Circulation, Vol 58, 1107-1122, Copyright © 1978 by American Heart Association
M Rabinovitch, SG Haworth, AR Castaneda, AS Nadas and LM Reid
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.
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Lung biopsy in congenital heart disease: a morphometric approach to pulmonary vascular disease
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