title: Pulmonary vascular disease associated with pulmonary hypertension in 445 patients: diagnosis from lung biopsy and autopsy.
abstract: OBJECTIVE
  Diagnosis from lung biopsy or autopsy was performed in 445 patients with congenital (385) or acquired (60) heart disease from all over Japan. The purpose of this study is the presentation of these prospective data collections.
  
  
  METHODS
  Of the patients with congenital heart disease, 354 were biopsied to determine whether surgery was indicated. Decisions regarding surgery were based on the index of pulmonary vascular disease in simple cardiac anomalies or atrioventricular septal defects (AVSD). In total anomalous pulmonary venous connection (TAPVC), operative indication was determined by the degree of hypoplasia of small pulmonary arteries. Operability of Fontan procedure was based on the degree of residual medial hypertrophy after pulmonary artery banding.
  
  
  RESULTS
  In patients with simple cardiac anomalies, radical surgery was indicated in 166. Radical surgery was indicated in 50 patients with AVSD. In 26 patients with TAPVC, radical surgery was not indicated in 10. In 68 Fontan candidates, surgery was not indicated in 49. Among 7 patients with tetralogy of Fallot, 1 was not a surgical candidate. Of the 60 acquired heart disease patients, 16 had idiopathic pulmonary arterial hypertension and 36 had chronic thromboembolic pulmonary hypertension. In 6 patients, lung biopsy revealed pulmonary veno-occlusive disease; 2 patients had combined valvular disease.
  
  
  CONCLUSIONS
  The cardiac surgeon, pediatric cardiologist, and cardiologist who requested diagnosis from lung biopsy or autopsy were gratified with the results.
authors.affiliation: Japanese Research Institute of Pulmonary Vasculature, 40-1 Usagisaku, Shiroishi, Miyagi 989-0228, Japan. syamaki@ff.iij4u.or.jp
authors.forename: Shigeo
authors.initials: S
authors.lastname: Yamaki
mesh_terms: D000293:Adolescent; D001344:Autopsy; D001706:Biopsy; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D006330:Heart Defects, Congenital / Q000473:pathology* / Q000601:surgery; D006331:Heart Diseases / Q000473:pathology / Q000601:surgery; D006345:Heart Septal Defects, Ventricular / Q000473:pathology / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D007564:Japan; D008168:Lung / Q000473:pathology* / Q000601:surgery; D008297:Male; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000002:abnormalities; D011667:Pulmonary Veins / Q000002:abnormalities