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
Absence or Atresia of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Symptoms and Quality of Life Associated with Pulmonary Venous Obstruction
Diagnostic Testing. Noninvasive
Diagnostic Testing. Invasive
Pulmonary Venous Pathology
Catheter-mediated Interventions: Efficacy or Lack of Efficacy
Year of Publication: 2002
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