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Secondary Pulmonary Vein Stenosis Due to Total Anomalous Pulmonary Venous Connection Repair in Children:
Edward Y Lee1, Sara O Vargas2, Kathy J Jenkins3
1Department of Radiology, Boston Children's Hospital and Harvard Medical School, Boston, MA 02115, USA.
Insights
Secondary pulmonary vein stenosis (PVS) in children after total anomalous pulmonary venous connection (TAPVC) repair shows characteristic extravascular findings on CT angiography, including mediastinal soft tissue masses. These findings aid in diagnosing PVS in this pediatric population.
Area of Science:
- Pediatric Radiology
- Cardiovascular Imaging
- Thoracic Imaging
Background:
- Secondary pulmonary vein stenosis (PVS) is a complication following surgical repair of total anomalous pulmonary venous connection (TAPVC).
- Accurate diagnosis and characterization of PVS are crucial for managing pediatric patients post-TAPVC repair.
Purpose of the Study:
- To evaluate and characterize extravascular thoracic findings on multi-detector computed tomography (MDCT) angiography in children with secondary PVS after TAPVC repair.
- To identify novel imaging features associated with this condition.
Main Methods:
- Retrospective review of thoracic MDCT angiography studies in pediatric patients (≤18 years) with confirmed secondary PVS post-TAPVC repair.
- Independent review by two pediatric radiologists to identify and evaluate extravascular abnormalities in the lungs, pleura, and mediastinum.
- Assessment of interobserver agreement using kappa statistics.
Main Results:
- Twenty pediatric patients with secondary PVS post-TAPVC repair were analyzed.
- Common findings included ground-glass opacity (95%), pleural thickening (85%), and a characteristic non-calcified mediastinal soft tissue mass (85%).
- Excellent interobserver agreement (kappa = 0.99) was achieved for detecting these abnormalities.
Conclusions:
- Thoracic MDCT angiography reveals characteristic extravascular findings in pediatric secondary PVS due to TAPVC repair.
- Ground-glass opacity, septal thickening, and pleural thickening are frequent pulmonary and pleural findings.
- A mildly enhancing, non-calcified mediastinal soft tissue mass adjacent to the pulmonary veins is a novel and significant imaging finding.
Purpose:
To evaluate extravascular findings on thoracic MDCT angiography in secondary pulmonary vein stenosis (PVS) due to total anomalous pulmonary venous connection (TAPVC) repair in children.
Materials And Methods:
All patients aged ≤18 years with a known diagnosis of secondary PVS after TAPVC repair, confirmed by echocardiography, conventional angiography, and/or surgery, who underwent thoracic MDCT angiography studies between July 2008 and April 2021 were included. Two pediatric radiologists independently examined MDCT angiography studies for the presence of extravascular thoracic abnormalities in the lung, pleura, and mediastinum. The location and distribution of each abnormality (in relation to the location of PVS) were also evaluated. Interobserver agreement between the two independent pediatric radiology reviewers was studied using kappa statistics.
Results:
The study group consisted of 20 consecutive pediatric patients (17 males, 3 females) with secondary PVS due to TAPVC repair. Age ranged from 2 months to 8 years (mean, 16.1 months). In children with secondary PVS due to TAPVC repair, the characteristic extravascular thoracic MDCT angiography findings were ground-glass opacity (19/20; 95%), septal thickening (7/20; 35%), pleural thickening (17/20; 85%), and a poorly defined, mildly heterogeneously enhancing, non-calcified soft tissue mass (17/20; 85%) which followed the contours of affected pulmonary veins outside the lung. There was excellent interobserver kappa agreement between two independent reviewers for detecting extravascular abnormalities on thoracic MDCT angiography studies (k = 0.99).
Conclusion:
Our study characterizes the extravascular thoracic MDCT angiography findings in secondary pediatric PVS due to TAPVC repair. In the lungs and pleura, ground-glass opacity, interlobular septal thickening, and pleural thickening are common findings. Importantly, the presence of a mildly heterogeneously enhancing, non-calcified mediastinal soft tissue mass in the distribution of the PVS is a novel characteristic thoracic MDCT angiography finding seen in pediatric secondary PVS due to TAPVC repair.
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