title: Pulmonary vein stenosis: Case report and literature review.
abstract: BACKGROUND
  Pulmonary vein stenosis is a rare cause of pulmonary hypertension, with variable onset and presentation. One or more of the four pulmonary veins can be primarily or secondarily affected. A five-month-old girl presented with respiratory distress, lethargy and cyanosis requiring intubation.
  
  
  METHODS
  Echocardiography showed right ventricular dilation, a right ventricular systolic pressure of 97 mmHg, decreased ejection fraction and turbulent flow at the left atrium. Cardiac catheterization revealed stenosis of the left-sided pulmonary veins, for which she underwent a Coles procedure.
  
  
  RESULTS
  Postoperatively, there was a transient improvement in the patient's pulmonary hypertension but she subsequently deteriorated. Her prognosis was considered bleak, and a decision was made with the family to withdraw care.
  
  
  CONCLUSIONS
  Pulmonary vein stenosis is a rare cause of pulmonary hypertension, and is associated with significant morbidity and mortality. Surgical intervention may be of benefit in selected cases. It stands to reason that any treatment will have the best chance of success if completed before the pulmonary hypertension becomes fixed. Early diagnosis depends on a high index of clinical suspicion.
authors.affiliation: The Hospital for Sick Children, Toronto, Ontario, Canada. reshma.amin@sickkids.ca
authors.forename: R
authors.initials: R
authors.lastname: Amin
authors.forename: S
authors.initials: S
authors.lastname: Kwon
authors.forename: Y
authors.initials: Y
authors.lastname: Moayedi
authors.forename: N
authors.initials: N
authors.lastname: Sweezey
mesh_terms: D006328:Cardiac Catheterization; D003251:Constriction, Pathologic / Q000150:complications; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D007223:Infant; D011667:Pulmonary Veins / Q000000981:diagnostic imaging* / Q000473:pathology*; D016896:Treatment Outcome; D014463:Ultrasonography