INTRODUCTIONCongenital pulmonary vein stenosis (PVS) is a rare condition, resulting from abnormal incorporation of the common pulmonary vein into the left atrium. (1)(2)(3) One or more of the 4 pulmonary veins may be involved and it is commonly associated with other congenital heart defects (CHD), but can occur in isolation. (2)(3)(4) Commonly, patients present with respiratory symptoms with varying degrees of pulmonary hypertension. (5,6) Surgical results are generally poor with a high risk of recurrence and high mortality.(3) Reports are limited to small case series with little data on outcomes. (7) The aim of the study was to describe a series of cases with congenital pulmonary vein stenosis seen at the Chris Hani Baragwanath Academic Hospital, a large Southern African tertiary institution, over a 25-year period. METHODS AND SUBJECTSPatients were retrospectively identified using an electronic paediatric cardiology database system dating from January 1990 to January 2015. Clinical notes, catheterisation and imaging data of patients were reviewed. Data extracted for analysis included patient demographics, site of the lesion and the number of veins affected, associated congenital heart defects (CHD), and management strategies and outcomes. Patients with anomalous venous connection were excluded from this analysis. Background: Congenital pulmonary vein stenosis (PVS) is a rare condition, which results from abnormal embryological incorporation of the common pulmonary vein into the left atrium. Methods: A retrospective descriptive case series study was conducted with the aim of describing the characteristics and outcome of children with congenital PVS at an African tertiary care centre over a 25-year period. A computerised paediatric cardiology database initiated in the early 1990s was sourced to identify patients, following which clinical records were retrieved and reviewed. Results: Five cases of congenital PVS were identifi ed between January 1990 and January 2016 and accounted for 0.0007% of all congenital heart defects seen at the centre during the study period. The age at diagnosis ranged from 22 months -13 years. Most patients presented with respiratory symptoms, with 2 patients presenting with recurrent haemoptysis. The diagnosis of PVS was confi rmed by cardiac catheterisation and pulmonary angiography in all patients. All cases were right-sided unilateral PVS and all were associated with one or more congenital heart defects. Mild pulmonary hypertension and elevated capillary wedge pressures were found in all patients. Only one patient underwent specifi c surgery to relieve the PVS, which subsequently recurred. Another patient underwent a lobectomy following recurrent haemoptysis, but subsequently died of sepsis. Conclusion: Congenital PVS is a rare condition often associated with other congenital cardiac defects. Respiratory symptoms are common at presentation, with haemoptysis forming part of the presenting clinical spectrum, which is in keeping with the published literature. Elevated pulmonary artery pre...