title: Severe pulmonary vascular disease consisting of ventricular septal defect and patent ductus arteriosus. A case diagnosed as the indication for total correction by the hemodynamic study.
abstract: A 12-year-old girl with ventricular septal defect, patent ductus arteriosus and pulmonary hypertension was hemodynamically diagnosed as the indication for total correction by the initial cardiac catheterization. However, histologic assessment by lung biopsy revealed the terminal stage of plexogenic pulmonary arteriopathy, i.e. plexiform lesions, dilatation lesions and fibrinoid necrosis of the media which denied the possibility of corrective surgery. We would like to stress that the open lung biopsy should be performed when the hemodynamic study indicates marginal operability, especially in older children and adults in order to investigate the grade of occlusive pulmonary vascular disease.
mesh_terms: D002648:Child; D004374:Ductus Arteriosus, Patent / Q000150:complications* / Q000503:physiopathology; D005260:Female; D006345:Heart Septal Defects, Ventricular / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008171:Lung Diseases / Q000209:etiology* / Q000473:pathology / Q000503:physiopathology