Progressive pulmonary venous obstruction and pulmonary interstitial fibrosis associated with tetralogy of Fallot

Pediatr Cardiol. 1994 Mar-Apr;15(2):95-9. doi: 10.1007/BF00817617.


Stenosis of individual pulmonary veins is a rare condition associated with a very high mortality. The present report is the first known case of progressive pulmonary venous obstruction associated with the tetralogy of Fallot. This clinical and anatomical study suggests that initial dynamic and possibly reversible occlusion occurs at the junction of the pulmonary vein and left atrium. Anatomical lesions in pulmonary veins then develop, possibly secondary to functional stenosis. The usually fatal outcome of these cases is due to the development of fixed pulmonary venous occlusion and possibly pulmonary interstitial fibrosis. The rapid progression of pulmonary venous stenosis strongly suggests that discrete veno-atrial stenosis should be surgically corrected as early as possible to prevent the irreversible diffuse pulmonary venous obstruction associated with an invariably fatal prognosis.

Publication types

  • Case Reports

MeSH terms

  • Angiography
  • Ductus Arteriosus, Patent / complications*
  • Ductus Arteriosus, Patent / diagnostic imaging
  • Ductus Arteriosus, Patent / surgery
  • Fatal Outcome
  • Female
  • Humans
  • Infant
  • Infant, Low Birth Weight
  • Infant, Newborn
  • Infant, Premature
  • Infant, Premature, Diseases / diagnostic imaging
  • Infant, Premature, Diseases / physiopathology*
  • Infant, Premature, Diseases / surgery
  • Pulmonary Fibrosis / etiology*
  • Pulmonary Fibrosis / pathology
  • Pulmonary Veno-Occlusive Disease / diagnostic imaging
  • Pulmonary Veno-Occlusive Disease / etiology*
  • Pulmonary Veno-Occlusive Disease / pathology
  • Pulmonary Veno-Occlusive Disease / surgery
  • Tetralogy of Fallot / complications*
  • Tetralogy of Fallot / diagnostic imaging
  • Tetralogy of Fallot / surgery
  • Ultrasonography