Eisenmenger syndrome: medical prevention and management strategies.

The original definition of Eisenmenger syndrome refers to an unrestrictive post-tricuspid valve congenital systemic-to-pulmonary shunt. When the pulmonary arterial systolic pressure becomes equal to the systemic arterial systolic pressure, the direction of the shunt becomes pulmonary-to-systemic. The latter leads to progressive cyanosis, and exercise intolerance is initially proportional to the degree of hypoxaemia. Later, congestive heart failure may occur . The management principle is to avoid any factors that destabilise this delicately balanced physiology. Until recently, this could only be achieved by symptomatic therapy; however, when patients are severely incapacitated, transplantation is needed. At present, new drugs, which are more selective pulmonary vasodilators, are available to interfere with the ongoing disease process to improve functional capacity and delay the decision for transplantation.