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Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and...
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Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management

A special issue of Children (ISSN 2227-9067).

Deadline for manuscript submissions: closed (30 April 2022) | Viewed by 38991

Special Issue Editor

Prof. Dr. Kathy J. Jenkins

E-Mail Website
Guest Editor
Department of Cardiology, Boston Children's Hospital and Harvard Medical School, 300 Longwood Ave, Boston, MA, 02115, USA
Interests: pulmonary vein stenosis; Pediatrics

Special Issue Information

Dear Colleagues,

Progressive obliteration of the pulmonary vein lumen, known as pulmonary vein stenosis (PVS), is a rare condition that typically occurs in infants and very young children. PVS can be present at birth, but more commonly develops later in association with congenital heart disease or pulmonary conditions, especially in premature infants, and rarely in isolation. Unlike other congenital heart defects, a hallmark feature of the disease is the rapid recurrence of obstruction even after successful anatomic intervention. When PVS develops in multiple vessels, it rapidly leads to pulmonary edema, pulmonary hypertension, right heart failure, and infant death.

We now know that lumen obliteration occurs from overactivity of myofibroblast-like cells in the neointima of the pulmonary veins, though the inciting etiology is not known. Much progress has been made over the past decade to understand the biology of PVS, refine surgical and catheter-based treatment, reduce cellular activity, and improve survival, towards the elusive goal of PVS prevention.

This Special Issue focuses on the underlying biology that leads to PVS, a deeper understanding of how vessels are affected including pathophysiology and prognosis, diagnostic and treatment strategies, and long-term consequences for the new population of survivors. We welcome original articles and reviews that explore these issues from basic science, clinical, or epidemiologic perspectives. Exploratory papers with leading ideas about causal mechanisms are encouraged.

Codifying what is currently known about PVS, etiology, and treatment is an important step towards improving survival for the growing population of infants affected by this devastating disease.

Prof. Dr. Kathy J. Jenkins
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • pulmonary vein stenosis
  • myofibroblast
  • congenital heart defect
  • prematurity
  • infant

Published Papers (17 papers)

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Editorial

Jump to: Research, Review, Other

4 pages, 148 KiB  
Editorial
The Brightest Rainbow Follows the Darkest Storm
by Douglas Lake
Children 2020, 7(11), 223; https://0-doi-org.brum.beds.ac.uk/10.3390/children7110223 - 10 Nov 2020
Viewed by 1882
Abstract
A parent’s perspective on pulmonary vein stenosis through the experience of two children with the disease. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)

