Pulmonary venous obstruction. Report of a case mimicking primary pulmonary artery hypertension, with a review of the literature

Jon B. Tingelstad, Kurt Aterman, Edward C. Lambert

Children’s Hospital, Buffalo.
United States

American Journal of Diseases in Children
Am J Dis Child 1969; 117: 219-227
DOI: 10.1001/archpedi.1969.02100030221019

Abstract
While the literature on the pathology of the pulmonary arteries and arterioles in cor pulmonale is extensive, only a few cases of primary changes in the small pulmonary veins and venules leading to hypertension in the lesser circulation have been described. Since the clinical findings in patients with pulmonary vascular obstruction of either type are similar, a definite diagnosis may not be established until cardiac catheterization has been done, or may be proved only at necropsy. To our knowledge cardiovascular studies have been performed in only three of the published cases of pulmonary venous obstruction.1-3 Because this condition is rare in young children and is difficult to distinguish from primary pulmonary artery hypertension, we are describing a 21-month-old child with idiopathic pulmonary venous subintimal fibrosis and severe vascular obstruction proved by necropsy. The symptoms, clinical findings, the electrocardiogram and the results of cardiovascular studies, including cardiac catheterization and selective

Category
Uncategorized: Potential early case of pediatric pulmonary veno-occlusive disease
Pulmonary Venous Pathology

Year of Publication: 1969

Age Focus: Pediatric

Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients)

Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: No