Pulmonary veno-occlusive disease.
Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension that mainly affects children and young adults. Its cause is unknown, although viral infections and drugs have been implicated. Patients with PVOD present with symptoms of right-sided heart failure. Radiologic examination shows prominent pulmonary arteries with Kerley B lines, pleural effusion, and mediastinal adenopathy. The definite diagnosis is made by histologic examination. Eccentric intimal fibrosis and recanalized thrombi in pulmonary veins and venules, arterialized veins, alveolar edema, and medial hypertrophy of arteries are seen on lung biopsy. No effective treatment is available; lung transplantation has been tried. The prognosis associated with PVOD is poor.
Veeraraghavan
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S
Division of Pulmonary and Critical Care Medicine, University of Southern California School of Medicine, Los Angeles 90033, USA.
Koss
M N
MN
Sharma
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OP
D000328:Adult; D002303:Cardiac Output, Low / Q000503:physiopathology; D002648:Child; D003952:Diagnostic Imaging; D005355:Fibrosis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D008168:Lung / Q000098:blood supply; D008206:Lymphatic Diseases / Q000503:physiopathology; D010996:Pleural Effusion / Q000503:physiopathology; D011379:Prognosis; D011650:Pulmonary Alveoli / Q000473:pathology; D011654:Pulmonary Edema / Q000473:pathology; D011655:Pulmonary Embolism / Q000503:physiopathology; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D017539:Tunica Intima / Q000473:pathology