1. Therapeutic methods used in patients with Eisenmenger syndrome.
- Author
-
Trojnarska O and Plaskota K
- Subjects
- Anemia, Iron-Deficiency etiology, Anemia, Iron-Deficiency prevention & control, Antihypertensive Agents therapeutic use, Arrhythmias, Cardiac etiology, Arrhythmias, Cardiac prevention & control, Blood Coagulation Disorders etiology, Blood Coagulation Disorders prevention & control, Blood Viscosity, Cyanosis etiology, Cyanosis prevention & control, Eisenmenger Complex complications, Eisenmenger Complex diagnosis, Eisenmenger Complex drug therapy, Eisenmenger Complex etiology, Endothelin Receptor Antagonists, Female, Heart Defects, Congenital complications, Heart Defects, Congenital physiopathology, Humans, Hypertension, Pulmonary etiology, Hypertension, Pulmonary prevention & control, Male, Nitric Oxide therapeutic use, Phosphodiesterase Inhibitors therapeutic use, Polycythemia etiology, Polycythemia prevention & control, Pregnancy, Pregnancy Complications, Cardiovascular etiology, Pregnancy Complications, Cardiovascular prevention & control, Prostaglandins I therapeutic use, Risk Reduction Behavior, Treatment Outcome, Eisenmenger Complex physiopathology, Eisenmenger Complex therapy
- 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.
- Published
- 2009