title: Inoperable pulmonary vascular disease in infants with congenital heart disease.
abstract: BACKGROUND
  Among 120 infants less than 12 months of age who had lung biopsy and autopsy, 20 were inoperable because of severe irreversible pulmonary vascular disease.
  
  
  METHODS
  The infants were classified into three groups. Group 1 comprised 6 patients who showed complete obstruction of the small pulmonary arterial lumen and atrophy of the peripheral arterial media and who were considered to have absolute operative contraindications. Group 2 comprised 6 patients who had no pathologic findings of absolute operative contraindication and had an index of pulmonary vascular disease of more than 2.2. They were isolated as having advanced plexogenic pulmonary arteriopathy. Group 3 comprised 8 patients who had extremely thickened media of small pulmonary arteries, with abnormally thickened media extending into the small peripheral arteries characterized by extremely narrow lumina and medial thickness exceeding luminal diameter.
  
  
  RESULTS
  Six of the 9 patients in whom operative repair was abandoned on the basis of preoperative or intraoperative lung biopsy are still alive. Of the 11 patients who underwent operation without biopsy, none survived.
  
  
  CONCLUSIONS
  Preoperative or intraoperative lung biopsy and assessment of arteriopathy based on the above criteria are recommended in all patients in whom fatal pulmonary vascular disease is suspected.
authors.affiliation: Department of Cardiology, Katta General Hospital, Shiroishi, Japan. s-heart@mail.cc.tohoku.ac.jp
authors.forename: S
authors.initials: S
authors.lastname: Yamaki
authors.forename: A
authors.initials: A
authors.lastname: Abe
authors.forename: K
authors.initials: K
authors.lastname: Tabayashi
authors.forename: M
authors.initials: M
authors.lastname: Endo
authors.forename: H
authors.initials: H
authors.lastname: Mohri
authors.forename: T
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D001284:Atrophy; D006330:Heart Defects, Congenital / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications*; D007223:Infant; D008168:Lung / Q000473:pathology; D011651:Pulmonary Artery / Q000473:pathology*; D015996:Survival Rate; D017540:Tunica Media / Q000473:pathology