Recurrent unilateral bacterial pneumonias and interstitial fibrosis associated with pulmonary vein atresia: Successful treatment with endovascular stent implantation

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.