Congenital pulmonary vein stenosis: Structural changes in a patient with normal pulmonary artery wedge pressure

A male infant is described who died at 13 months of age with stenosis of all extrapulmonary veins except the left upper vein. The pulmonary artery wedge pressure was normal, the first time this is reported in this condition. At autopsy, there were structural changes of the pulmonary arteries and vei...

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Bibliographic Details
Published in:Journal of the American College of Cardiology 1984-01, Vol.3 (1), p.193-199
Main Authors: Geggel, Robert L., Fried, Ruthellen, Tuuri, Dwight T., Fyler, Donald C., Reid, Lynne M.
Format: Article
Language:English
Subjects:
Cardiac Catheterization
Constriction, Pathologic
Humans
Hypertension, Pulmonary - pathology
Infant
Lung - pathology
Male
Pulmonary Artery - pathology
Pulmonary Circulation
Pulmonary Veins - abnormalities
Pulmonary Veins - pathology
Pulmonary Wedge Pressure
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Summary:A male infant is described who died at 13 months of age with stenosis of all extrapulmonary veins except the left upper vein. The pulmonary artery wedge pressure was normal, the first time this is reported in this condition. At autopsy, there were structural changes of the pulmonary arteries and veins in all lobes with or without pulmonary vein stenosis. Arterial changes—muscle extension, medial hypertrophy and decreased arterial size— analyzed quantitatively were found to be similar in all lobes. Venous medial hypertrophy was more marked in obstructed lobes. These anatomic changes are presumably due to fixed venous obstruction in the pulmonary lobes drained by stenotic veins and to high flow in the left upper lobe.
ISSN:0735-1097
1558-3597
DOI:10.1016/S0735-1097(84)80447-7