Research

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11 pages, 5227 KiB  
Article
Lung and Pleural Findings of Children with Pulmonary Vein Stenosis with and without Aspiration: MDCT Evaluation
by Abbey J. Winant, Ryan Callahan, Sara O. Vargas, Kathy J. Jenkins, Vanessa Rameh, Patrick R. Johnston, Maria Niccum, Mirjam L. Keochakian and Edward Y. Lee
Children 2022, 9(4), 543; https://0-doi-org.brum.beds.ac.uk/10.3390/children9040543 - 12 Apr 2022
Viewed by 1564
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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11 pages, 4613 KiB  
Article
Pleuropulmonary MDCT Findings: Comparison between Children with Pulmonary Vein Stenosis and Prematurity-Related Lung Disease
by Abbey J. Winant, Sara O. Vargas, Kathy J. Jenkins, Ryan Callahan, Vanessa Rameh, Katie A. Krone, Patrick R. Johnston, Mirjam L. Keochakian and Edward Y. Lee
Children 2022, 9(3), 355; https://0-doi-org.brum.beds.ac.uk/10.3390/children9030355 - 04 Mar 2022
Cited by 1 | Viewed by 1747
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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11 pages, 442 KiB  
Article
Aspiration Is Associated with Poor Treatment Response in Pediatric Pulmonary Vein Stenosis
by Maria Niccum, Ryan Callahan, Kimberlee Gauvreau and Kathy J. Jenkins
Children 2021, 8(9), 783; https://0-doi-org.brum.beds.ac.uk/10.3390/children8090783 - 07 Sep 2021
Cited by 4 | Viewed by 1619
Abstract
Intraluminal pulmonary vein stenosis is a disease with significant morbidity and mortality, though recent progress has been made using multimodal therapy with antiproliferative agents. The aim of this study was to evaluate the association between aspiration and poor treatment response in patients with [...] Read more.
Intraluminal pulmonary vein stenosis is a disease with significant morbidity and mortality, though recent progress has been made using multimodal therapy with antiproliferative agents. The aim of this study was to evaluate the association between aspiration and poor treatment response in patients with intraluminal pulmonary vein stenosis. A retrospective, single-center cohort analysis was performed of patients treated with a combination of imatinib mesylate and multimodal anatomic relief between March 2009 and November 2019. Analysis focused on 2-ventricle patients due to small numbers and clinical heterogeneity of single ventricle patients. Among the 84 patients included, 15 had single ventricle physiology and 69 had 2-ventricle physiology. Among the 2-ventricle group, multivariable analysis revealed that patients with clinical aspiration had nearly five times higher odds of poor treatment response than patients without aspiration (OR 4.85, 95% CI [1.37, 17.2], p = 0.014). Furthermore, male patients had higher odds of poor treatment response than their female counterparts (OR 3.67, 95% CI [1.04, 12.9], p = 0.043). Aspiration is a novel, potentially modifiable risk factor for poor treatment response in pediatric multi-vessel intraluminal pulmonary vein stenosis in patients with 2-ventricle physiology. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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13 pages, 8616 KiB  
Article
Secondary Pulmonary Vein Stenosis Due to Total Anomalous Pulmonary Venous Connection Repair in Children: Extravascular MDCT Findings
by Edward Y. Lee, Sara O. Vargas, Kathy J. Jenkins, Ryan Callahan, Halley J. Park, Zachary Gauthier and Abbey J. Winant
Children 2021, 8(9), 726; https://0-doi-org.brum.beds.ac.uk/10.3390/children8090726 - 25 Aug 2021
Cited by 2 | Viewed by 1915
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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12 pages, 4719 KiB  
Article
Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect
by Edward Y. Lee, Ryan Callahan, Sara O. Vargas, Kathy J. Jenkins, Halley J. Park, Zachary Gauthier and Abbey J. Winant
Children 2021, 8(8), 667; https://0-doi-org.brum.beds.ac.uk/10.3390/children8080667 - 30 Jul 2021
Cited by 3 | Viewed by 1695
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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21 pages, 14776 KiB  
Article
Prognostic Significance of Computed Tomography Findings in Pulmonary Vein Stenosis
by Laureen Sena, Ryan Callahan, Lynn A. Sleeper and Rebecca S. Beroukhim
Children 2021, 8(5), 402; https://0-doi-org.brum.beds.ac.uk/10.3390/children8050402 - 17 May 2021
Cited by 6 | Viewed by 2166
Abstract
(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and [...] Read more.
(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and lower; and left upper and lower. Severity of vein stenosis (0–4 = no disease–atresia) and lung segments (0–3 = unaffected–severe) were scored. A PVS severity score (sum of all veins + 2 if bilateral disease; maximum = 22) and a total lung severity score (sum of all lung segments; maximum = 15) were reported; (3) Of 43 CT examinations (median age 21 months), 63% had bilateral disease. There was 30% mortality by 4 years after CT. Individual-vein PVS severity was associated with its corresponding lung segment severity (p < 0.001). By univariate analysis, PVS severity score >11, lung cysts, and total lung severity score >6 had higher hazard of death; and perihilar induration had lower hazard of death; (4) Multiple CT-derived variables of PVS severity and lung disease have prognostic significance. PVS severity correlates with lung disease severity. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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12 pages, 1320 KiB  
Article
Outcomes in Establishing Individual Vessel Patency for Pediatric Pulmonary Vein Stenosis
