O. Sacco, B. Fregonese, L. Fregonese, C. Gambini, G. Pongiglione, G. A. Rossi
I.R.C.C.S. Giannina Gaslini.
Italy
Pediatric Pulmonology
Pediatr Pulmonol 2002; 34: 324-328
DOI: 10.1002/ppul.10158
Abstract
A variety of pulmonary vascular disorders, such as hemangiomatosis, telangectasia, and veno-occlusive disease, may be involved in the pathogenesis of interstitial lung diseases. We describe the case of a girl with recurrent bacterial pneumonia and progressive interstitial fibrosis affecting the right lung. Morphologic evaluation of the lung biopsy showed structural changes of the vessel walls suggesting pulmonary hypertension. The echocardiogram showed the presence of centripetal blood flow in the right pulmonary artery from the periphery of the lung to the heart. A selective right angiography demonstrated the presence of pulmonary venous obstruction at the veno-atrial junction, successfully treated by endovascular stent implantation during cardiac catheterization.
Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Surgical and Catheter-mediated Interventions for Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
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