Evaluation of Primary Pulmonary Vein Stenosis in Children: Comparison of Radionuclide Perfusion Lung Scan and Angiography.

Abstract

The purpose of this study was to evaluate the utility of radionuclide perfusion lung scanning in the evaluation of primary pulmonary vein stenosis (PVS) in pediatric patients by comparing it with angiography. We retrospectively identified pediatric patients with primary PVS who underwent both lung scanning and angiography. A cohort of 34 patients was evaluated. The presence of PVS in the right upper, right lower, left upper, and left lower pulmonary veins on angiograms was recorded. Two nuclear medicine physicians evaluated the lung scans for perfusion defects. Agreement between lung scan and angiographic findings was assessed with contingency tables. Sensitivity and specificity of lung scanning for accurate detection of PVS with angiographic findings as the reference standard were assessed by ROC analysis. Angiography depicted PVS in 90 of the total 136 pulmonary veins (66%). Lung scans correctly depicted 65 (72%) of the cases of PVS diagnosed with angiography. The sensitivity and specificity of lung scans were 76.0% and 88.9% for the right upper pulmonary vein, 70.6% and 94.1% for the right lower pulmonary vein, 77.3% and 58.3% for the left upper pulmonary vein, and 65.4% and 87.5% for the left lower pulmonary vein. Lung scan findings correlate with angiographic findings in the detection of primary PVS in pediatric patients. Perfusion lung scanning may have a role in angiographically diagnosed PVS by noninvasively showing relative perfusion at the tissue level.