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Purpose: To retrospectively compare the lung and pleural findings in children with pulmonary vein stenosis (PVS) with and without aspiration on multidetector computed tomography (MDCT). Materials and Methods: All consecutive children (≤18 years old) with PVS who underwent thoracic MDCT studies from August 2004 to December 2021 were categorized into two groups: children with PVS with aspiration (Group 1) and children with PVS without aspiration (Group 2). Two independent pediatric radiologists retrospectively evaluated thoracic MDCT studies for the presence of lung and pleural abnormalities as follows: (1) in the lung (ground-glass opacity (GGO), consolidation, nodule, mass, cyst(s), interlobular septal thickening, and fibrosis) and (2) in the pleura (thickening, effusion, and pneumothorax). Interobserver agreement between the two reviewers was evaluated by the proportion of agreement and the Kappa statistic. Results: The final study population consisted of 64 pediatric patients (36 males (56.3%) and 43 females (43.7%); mean age, 1.7 years; range, 1 day–17 years). Among these 64 patients, 19 patients (29.7%) comprised Group 1 and the remaining 45 patients (70.3%) comprised Group 2. In Group 1 (children with PVS with aspiration), the detected lung and pleural MDCT abnormalities were: GGO (17/19; 89.5%), pleural thickening (17/19; 89.5%), consolidation (16/19; 84.5%), and septal thickening (16/19; 84.5%). The lung and pleural MDCT abnormalities observed in Group 2 (children with PVS without aspiration) were: GGO (37/45; 82.2%), pleural thickening (37/45; 82.2%), septal thickening (36/45; 80%), consolidation (3/45; 6.7%), pleural effusion (1/45; 2.2%), pneumothorax (1/45; 2.2%), and cyst(s) (1/45; 2.2%). Consolidation was significantly more common in pediatric patients with both PVS and aspiration (Group 1) (p < 0.001). There was high interobserver agreement between the two independent reviewers for detecting lung and pleural abnormalities on thoracic MDCT studies (Kappa = 0.98; CI = 0.958, 0.992). Conclusion: Aspiration is common in pediatric patients with PVS who undergo MDCT and was present in nearly 30% of all children with PVS during our study period. Consolidation is not a typical radiologic finding of PVS in children without clinical evidence of aspiration. When consolidation is present on thoracic MDCT studies in pediatric patients with PVS, the additional diagnosis of concomitant aspiration should be considered. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Pulmonary vein stenosis is a serious condition characterized by restriction or blockage due to fibrotic tissue ingrowth that develops in the pulmonary veins of infants or children. It is often progressive and can lead to severe pulmonary hypertension and death. Efforts to halt or reverse disease progression include surgery and catheter-based balloon dilation and stent implantation. Its cause and mechanism of progression are unknown. In this pilot study, we propose and explore the hypothesis that elevated wall shear stress at discrete pulmonary venous sites triggers stenosis. To assess this theory, we retrospectively analyzed cardiac catheterization, lung scan, and X-ray computed tomography data to estimate wall shear stress in the pulmonary veins at multiple time points during disease progression in two patients. Results are consistent with the existence of a level of elevated wall shear stress above which the disease is progressive and below which progression is halted. The analysis also suggests the possibility of predicting the target lumen size necessary in a given vein to reduce wall shear stress to normal levels and remove the trigger for stenosis progression [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

A nurse practitioner’s experience in managing children with intraluminal pulmonary vein stenosis. A case study of a 3-year-old patient with multi–vessel intraluminal pulmonary vein stenosis. [ABSTRACT FROM AUTHOR]

Pulmonary vein stenosis (PVS) is a rare, but high mortality and resource intensive disease caused by mechanical obstruction or intraluminal myofibroproliferation, which can be post-surgical or idiopathic. There are increasing options for management including medications, cardiac catheterization procedures, and surgery. We queried the International Quality Improvement Collaborative for Congenital Heart Disease (IQIC) database for cases of PVS and described the cohort including additional congenital lesions and surgeries as well as infectious and mortality outcomes. IQIC is a quality improvement project in low-middle-income countries with the goal of reducing mortality after congenital heart surgery. Three cases were described in detail with relevant images. We identified 57 cases of PVS surgery, with similar mortality to higher income countries. PVS should be recognized as a global disease. More research and collaboration are needed to understand the disease, treatments, and outcomes, and to devise treatment approaches for low resource environments. [ABSTRACT FROM AUTHOR]

Pulmonary vein stenosis (PVS) is a rare, frequently lethal disease with heterogeneous phenotypes and an unclear etiology. Limited studies have reported associations between PVS and congenital heart disease (CHD), chronic lung disease (CLD), and/or prematurity; however, to date, there have been no studies that report detailed clinical syndromic phenotypes and the potential role of genetics in PVS. An existing registry of multivessel PVS patients seen at Boston Children's Hospital (BCH) was queried between August 2006 and January 2017 for all existing genetic testing data on these patients. PVS was defined as an intraluminal pulmonary venous obstruction in ≥2 vessels with mean pressure gradients > 4 mmHg. One-hundred-and-fifty-seven patients (46% female, with a median age at PVS diagnosis of 3 months) formed the cohort. Seventy-one (45%) patients had available genetic testing information. Of the 71 patients, a likely genetic diagnosis was found in 23 (32%) patients: 13 (57%) were diagnosed with Trisomy 21 (T21), five (22%) with Smith-Lemli-Opitz Syndrome, five (22%) had other pathologic genetic disease, and 24 (33%) had variants of unknown significance. The majority of 13 patients with T21 and PVS had common atrioventricular canal (CAVC) (10, 77%) and all had severe pulmonary hypertension (PHTN), which led to their PVS diagnosis. In our study, PVS was associated with T21, the majority of whom also had CAVC and PHTN. Therefore, complete assessment of the pulmonary veins should be considered for all T21 patients, especially those with CAVC presenting with PHTN. Furthermore, prospective standardized genetic testing with detailed clinical phenotyping may prove informative about potential genetic etiologies of PVS. [ABSTRACT FROM AUTHOR]

We conducted a study to determine whether patients born with Trisomy 21 and left-to-right shunts who develop pulmonary vein stenosis (PVS) have a longer exposure to shunt physiology compared to those who do not develop PVS. We included patients seen at Boston Children's Hospital between 15 August 2006 and 31 August 2017 born with Trisomy 21 and left-to-right shunts who developed PVS within 24 months of age. We conducted a retrospective 3:1 matched case-control study. The primary predictor was length of exposure to shunt as defined as date of birth to the first echocardiogram showing mild or no shunt. Case patients with PVS were more likely to have a longer exposure to shunt than patients in the control group (6 vs. 3 months, p-value 0.002). Additionally, PVS patients were also more likely to have their initial repair ≥ 4 months of age (81% vs. 42%, p-value 0.003) and have a gestational age ≤ 35 weeks (48% vs. 13%, p-value 0.003). Time exposed to shunts may be an important modifiable risk factor for PVS in patients with Trisomy 21. [ABSTRACT FROM AUTHOR]

Pulmonary vein stenosis is a rare progressive narrowing of the extrapulmonary pulmonary veins, presenting predominantly in infancy and virtually always lethal. It typically arises following repair of congenital heart disease, particularly anomalous pulmonary venous return. Histologic characterization of pediatric pulmonary vein stenosis, not previously well described, may provide insight into the disease pathobiology. We retrieved archival lung specimens (biopsy, explant, or autopsy) from patients with pediatric pulmonary vein stenosis. Medical records were reviewed. Microscopic examination included hematoxylin and eosin (H&E)-stained slides, and for a subset of patients, elastic, trichrome, smooth-muscle actin, and D2-40. Groups with different clinical disease features were compared using Fisher's exact test. A total of 33 patients (median age, 7 months) had available tissue and 52% had congenital heart disease; 18% were premature. Within the lungs, interlobular septal veins showed thickened muscular coats (in 58%), proliferation/tortuosity (in 6%), and fibromyxoid intimal proliferation (in 3%). Associated arterial hypertensive changes were seen in 30 (91%). The one patient with intrapulmonary venous fibromyxoid intimal proliferation was the only patient with apparent primary familial disease. Lymphangiectasia and arterial medial hypertrophy were histologic features that correlated with clinical grouping. We conclude that in pediatric pulmonary vein stenosis, intrapulmonary pulmonary veins commonly show muscular thickening, best interpreted as venous hypertensive remodeling. Fibromyxoid intimal proliferation resembling that of the extrapulmonary pulmonary veins is uncommon. Awareness of intrapulmonary features in various clinical subtypes of pulmonary vein stenosis may be diagnostically and therapeutically informative considering that current catheter-based and surgical therapy is directed at the extrapulmonary component of pulmonary vein stenosis. [ABSTRACT FROM AUTHOR]

Objective We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless surgical repair, catheter interventions, and adjunctive chemotherapy. We report our outcomes after surgery. Methods Between January 2007 and August 2013, 49 patients with multivessel pulmonary vein stenosis underwent operations at our institution. We retrospectively reviewed data from a pulmonary vein stenosis registry and the medical records. Results At the time of the index operation, the median patient age was 6 months (range, 32 days-48 months) and weight was 4.9 kg (range, 2.1-13.4 kg). Fourteen patients (28%) died during the follow-up period (median follow-up was 0.5 years [range, 0.04-4.9 years]). There were 2 deaths (4%) within 30 days. Age at repair <6 months, weight at repair <3 kg, and a preoperative right ventricular systolic pressure < ¾ systemic were found to be associated with mortality. One patient required repeat operation for recurrent stenosis. Thirty-nine patients (80%) underwent postoperative catheterizations. The median number of catheterizations per patient was 2 (range, 0-14). Twenty-nine patients (59%) underwent catheterizations with pulmonary vein intervention. The median number of catheterizations with intervention per patient was 1 (range, 0-14). There were no identifiable associations with need for or number of catheterizations with intervention. Ten patients were listed for lung transplantation: 4 patients were de-listed, 3 patients died waiting, and 3 patients underwent transplant. Conclusions Using a multimodality approach, we observed acceptable early survival after operation in patients with pulmonary vein stenosis, despite the need for catheter reinterventions. Lung transplantation remains a viable option. [ABSTRACT FROM AUTHOR]

The study objective was to evaluate outcomes of pulmonary vein stenosis repair in a large single-center cohort. Clinical data from a pulmonary vein stenosis registry were retrospectively reviewed identifying patients who underwent pulmonary vein stenosis repair. The primary/index operation was defined as the patient's first pulmonary vein stenosis operation during the study period. Between January 2007 and August 2019, 174 patients underwent pulmonary vein stenosis repair. Bilateral pulmonary vein stenosis occurred in 111 patients (64%); 71 patients (41%) had 4-vessel disease. Fifty-nine patients (34%) had primary pulmonary vein stenosis. Median age was 9 months (interquartile range, 5-27) and weight was 6.5 kg (4.7-10.2). Surgical techniques evolved and included ostial resection, unroofing, reimplantation, sutureless, modified sutureless, and a newer anatomically focused approach of pulmonary vein stenosis resection with lateralization or patch enlargement of the pulmonary vein–left atrium connection. Twenty-three patients (13%) required reoperation. Cumulative 2-year incidence of postoperative transcatheter intervention (balloon dilation ± stenting) was 64%. One-, 2-, and 5-year survivals were 71.2%, 66.8%, and 60.6%, respectively. There was no association between surgery type and reoperation rate (hazard ratio, 2.38, P =. 25) or transcatheter intervention (hazard ratio, 0.97, P =. 95). The anatomically focused repair was associated with decreased mortality on univariate (hazard ratio, 0.38, P =. 042) and multivariable analyses (hazard ratio, 0.19, P =. 014). Antiproliferative chemotherapy was also associated with decreased mortality (hazard ratio, 0.47, P =. 026). This large single-center surgical pulmonary vein stenosis experience demonstrates encouraging midterm results. A new anatomically focused repair strategy aims to alleviate pulmonary vein angulation to minimize turbulence and shows promising early outcomes. Continued follow-up is required to understand longer-term outcomes for this surgical approach. The PVs can take a long, angulated course as they pass from the lung parenchyma to the LA. This angulated course appears to occur due to the fulcrum effect of structures adjacent to the PVs (ie, descending aorta, airway, pulmonary artery, and pericardial reflection). The anatomically focused repair strategy aims to target this long, angulated PV course, which may be the nidus for turbulent blood flow and subsequent PVS. This repair involves extensive takedown of the pericardial reflection, resection of stenosing/scar tissue, and lateralizing/patch augmenting the PV–LA connection with the goal of creating a shorter and straighter pulmonary venous course. Early follow-up suggests improved survival with the anatomically focused repair strategy compared with conventional repair techniques (ie, sutureless and modified sutureless repair). [Display omitted] [ABSTRACT FROM AUTHOR]

Abstract: Background: Primary pulmonary vein stenosis (PVS) is a progressive disorder of infants. Although catheter based intervention and chemotherapy are used to manage the disorder, the benefit of these approaches is reduced considerably by restenosis. The nature of the intimal cells causing the occlusive lesions in PVS is poorly understood. Methods: Seven PVS cases were studied with antibodies for smooth muscle actin (SMA), muscle-specific actin (MSA), monoclonal desmin, S100 protein, CD31, CD34, CD45RO, CD68, CD99, Ki-67 (MIB-I), and with antibodies directed against several receptor tyrosine kinases (RTK), including platelet-derived growth factor alpha and beta receptor (PDGFR-α and -β), epidermal growth factor receptor (EGFR), fibroblast growth factor receptor (FGFR), vascular endothelial growth factor 1 and 2 receptor (VEGFR), and stem cell factor receptor (c-kit). Results: Lesional cells stained strongly and diffusely with SMA and MSA, but not for macrophage, lymphocyte, endothelial markers, or for Ki-67. RTK expression was strong and diffuse for PDGFR-α and -β, FGFR, and VEGFR-2. Lesional cells stained for VEGF and PDGF β receptor was phosphorylated. Conclusions: The histologic appearance, and the strong diffuse immunoreactivity for smooth muscle markers, indicates that the intimal lesional cells are myofibroblast-like. Expression of various receptor tyrosine kinases and some ligands suggests an autocrine or paracrine role of these proteins in the pathogenesis of the intimal occlusive lesion in PVS. [Copyright &y& Elsevier]