The many faces and outcomes of pulmonary vein stenosis in early childhood

Humpl, Tilman; Fineman, Jeffrey R.; Qureshi, Athar M.

doi:10.1002/ppul.24848

Cited by 12 publications

(17 citation statements)

References 44 publications

(65 reference statements)

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“…Mainly due to its rarity and non-specific clinical presentation, PVS diagnosis is often missed or delayed, resulting in suboptimal patient care. Unfortunately, delayed diagnosis of PVS is associated with increased morbidity and mortality [ 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 ]. Consequently, imaging studies, which can visualize the narrowed pulmonary veins associated with PVS as well as the extravascular findings of PVS, are critical for early and accurate diagnosis [ 11 , 12 , 13 , 14 ].…”

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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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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“…Pulmonary vein stenosis (PVS) is defined as a progressive reduction in luminal caliber of one or more large (extra-pulmonary) pulmonary veins [1][2][3][4]. In adults, PVS can occur as a complication of ablation for atrial fibrillation near the pulmonary veins [5,6].…”

Section: Introductionmentioning

confidence: 99%

“…Pediatric PVS typically arises in infancy, and may be primary or secondary. Primary PVS arises in the absence of identifiable congenital heart disease, and is sometimes associated with prematurity [1][2][3][4]7]. Secondary PVS is more common, occurring after surgical repair of congenital heart disease (e.g., anomalous pulmonary venous connections or cor triatriatum), or may also arise "secondarily" in congenital cardiovascular anomalies that have not been operated upon, especially those with prolonged exposure to left to right shunts [8].…”

Section: Introductionmentioning

confidence: 99%

“…Early and accurate diagnosis of each of these conditions is critical to guiding appropriate treatment and management. To the best of our knowledge, there has been no prior study that directly compares the pleuropulmonary MDCT findings in children with PVS and PLD [1,4,21]. Therefore, the goal of our study was to retrospectively compare the pleuropulmonary MDCT findings in children with PVS and PLD.…”

Section: Introductionmentioning

confidence: 99%

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Pleuropulmonary MDCT Findings: Comparison between Children with Pulmonary Vein Stenosis and Prematurity-Related Lung Disease

et al. 2022

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Purpose: To retrospectively compare the pleuropulmonary MDCT findings in children with pulmonary vein stenosis (PVS) and prematurity-related lung disease (PLD). Materials and Methods: All consecutive infants and young children (≤18 years old) who underwent thoracic MDCT studies from July 2004 to November 2021 were categorized into two groups—children with PVS (Group 1) and children with PLD without PVS (Group 2). Two pediatric radiologists independently evaluated thoracic MDCT studies for the presence of pleuropulmonary abnormalities as follows—(1) in the lung (ground-glass opacity (GGO), triangular/linear plaque-like opacity (TLO), consolidation, nodule, mass, cyst(s), interlobular septal thickening, and fibrosis); (2) in the airway (bronchial wall thickening and bronchiectasis); and (3) in the pleura (thickening, effusion, and pneumothorax). Interobserver agreement between the two reviewers was evaluated with the Kappa statistic. Results: There were a total of 103 pediatric patients (60 males (58.3%) and 43 females (41.7%); mean age, 1.7 years; range, 2 days–7 years). Among these 103 patients, 49 patients (47.6%) comprised Group 1 and the remaining 54 patients (52.4%) comprised Group 2. In Group 1, the observed pleuropulmonary MDCT abnormalities were—pleural thickening (44/49; 90%), GGO (39/49; 80%), septal thickening (39/49; 80%), consolidation (4/49; 8%), and pleural effusion (1/49; 2%). The pleuropulmonary MDCT abnormalities seen in Group 2 were—GGO (45/54; 83%), TLO (43/54; 80%), bronchial wall thickening (33/54; 61%), bronchiectasis (30/54; 56%), cyst(s) (5/54; 9%), pleural thickening (2/54; 4%), and pleural effusion (2/54; 4%). Septal thickening and pleural thickening were significantly more common in pediatric patients with PVS (Group 1) (p < 0.001). TLO, bronchial wall thickening, and bronchiectasis were significantly more frequent in pediatric patients with PLD without PVS (Group 2) (p < 0.001). There was high interobserver kappa agreement between the two independent reviewers for detecting pleuropulmonary abnormalities on thoracic MDCT angiography studies (k = 0.99). Conclusion: Pleuropulmonary abnormalities seen on thoracic MDCT can be helpful for distinguishing PVS from PLD in children. Specifically, the presence of septal thickening and pleural thickening raises the possibility of PVS, whereas the presence of TLO, bronchial wall thickening and bronchiectasis suggests PLD in the pediatric population.

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“…For a comprehensive literature review of PVS, please refer to the several recently published reviews. [1][2][3][4][5][6] Overview Pediatric intraluminal PVS is a disease of pulmonary vein wall thickening. Specifically, hyperplasia (neointimal proliferation) of myofibroblast-like cells in a loose myxocollagenous matrix, which typically originates at the venoatrial junction, can extend distally or upstream into the intrapulmonary veins and lead to lumen obliteration.…”

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confidence: 99%