ABSTRACT
Cyanotic congenital heart diseases (CHD) in adulthood include patients with pulmonary arterial hypertension (Eisenmenger syndrome is at the extreme end of the spectrum) and those without pulmonary arterial hypertension. Each group must be distinguished given the essential difference in pathophysiology and the potential for surgical repair in those without pulmonary arterial hypertension. Eisenmenger syndrome is an extreme form of pulmonary vascular obstructive disease with irreversible pulmonary hypertension in the presence of high pulmonary vascular resistance associated with shunt-related CHD. Five to 10 per cent of patients with CHD develop pulmonary arterial hypertension of variable severity. The development of Eisenmenger syndrome depends on timely detection and operation of a shunt-related CHD in childhood. In Eisenmenger syndrome, there is a massive dilatation of pulmonary trunk and dilatation of central pulmonary branches. There may even be aneurysmatic dilatation of the pulmonary artery. In the periphery, there is a distinct reduction of the lumen of pulmonary arteries, causing higher transparency of the lungs.
