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Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect
Edward Y Lee1, Ryan Callahan2, Sara O Vargas3
1Department of Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Avenue, Boston, MA 02115, USA.
Insights
Pulmonary vein stenosis (PVS) in children with cardiac septal defects shows distinct chest imaging signs on MDCT angiography. Findings include ground-glass opacity, septal thickening, pleural thickening, and mediastinal masses.
Area of Science:
- Radiology
- Pediatric Imaging
- Cardiovascular Imaging
Background:
- Pulmonary vein stenosis (PVS) is a serious condition in children, often associated with cardiac septal defects.
- Accurate diagnosis of PVS and its associated findings is crucial for effective management.
Purpose of the Study:
- To identify and describe the extravascular thoracic findings on multi-detector computed tomography (MDCT) angiography in pediatric patients with PVS and cardiac septal defects.
- To correlate imaging findings with the presence of PVS.
Main Methods:
- Retrospective analysis of thoracic MDCT angiography studies in pediatric patients (age ≤ 18 years) with confirmed cardiac septal defect and PVS.
- Independent review by two pediatric radiologists to assess lung, airway, pleural, and mediastinal abnormalities.
- Interobserver agreement assessed using kappa statistics.
Main Results:
- Twenty pediatric patients with cardiac septal defect and PVS were included.
- Common findings included ground-glass opacity (90%), septal thickening (45%), pleural thickening (80%), and mediastinal soft tissue masses (45%).
- High interobserver agreement (k = 0.99) was observed for detecting extravascular abnormalities.
Conclusions:
- PVS in children with cardiac septal defects presents characteristic extravascular thoracic MDCT angiography findings.
- Ground-glass opacity, septal thickening, and pleural thickening are frequent in the lungs and pleura.
- Mediastinal soft tissue masses adjacent to pulmonary veins are observed in nearly half of these patients.
Abstract:
Purpose: To retrospectively investigate the extravascular thoracic MDCT angiography findings of pulmonary vein stenosis (PVS) in children with a cardiac septal defect. Materials and Methods: Pediatric patients (age ≤ 18 years) with cardiac septal defect and PVS, confirmed by echocardiogram and/or conventional angiography, who underwent thoracic MDCT angiography studies from April 2009 to April 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT angiography studies for the presence of extravascular thoracic abnormalities in: (1) lung and airway (ground-glass opacity (GGO), consolidation, pulmonary nodule, mass, cyst, septal thickening, fibrosis, and bronchiectasis); (2) pleura (pleural thickening, pleural effusion, and pneumothorax); and (3) mediastinum (mass and lymphadenopathy). Interobserver agreement between the two independent pediatric radiology reviewers was evaluated with kappa statistics. Results: The final study group consisted of 20 thoracic MDCT angiography studies from 20 consecutive individual pediatric patients (13 males (65%) and 7 females (35%); mean age: 7.5 months; SD: 12.7; range: 2 days to 7 months) with cardiac septal defect and PVS. The characteristic extravascular thoracic MDCT angiography findings were GGO (18/20; 90%), septal thickening (9/20; 45%), pleural thickening (16/20; 80%), and ill-defined, mildly heterogeneously enhancing, non-calcified soft tissue mass (9/20; 45%) following the contours of PVS in the mediastinum. There was a high interobserver kappa agreement between two independent reviewers for detecting extravascular abnormalities on thoracic MDCT angiography studies (k = 0.99). Conclusion: PVS in children with a cardiac septal defect has a characteristic extravascular thoracic MDCT angiography finding. In the lungs and pleura, GGO, septal thickening, and pleural thickening are frequently seen in children with cardiac septal defect and PVS. In the mediastinum, a mildly heterogeneously enhancing, non-calcified soft tissue mass in the distribution of PVS in the mediastinum is seen in close to half of the pediatric patients with cardiac septal defect and PVS.
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