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

Children ◽  
2021 ◽  
Vol 8 (9) ◽  
pp. 726
Author(s):  
Edward Y. Lee ◽  
Sara O. Vargas ◽  
Kathy J. Jenkins ◽  
Ryan Callahan ◽  
Halley J. Park ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Pulmonary Vein ◽  
Soft Tissue Mass ◽  
Ground Glass Opacity ◽  
Pulmonary Vein Stenosis ◽  
Mdct Angiography ◽  
Tissue Mass ◽  
Pleural Thickening ◽  
Vein Stenosis ◽  
Anomalous Pulmonary Venous Connection

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.

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

Children ◽  
2021 ◽  
Vol 8 (8) ◽  
pp. 667
Author(s):  
Edward Y. Lee ◽  
Ryan Callahan ◽  
Sara O. Vargas ◽  
Kathy J. Jenkins ◽  
Halley J. Park ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Pulmonary Vein ◽  
Pediatric Patients ◽  
Septal Defect ◽  
Soft Tissue Mass ◽  
Pulmonary Vein Stenosis ◽  
Mdct Angiography ◽  
Tissue Mass ◽  
Pleural Thickening ◽  
Vein Stenosis

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.

Congenital Pulmonary Vein Stenosis with Anomalous Pulmonary Venous Connection

2007 ◽  
Vol 15 (5) ◽  
pp. 438-440 ◽  
Author(s):  
Shin Takabayashi ◽  
Hideto Shimpo ◽  
Kazuto Yokoyama ◽  
Masaki Kajimoto ◽  
Koji Onoda
Keyword(s):  
Pulmonary Vein ◽  
Septal Defect ◽  
Superior Vena ◽  
Vena Cava ◽  
Pulmonary Vein Stenosis ◽  
The Right ◽  
Vein Stenosis ◽  
Anomalous Pulmonary Venous Connection ◽  
Right Lower Lobectomy ◽  
Congenital Pulmonary Vein Stenosis

An 11-month-old boy with congenital pulmonary vein stenosis, partial anomalous pulmonary venous connection, and ventricular septal defect is described. Angiocardiography demonstrated stenosis between the right upper pulmonary vein and high superior vena cava and obstruction of the right lower pulmonary vein. For pulmonary vein stenosis, we performed transverse sutured plasty for the right upper pulmonary vein, followed by right lower lobectomy. In some patients, combined management for pulmonary vein stenosis is effective.

A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution

2016 ◽  
Vol 27 (5) ◽  
pp. 870-876 ◽  
Author(s):  
Brian Kogon ◽  
Jan Fernandez ◽  
Subhadra Shashidharan ◽  
Kirk Kanter ◽  
Bahaaldin Alsoufi
Keyword(s):  
Risk Factors ◽  
Cardiopulmonary Bypass ◽  
Pulmonary Vein ◽  
Mixed Type ◽  
Significant Risk ◽  
Pulmonary Vein Stenosis ◽  
Clinical Records ◽  
Vein Stenosis ◽  
Anomalous Pulmonary Venous Connection

AbstractBackgroundPatients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome.MethodsWe reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015.ResultsA total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04).ConclusionThe surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

scholarly journals Complex treatment of recurrent pulmonary vein stenosis

2018 ◽  
pp. 94-96
Author(s):  
Я. Б. Микичак
Keyword(s):  
Pulmonary Vein ◽  
Pulmonary Veins ◽  
Mortality Rates ◽  
Pulmonary Vein Stenosis ◽  
Interventional Treatment ◽  
Initial Surgery ◽  
Aggressive Interventions ◽  
Vein Stenosis ◽  
Anomalous Pulmonary Venous Connection

Pulmonary vein stenosis (PVS) in children is a rare heart disease characterized by high recurrence and mortality rates. Objective. We describe a case of recurrent PVS, which was treated surgically and subsequently with repeated balloon angioplasties. Materials and methods. Echocardiogram showed total anomalous pulmonary venous connection, mixed form. A CT scan of the chest confirmed the diagnosis. Month later after initial surgery angiography detected the presence of stenosis in all pulmonary veins. Results. Currently patient has moderate residual stenosis in each PV. His prognosis is considered bleak due to continuous requirement in aggressive interventions in order at least temporarily resolve pulmonary vein stenosis. Conclusions. Pulmonary vein stenosis still bears high recurrence, reintervention and mortality rates. Rigorous surveillance and combination of surgical and interventional treatment is warranted in this subset of patients to maintain quality of life.

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