ABSTRACT
Pulmonary arterial hypertension (PAH) is a common complication of congenital heart disease (CHD), particularly when a significant left-to-right (systemic-to-pulmonary) shunt remains uncorrected. Long-term exposure of the pulmonary bed to increased blood flow and pressure can result in vascular remodeling and dysfunction. This will, in turn, lead to a rise in pulmonary vascular resistance (PVR) and, if severe enough, will cause reversal of the shunt and the development of Eisenmenger’s syndrome. First described by Victor Eisenmenger in 1897 (1), Eisenmenger’s syndrome was defined in 1958 by Paul Wood as “pulmonary hypertension at systemic level due to high PVR with reversed or bidirectional blood flow through a septal defect” (2). Eisenmenger’s syndrome represents the most advanced form of pulmonary arterial hypertension related to congenital heart disease (PAH-CHD).
