapeutic efforts should be directed mainly towards preventing complications. As a rule, we
should avoid agents with no established therapeutic efficacy and try to alleviate symptoms
without any additional risk, so as not to disrupt the existing clinical balance.
Keywords
treatment; Eisenmenger syndrome
Title
Therapeutic methods used in patients with Eisenmenger syndrome
Journal
Cardiology Journal
Issue
Vol 16, No 6 (2009)
Article type
Review Article
Pages
500-506
Published online
2009-11-19
Page views
659
Article views/downloads
1294
DOI
10.5603/cj.21432
Bibliographic record
Cardiol J 2009;16(6):500-506.
Keywords
treatment
Eisenmenger syndrome
Authors
Olga Trojnarska
Karolina Plaskota
About this article