Pulmonary vascular disease in children with truncus arteriosus (original) (raw)

Pulmonary vascular structure was analyzed using quantitative morphometric techniques in lung biopsy (n = 17) and/or postmortem specimens (n = 18) from 23 patients aged 18 days to 13 years, with truncus arteriosus (TA) type I or II. All 14 patients younger than age 1 year, showed abnormal extension of muscle and an increase in pulmonary arterial medial thickness (p <0.01 in 18 of 24 biopsy and autopsy specimens), whereas 6 showed intimal proliferation. Structural abnormalities were usually potentially reversible, even in 5 infants with a pulmonary arteriolar resistance of >8 units-m 2. Abnormalities were more severe in 9 patients aged 3.5 to 13 years, 6 with medial atrophy and 4 with pul-From the