Methods : We reviewed all available clinical and laboratory data in
CARDIOVASCULAR JOURNAL OF AFRICA • Vol 24, No 1, January/February 2013 244 AFRICA patients with a diagnosis of pulmonary vein stenosis. We excluded patients with total anomalous pulmonary venous drainage, atrial isomerism and gestational age ≤ 35 weeks. Results : We identified 11 patients with pulmonary vein stenosis, eight were male, median gestational age was 27 weeks (25–34), median birth weight was 860 g (432–2 100 g), and three patients were of twin pregnancies whose twin siblings were unaffected. Most patients were diagnosed with chronic lung disease and needed significant respiratory support after birth. In nine/11 initial neonatal echocardiograms did not report abnormal pulmonary vein flow. The median age at diagnosis was 5 months (3 months –2 years) the diagnosis was most often made by or suspected by echocardiography because of apparent worsening of chronic lung disease. Eight/11 patients underwent CT scan or MRI. The left pulmonary vein was the most commonly stenosed (91%) and all patients underwent a suture- less surgical repair. Median survival after pulmonary vein surgery was six months (4–10). In seven/11 PVS recurred and four/11 patients died. Conclusion : Pulmonary vein stenosis should be considered if an ex-premature baby has late deterioration of chronic lung disease or evidence of pulmonary hypertension by echocardiogram. Further imaging by CT scan or MRI may be required to complete the diag- nosis. Pulmonary vein stenosis appears to develop postnatally but the aetiology remains unknown. The response to surgery and late recurrence appear to be similar to pulmonary vein stenosis in infants born at term. 1483: SERIAL MEASURES OF SYSTEMIC-TO-PULMONARY ARTERIAL COLLATERAL FLOW IN PATIENTS WITH SUPERIOR AND TOTAL CAVOPULMONARY CONNEC- TIONS Kevin Whitehead 1 , Matthew Harris 1 , Andrew Glatz 1 , Yoav Dori 1 , Marc Keller 2 , Jonathan Rome 1 , Mark Fogel 1 1 Division of Cardiology, Children’s Hospital of Philadelphia, USA 2 Department of Radiology, Children’s Hospital of Philadelphia, USA Background: We have described a method of quantifying systemic- to-pulmonary collateral (CollF) flow in patients with superior (SCPC) and total (TCPC) cavopulmonary connections using magnet- ic resonance phase-contrast velocity mapping (PC-MRI). Cross- sectional data suggests that CollF decreases with time after TCPC completion, but may take years. We wished to examine more acute changes in physiology and CollF from SCPC to TCPC by comparing serial (paired) PC-MRI data. Methods: We retrospectively reviewed PC-MRI data to identify patients who had CollF quantified at both SCPC and TCPC. CollF, systemic blood flow (Qs = total caval flow), and pulmonary blood flow (Qp = total pulmonary vein flow) were quantified as previously described. Fenestration flow was quantified in TCPC by the differ- ence in caval and pulmonary artery flow.
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- pulmonary artery, CardioVascular Journal of Africa, cardiovascular journal