The Pathophysiology of Pulmonary Vascular Disease Associated with Congenital Cardiac Lesions

Abstract

Many types of congenital cardiac lesions are associated with the development of pulmonary vascular obstructive disease (PVOD). This connection was first reported by Eisenmenger (1897) in reference to a ventricular septal defect. Abbott and Dawson (1924) created some order in the anatomic classifications, and various lists of lesions predisposing to the Eisenmenger reaction have been compiled, but in these, lesions tend to be grouped according to the level of the shunt (Abbott and Dawson 1924, Baumgartner and Abbott 1929, Graham 1979, Moschowitz 1927, O’Rourke 1967, Rosedale 1935, Wood 1958). The lumping of congenital defects according to anatomy is predictive in only the most general sense; such a grouping does not necessarily correlate with rate or severity of pulmonary vascular disease. For example, total anomalous pulmonary venous drainage and atrial septal defect are not similar in their pulmonary vascular effects, although both have atrial-level shunts, nor is atrioventricular septal defect similar to typical large VSD in tendency to cause vascular disease. These different defects at the same “shunt level” do have differing hemodynamics, but the relationship of the differences to the progression of pulmonary vascular disease has not been clarified.