Reshma Amin, S. Kwon, Y. Moayedi, Neil Sweezey
Hospital for Sick Children.
Canada
Canadian Respiratory Journal
Can Respir J 2009; 16: e77-e80
DOI: 10.1155/2009/791653
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.
Conclusion: 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.
Category
Stenosis or Obstruction of Normal Pulmonary Venous Connections
Pulmonary Hypertension Associated with Stenosis or Atresia of Pulmonary Veins
Surgical Interventions for Pulmonary Venous Obstruction After the Onset of Disease
Year of Publication: 2009
Age Focus: Pediatric
Article Type: Case Reports or Retrospective Observations in Small Groups of Patients (≤10 patients). Literature Review and Analysis.
Article Access: Free PDF File or Full Text Article Available Through PubMed or DOI: Yes