[Eisenmenger's syndrome. Advances in pathobiology and treatment]

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.