Primary pulmonary vein stenosis during infancy: state of the art review

Frank, David B.; Levy, Paul C.; Stiver, Corey; Boe, Brian A.; Baird, Christopher W.; Callahan, Ryan; Smith, Charles V.; Vanderlaan, Rachel D.; Backes, Carl H.

doi:10.1038/s41372-021-01008-7

Cited by 23 publications

(20 citation statements)

References 78 publications

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“…Pulmonary vein stenosis (PVS) is a rare and often challenging diagnosis, with an unfavorable prognosis, particularly in the pediatric population [1][2][3][4][5]. Characterized by a progressive reduction in the luminal size of one or more pulmonary veins, PVS presents with non-specific clinical signs and symptoms, such as failure to thrive, feeding intolerance, and shortness of breath, due to elevated pulmonary venous pressure [1,2,4].…”

Section: Introductionmentioning

confidence: 99%

Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect

et al. 2021

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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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Section: Introductionmentioning

confidence: 99%

Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect

et al. 2021

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“…Pulmonary vein stenosis (PVS) is caused by a decrease in luminal caliber of one or more of the extra-pulmonary veins. PVS is mainly classified into two types: primary and secondary [ 1 , 2 ]. While primary PVS occurs in the absence of previous intervention, secondary PVS is known to develop following total anomalous pulmonary venous connection (TAPVC) repair with an incidence of 11–17% [ 1 , 2 , 3 , 4 , 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

Secondary Pulmonary Vein Stenosis Due to Total Anomalous Pulmonary Venous Connection Repair in Children: Extravascular MDCT Findings

et al. 2021

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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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“…Pulmonary vein stenosis (PVS) remains one of the most challenging problems in pediatric heart disease. It is well known that effective management of PVS requires an interdisciplinary team in order to implement multimodal therapy [1]. While medical and transcatheter therapies represent two cornerstones of treatment the surgical repair of PVS remains a critical component.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Vein Stenosis—Evolving Surgical Management of a Challenging Disease

2021

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Pulmonary vein stenosis (PVS) is an extremely challenging clinical problem in congenital heart disease. It has traditionally required multimodal therapy given its complex underlying pathophysiology. As with other modalities, surgical therapy has undergone tremendous evolution since the 1950s. These evolving strategies have been based upon an improved understanding of the substrates that cause PVS and recurrent vein obstruction. More recent anatomic-based surgical strategies have focused on the pulmonary vein course, and how adjacent mediastinal structures can create a fulcrum effect on the pulmonary veins as they pass from the lung parenchyma to the left atrium. The consequent angulation of pulmonary veins creates altered wall shear stress and likely serves as a nidus for recurrent PVS. Encouraging early results suggest that eliminating pulmonary vein angulation and shortening/straightening the pulmonary vein course may prove effective in surgically managing PVS.

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Primary pulmonary vein stenosis during infancy: state of the art review

Cited by 23 publications

References 78 publications

Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect

Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect

Secondary Pulmonary Vein Stenosis Due to Total Anomalous Pulmonary Venous Connection Repair in Children: Extravascular MDCT Findings

Pulmonary Vein Stenosis—Evolving Surgical Management of a Challenging Disease

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