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Fifty patients with congenital heart disease, ages 2 days-30 years (median 12 months) at cardiac surgery, underwent lung biopsy to assess pulmonary vascular disease (PVD). Twenty-six had ventricular septal defects (VSD), 17 d-transposition of the great arteries (D-TGA), and seven, defects of the atrioventricular canal (AVC). Quantitative morphologic data was correlated with hemodynamic data. Three new grades of PVD were observed. Abnormal extension of muscle into peripheral arteries (grade A) was found in all patients; all had increased pulmonary blood flow. In addition, 38 of 50 patients had an increase in percentage arterial wall thickness (grade B); this correlated with elevation in pulmonary artery (PA) pressure (r = 0.59). Another 10 of 50 patients had, in addition to A and B, a reduction in the number of small arteries (grade C); nine of 10 were patients with elevated PA resistance greater than 3.5 mu/m2 (P less than 0.005). All three patients with Heath-Edwards changes of grade III or worse also had grade C. Reduction in peripheral arterial number probably precedes obliterative PVD and may identify those patients in whom, despite corrective surgery, PVD will progress.

Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease.