Vein Stenosis In Children Research Articles

Balloon angioplasty versus stent placement for the treatment of portal vein stenosis in children: a single center experience.

To evaluate the safety and efficacy of percutaneous interventional treatment of portal vein stenosis in children. A retrospective analysis of all interventional treatments for portal vein stenosis in pediatric patients at a single institution from 2010 to 2021 was conducted. Platelet count, spleen size and portal vein flow velocity were assessed during the follow-up period. Primary and primary assisted patency time were determined. A total of ten children (median age 28.5months, interquartile range (IQR): 2.75-52.5months) with portal vein stenosis after Mesorex-Shunt (n = 4), liver transplantation (n = 3) and other etiologies (n = 3) underwent 15 interventional procedures. There were five reinterventions and one discontinued intervention. The technical success rate was 93.3% (14/15) and clinical success of treated patients was 100% (14/14). Median follow-up was 18months (IQR: 13.5-81months). The median primary patency time for stent placement was 70months (IQR: 13.5-127.25months). For balloon angioplasty, the median primary patency time was 9months (IQR 7.25-11.5months), while the median assisted primary patency time was 14months (IQR: 12 to 15months). Platelet count, spleen size and portal vein flow velocity reliably corresponded to recurrence of portal vein stenosis in asymptomatic patients during follow-up. Interventional treatment is a safe and efficient method to treat portal vein stenosis with long patency times, regardless of etiology. Primary stent placement shows a higher primary patency time than balloon angioplasty. Implementation of stent placement as the primary interventional method may improve patency times and reduce the need for repeat reinterventions in pediatric patients.

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

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.

Pulmonary Vein Stenosis in Children: A Programmatic Approach Employing Primary and Anatomic Therapy.

Pulmonary vein stenosis (PVS) is a difficult condition to treat due to recurrence and progression. In 2017, we developed a comprehensive PVS Program at our center to address the multidisciplinary needs of these patients. We discuss the components of our program and our approach to these patients, using a combination of primary (medical) therapy in addition to anatomic therapy to preserve vessel patency. A multidisciplinary approach to treating these challenging patients is critical.

Thoracic Multidetector Computed Tomography Angiography of Primary Pulmonary Vein Stenosis in Children: Evaluation of Characteristic Extravascular Findings.

The purpose of this study was to investigate the extravascular thoracic multidetector computed tomography (MDCT) angiography findings of pediatric primary pulmonary vein stenosis (PVS) by comparing extravascular thoracic MDCT angiography findings in children with and without PVS. All pediatric patients (age 18 y and below) with a known diagnosis of primary PVS, confirmed by echocardiogram and/or conventional angiography, who underwent thoracic MDCT angiography studies from July 2006 to December 2020 were included. A comparison group, comprised of age-matched and sex-matched pediatric patients without PVS who underwent thoracic MDCT angiography studies during the same study period, was also generated. Two pediatric radiologists independently evaluated thoracic MDCT angiography studies for the presence of extravascular thoracic abnormalities in the lung (ground-glass opacity [GGO], consolidation, pulmonary nodule, mass, cyst, septal thickening, fibrosis, and bronchiectasis), pleura (pleural thickening, pleural effusion and pneumothorax), and mediastinum (lymphadenopathy and mass). When a thoracic abnormality was identified, the location and distribution of the abnormality (in relation to the location of PVS) were also evaluated. Extravascular thoracic MDCT angiography findings of pediatric patients with and without primary PVS were compared. Interobserver agreement between the 2 independent reviewers was evaluated with κ statistics. The study group consisted of 15 thoracic MDCT angiography studies from 15 individual pediatric patients with primary PVS (8 males [53%] and 7 females [47%]; mean age: 10.9 mo; SD: 11.7 mo; range: 1 to 48 mo). The comparison group consisted of 15 thoracic MDCT angiography studies from 15 individual pediatric patients without PVS (8 males [53%] and 7 females [47%]; mean age: 10.2 mo; SD: 11.5 mo; range: 1 to 48 mo). In children with primary PVS, the characteristic extravascular thoracic MDCT angiography findings were GGO (14/15; 93%), septal thickening (5/15; 33%), pleural thickening (14/15; 93%), and ill-defined, mildly heterogeneously enhancing, noncalcified soft tissue mass (14/15; 93%) following the contours of PVS in the mediastinum. There was excellent interobserver κ agreement between 2 independent reviewers for detecting extravascular abnormalities on thoracic MDCT angiography studies (κ=0.99 for the study group and κ=0.98 for the comparison group). Children with primary PVS have characteristic extravascular thoracic MDCT angiography findings. In the lungs and pleura, GGO, septal thickening, and pleural thickening are common findings. Importantly, in the mediastinum, the presence of a mildly heterogeneously enhancing, noncalcified soft tissue mass in the distribution of PVS is a novel characteristic thoracic MDCT angiography finding unique to pediatric primary PVS. When this constellation of extravascular thoracic MDCT angiography findings is detected, although rare, primary PVS should be considered as a possible underlying diagnosis, especially in symptomatic children.

Comparison of Echocardiography and Computed Tomography/Magnetic Resonance Imaging for Assessing of Pulmonary Vein Stenosis in Children

Pulmonary vein (PV) stenosis is a rare disease with high recurrence and mortality rates.
 Objective. This study was aimed to evaluate the diagnostic accuracy of echocardiography versus cross-sectional modalities.
 Material and methods. Our study includes 11 consecutive patients who underwent a comprehensive PV anatomy assessment between April 2018 and June 2019 at Ukrainian Children’s Cardiac Center, Kyiv. Echocardiographic studies of each pulmonary vein followed by computed tomography (CT) or magnetic resonance imaging (MRI) were performed in all patients. A total of 27 CT/MRI studies were performed.
 Results. There were four patients whose echocardiography findings were completely confirmed by CT/MRI method. In some patients significant stenosis was underestimated by echocardiography. Sensitivity and specificity were 86.4 and 88.9 for echo, as well as 100 and 94.4 for CT/MRI, respectively.
 Conclusion. This study recognizes the limitations of echocardiography as an imaging tool for pulmonary veins morphology assessment. Cross-sectional studies overcome these limitations and provide excellent morphological as well as functional (MRI) evaluation of pulmonary veins.

Diagnostic performance of CT angiography to detect pulmonary vein stenosis in children.

To assess the diagnostic efficiency of CT angiography (CTA) to detect pulmonary vein stenosis in children. We retrospectively identify patients between 0 and 3years old with confirmed pulmonary vein stenosis with conventional angiography or surgery and available CTA. Patients without confirmed stenosis of the pulmonary veins were included as controls. We excluded patients with previous surgery involving the pulmonary veins, exclusively right-heart conventional angiography or insufficient data in the operation note to confirm the status of the pulmonary veins. Two pediatric radiologists evaluated and determine the presence of stenosis and the pulmonary veins affected. Disagreement between the readers were solved by consensus with a third reader. A pediatric cardiologist reviewed the available angiographic images to determine the presence of stenosis. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated. Kappa statistics was performed. 26 patients (15 boys, 11 Girls) were included. Conventional angiography (n = 20) and cardiothoracic surgery (n = 6) confirmed the diagnosis of pulmonary vein stenosis in 13 children. The diagnostic performance at patient level showed the sensitivity, specificity, PPV, and NPV were 84.6%, 92.3%, 91.6%, 87.5%, and 88.4%, respectively. The interobserver was k = 0.76. The performance at pulmonary vein level showed the sensitivity, specificity, PPV, and NPV were 63.3%, 97.4%, 90.4%,85.7% and 87.9%, respectively. The interobserver agreement was k = 0.62. Computed Tomography Angiography is an excellent and reliable image technique for ruling in pulmonary vein stenosis in young children.

Intraluminal Pulmonary Vein Stenosis in Children: A "New" Lesion.

Pulmonary vein stenosis (PVS) is a rare disorder that leads to progressive narrowing of the extrapulmonary veins. PVS has been reported in both children and adults and in its worse iteration leads to pulmonary hypertension, right ventricular failure, and death. Multiple etiologies of PVS have been described in children and adults. This review will focus on intraluminal PVS in children. Intraluminal PVS has an estimated incidence ranging from 0.0017% to 0.03%. It is associated with conditions such as prematurity, bronchopulmonary dysplasia, necrotizing enterocolitis, Smith-Lemli-Opitz syndrome, and Down syndrome. Cardiac catheterization and pulmonary vein angiography are the gold standard for diagnosis and anatomic delineation. Other imaging modalities including magnetic resonance imaging, chest tomography, and transesophageal echocardiography are increasingly being used. Mortality of PVS in children is approximately 50%. Predictors of mortality include involvement of ≥3 pulmonary veins, bilateral pulmonary vein involvement, onset of PVS in infancy, elevated pulmonary artery pressure or systolic pulmonary artery-to-aortic pressure ratio, right ventricular dysfunction, restenosis after surgery, distal/upstream disease, and disease progression to previously uninvolved pulmonary veins. Treatment includes catheter-based pulmonary vein dilations with or without stenting, surgical interventions, medical therapy, and in some instances, lung transplantation. Cardiac catheterization for PVS involves a comprehensive hemodynamic and anatomic assessment of the pulmonary veins as well as therapeutic transcatheter interventions. Several surgical strategies have been used. Sutureless repair is currently most commonly used, but patch venoplasty, endarterectomy, ostial resection, and reimplantation are used in select circumstances as well. Medical therapies such as imatinib mesylate and bevacizumab are increasingly being used in an effort to suppress the myofibroblastic proliferation seen in PVS patients. Lung transplantation has been used as an alternative treatment strategy for end-stage, refractory PVS. Nonetheless, despite the different innovative approaches used, morbidity and mortality remain high. At present, the preferred treatment strategy is frequent reassessment of disease progression to guide use of catheter-based and surgical interventions in conjunction with medical therapy.

Proximal pulmonary vein stenosis detection in pediatric patients: value of multiplanar and 3-D VR imaging evaluation

One of the important benefits of using multidetector computed tomography (MDCT) is its capability to generate high-quality two-dimensional (2-D) multiplanar (MPR) and three-dimensional (3-D) images from volumetric and isotropic axial CT data. However, to the best of our knowledge, no results have been published on the potential diagnostic role of multiplanar and 3-D volume-rendered (VR) images in detecting pulmonary vein stenosis, a condition in which MDCT has recently assumed a role as the initial noninvasive imaging modality of choice. The purpose of this study was to compare diagnostic accuracy and interpretation time of axial, multiplanar and 3-D VR images for detection of proximal pulmonary vein stenosis in children, and to assess the potential added diagnostic value of multiplanar and 3-D VR images. We used our hospital information system to identify all consecutive children (< 18 years of age) with proximal pulmonary vein stenosis who had both a thoracic MDCT angiography study and a catheter-based conventional angiography within 2 months from June 2005 to February 2012. Two experienced pediatric radiologists independently reviewed each MDCT study for the presence of proximal pulmonary vein stenosis defined as ≥ 50% of luminal narrowing on axial, multiplanar and 3-D VR images. Final diagnosis was confirmed by angiographic findings. Diagnostic accuracy was compared using the z-test. Confidence level of diagnosis (scale 1-5, 5 = highest), perceived added diagnostic value (scale 1-5, 5 = highest), and interpretation time of multiplanar or 3-D VR images were compared using paired t-tests. Interobserver agreement was measured using the chance-corrected kappa coefficient. The final study population consisted of 28 children (15 boys and 13 girls; mean age: 5.2 months). Diagnostic accuracy based on 116 individual pulmonary veins for detection of proximal pulmonary vein stenosis was 72.4% (84 of 116) for axial MDCT images, 77.5% (90 of 116 cases) for multiplanar MDCT images, and 93% (108 of 116 cases) for 3-D VR images with significantly higher accuracy with 3-D VR compared to axial (z = 4.17, P < 0.001) and multiplanar (z = 3.34, P < 0.001) images. Confidence levels for detection of proximal pulmonary vein stenosis were significantly higher with 3-D VR images (mean level: 4.6) compared to axial MDCT images (mean level: 1.7) and multiplanar MDCT images (mean level: 2.0) (paired t-tests, P < 0.001). Thus, 3-D VR images (mean added diagnostic value: 4.7) were found to provide added diagnostic value for detecting proximal pulmonary vein stenosis (paired t-test, P < 0.001); however, multiplanar MDCT images did not provide added value (paired t-test, P = 0.89). Interpretation time was significantly longer and interobserver agreement was higher when using 3-D VR images than using axial MDCT images or MPR MDCT images for diagnosing proximal pulmonary vein stenosis (paired t-tests, P < 0.001). Use of 3-D VR images in the diagnosis of proximal pulmonary vein stenosis in children significantly increases accuracy, confidence level, added diagnostic value and interobserver agreement. Thus, the routine use of this technique should be encouraged despite its increased interpretation time.

Circulation: Cardiovascular Interventions Editors’ Picks

<i>Circulation: Cardiovascular Interventions</i> Editors’ Picks

Outcomes After Stent Implantation for the Treatment of Congenital and Postoperative Pulmonary Vein Stenosis in Children

