Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The term Eisenmenger Syndrome is used to refer to any systemic to pulmonary congenital communication causing pulmonary vascular obstructive disease (PVOD) severe enough as to produce bidirectional or reversed shunt. Once established, the PVOD deteriorates the quality of life and limits the survival of the patients with congenital heart disease. In the last decade, there has been a significant advance in the knowledge of the pathobiology of PVOD. Potentially important pathologic processes include: genetic abnormalities, hemodynamically-induced changes associated to shear stress, endothelial abnormalities, dysfunction of potassium channels, and extracellular matrix alterations. Other processes such as--in situ--thrombosis, angiogenesis, and cellular apoptosis may also be involved. The medical management of this condition has improved. The role of phlebotomy and long-term oxygen therapy is now better defined. Furthermore, as a result of a better knowledge in pathobiology, new and promising forms of pharmacological treatment have appeared, including prostacyclin analogs, such as Epoprostenol (Flolan) for continuous intravenous use and Trepostinil sodium (Remodulin) for continuous subcutaneous administration.
 ...
PMID:[Eisenmenger's syndrome. Advances in pathobiology and treatment]. 1200 47

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.
 ...
PMID:Therapeutic methods used in patients with Eisenmenger syndrome. 1995 85