Pulmonary vein stenosis (PVS) is a rare condition that can lead to worsening pulmonary hypertension and cardiac failure in children, and it is frequently lethal. Surgical and transcatheter approaches are acutely successful but restenosis is common and rapid. We reviewed outcomes among patients who underwent transcatheter pulmonary vein stent implantation for congenital or postoperative PVS at <18 years of age. A total of 74 pulmonary veins were stented with bare metal, drug-eluting, or covered stents in 47 patients. Primary diagnoses included PVS associated with anomalous venous return in 51%, PVS associated with other congenital cardiovascular defects in 36%, and congenital ("de novo") PVS in 13% of patients. Median age at the time of pulmonary vein stent implantation was 1.4 years. During a median cross-sectional follow-up of 3.1 years, 21 patients died. Estimated survival was 62±8% at 1 year and 50±8% at 5 years after pulmonary vein stent implantation. Stent placement acutely relieved focal obstruction in all veins. Of the 54 stents reexamined with catheterization, 32 underwent reintervention. Freedom from reintervention was 62±7% at 6 months and 42±7% at 1 year. Stent occlusion was documented in 9 cases and significant in-stent stenosis in 17 cases. Stent implantation diameter ≥7 mm was associated with longer freedom from reintervention (hazard ratio, 0.32; P=0.015) and from significant in-stent stenosis (hazard ratio, 0.14; P=0.002). Major acute complications occurred in 5 cases. Transcatheter stent implantation can acutely relieve PVS in children, but reintervention is common. Larger stent lumen size at implantation is associated with longer stent patency and a lower risk of reintervention.

Non-invasive assessment of congenital pulmonary vein stenosis in children using cardiac-non-gated CT with 64-slice technology

Management of congenital pulmonary vein stenosis is a diagnostic challenge. Echocardiography may be insufficient and thus cardiac catheterization remains the reference standard in this setting. The aim of the study was to investigate the accuracy of cardiac-non-gated CT using 64-slice technology in detecting congenital pulmonary vein stenosis in children.

Non-invasive assessment of congenital pulmonary vein stenosis in children using cardiac-non-gated CT with 64-slice technology

Background Management of congenital pulmonary vein stenosis is a diagnostic challenge. Echocardiography may be insufficient and thus cardiac catheterization remains the reference standard in this setting. The aim of the study was to investigate the accuracy of cardiac-non-gated CT using 64-slice technology in detecting congenital pulmonary vein stenosis in children. Materials and methods CT examinations were consecutively performed from May 2005 to December 2006 in 13 children aged 1.5–12 months (median 5 months) for suspected congenital pulmonary vein stenosis. Cardiac-non-gated CT acquisitions were performed after the peripheral injection of contrast agent. Pulmonary veins were evaluated for their pattern of connectivity from the lung to the left atrium and for the presence of stenosis. CT findings of pulmonary vein stenosis were compared with combined findings available from echocardiography, catheterization and surgery. Results Pulmonary veins from the right lung ( n = 29) and left lung ( n = 26) were evaluated as separate structures ( N = 55). Of the 55 structures, 32 had surgical and/or catheterization data and 45 had echocardiography for comparison. CT visualized 100% (55/55) of the investigated structures, while echocardiography visualized 82% (45/55). In the 13 subjects CT identified 10 stenotic pulmonary veins. CT confirmed the echocardiography suspicion of pulmonary vein stenosis in 100% (7/7) and established a new diagnosis in 3 other patients. CT agreed with surgery/catheterization in 100% (10/10) of the available comparisons. Conclusion Cardiac-non-gated CT assessed the pulmonary veins more completely than echocardiography and should be considered as a viable alternative for invasive pulmonary venography for detecting pulmonary vein stenosis in children.

Angioplasty treatment of portal vein stenosis in children with segmental liver transplants: mid-term results.

Percutaneous venoplasty has showed excellent technical success and excellent early results in treating portal vein stenoses in children with reduced-size liver transplants. We review the mid-term results in 22 children in whom portal venoplasty was attempted. During 27 months, percutaneous transhepatic portal venoplasty was attempted in 22 children with portal vein stenoses. Patients presented with symptoms of portal vein hypertension or were identified by routine surveillance with posttransplantation Doppler sonography. All stenoses were verified with angiography. Venoplasty was performed by direct puncture of an intrahepatic portal vein before balloon angioplasty of the stenotic segment. Patients were followed up with sonographic surveillance, and reintervention was performed as needed. In patients who had suboptimal results after portal venoplasty or who developed recurrent stenoses, intravascular stents were placed across stenoses. In 16 of 22 patients, initial clinical and technical success was achieved. In the six patients who underwent unsuccessful procedures, complete occlusion of the portal vein precluded access to the extrahepatic portal vein. Of the 16 patients who underwent successful procedures, intravascular stents were placed at the time of initial venoplasty in five patients for elastic stenosis. In seven other patients, portal vein restenosis occurred after venoplasty (mean, 6.3 months), necessitating intravascular stent placement. Four patients who underwent successful venoplasty without stent placement have required no further intervention. Portal vein patency has been maintained in all 16 patients who underwent technically successful procedures for 4-29 months (mean, 20 months). In children with reduced-size liver transplants who experience delayed portal vein stenosis, percutaneous transhepatic portal venoplasty has achieved encouraging mid-term results. It is the procedure of choice in our hospital and often eliminates the need for surgical revision, portacaval shunting, or retransplantation.