by Ryan Callahan, Kimberlee Gauvreau, Audrey C. Marshall, Laureen M. Sena, Christopher W. Baird, Christina M. Ireland, Kerry McEnaney, Elsa C. Bjornlund, Juliana T. Mendonca and Kathy J. Jenkins
Children 2021, 8(3), 210; https://0-doi-org.brum.beds.ac.uk/10.3390/children8030210 - 10 Mar 2021
Cited by 7 | Viewed by 2018
Abstract
The purpose of this study was to determine what patient and pulmonary vein characteristics at the diagnosis of intraluminal pulmonary vein stenosis (PVS) are predictive of individual vein outcomes. A retrospective, single-center, cohort sub-analysis of individual pulmonary veins of patients enrolled in the [...] Read more.
The purpose of this study was to determine what patient and pulmonary vein characteristics at the diagnosis of intraluminal pulmonary vein stenosis (PVS) are predictive of individual vein outcomes. A retrospective, single-center, cohort sub-analysis of individual pulmonary veins of patients enrolled in the clinical trial NCT00891527 using imatinib mesylate +/− bevacizumab as adjunct therapy for the treatment of multi-vessel pediatric PVS between March 2009 and December 2014 was performed. The 72-week outcomes of the individual veins are reported. Among the 48 enrolled patients, 46 patients and 182 pulmonary veins were included in the study. Multivariable analysis demonstrated that patients with veins without distal disease at baseline (odds ratio, OR 3.69, 95% confidence interval, CI [1.52, 8.94], p = 0.004), location other than left upper vein (OR 2.58, 95% CI [1.07, 6.19], p = 0.034), or veins in patients ≥ 1 y/o (OR 5.59, 95% CI [1.81, 17.3], p = 0.003) were at higher odds of having minimal disease at the end of the study. Veins in patients who received a higher percentage of eligible drug doses required fewer reinterventions (IRR 0.76, 95% CI [0.68, 0.85], p < 0.001). The success of a multi-modal treatment approach to aggressive PVS depends on the vein location, disease severity, and drug dose intensity. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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9 pages, 1308 KiB  
Article
Pulmonary Vein Stenosis: A Rare Disease with a Global Reach
by Jennifer Schramm, Sivakumar Sivalingam, Guillermo E. Moreno, Dinh Quang Le Thanh, Kimberlee Gauvreau, Kaitlin Doherty-Schmeck and Kathy J. Jenkins
Children 2021, 8(3), 198; https://0-doi-org.brum.beds.ac.uk/10.3390/children8030198 - 06 Mar 2021
Cited by 3 | Viewed by 2387
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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12 pages, 3359 KiB  
Article
Correlation of Intravascular Ultrasound with Histology in Pediatric Pulmonary Vein Stenosis
by Ryan Callahan, Zachary Gauthier, Shuhei Toba, Stephen P. Sanders, Diego Porras and Sara O. Vargas
Children 2021, 8(3), 193; https://0-doi-org.brum.beds.ac.uk/10.3390/children8030193 - 04 Mar 2021
Cited by 6 | Viewed by 1754
Abstract
Preliminary intravascular ultrasound (IVUS) images of suspected pediatric intraluminal pulmonary vein stenosis (PVS) demonstrate wall thickening. It is unclear how the IVUS-delineated constituents of wall thickening correlate with the histology. We analyzed six postmortem formalin-fixed heart/lung specimens and four live patients with PVS [...] Read more.
Preliminary intravascular ultrasound (IVUS) images of suspected pediatric intraluminal pulmonary vein stenosis (PVS) demonstrate wall thickening. It is unclear how the IVUS-delineated constituents of wall thickening correlate with the histology. We analyzed six postmortem formalin-fixed heart/lung specimens and four live patients with PVS as well as control pulmonary veins using IVUS and light microscopic examination. In PVS veins, IVUS demonstrated wall thickening with up to two layers of variable echogenicity, often with indistinct borders. Histologically, the veins showed fibroblastic proliferation with areas rich in myxoid matrix as well as areas with abundant collagen and elastic fibers. Discrete vein layers were obscured by scarring and elastic degeneration. A lower reflective periluminal layer by IVUS corresponded with hyperplasia of myofibroblast-like cells in abundant myxoid matrix. The hyper-reflective layer by IVUS extended to the outer edge of the vessel and corresponded to a less myxoid area with more collagen, smooth muscle and elastic fibers. The outer less reflective edge of the IVUS image correlated with a gradual transition into adventitia. Normal veins had a thin wall, correlating with histologically normal cellular and extracellular components, without intimal proliferation. IVUS may provide further understanding of the anatomy and mechanisms of pediatric pulmonary vein obstruction. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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8 pages, 202 KiB  
Article
Longer Exposure to Left-to-Right Shunts Is a Risk Factor for Pulmonary Vein Stenosis in Patients with Trisomy 21
by Connie Choi, Kimberlee Gauvreau, Philip Levy, Ryan Callahan, Kathy J. Jenkins and Minghui Chen
Children 2021, 8(1), 19; https://0-doi-org.brum.beds.ac.uk/10.3390/children8010019 - 01 Jan 2021
Cited by 12 | Viewed by 3201
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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Graphical abstract

Review

Jump to: Editorial, Research, Other

12 pages, 2621 KiB  
Review
Pulmonary Vein Stenosis—Evolving Surgical Management of a Challenging Disease
by Eric N. Feins, Ryan Callahan and Christopher W. Baird
Children 2021, 8(8), 631; https://0-doi-org.brum.beds.ac.uk/10.3390/children8080631 - 25 Jul 2021
Cited by 7 | Viewed by 3543
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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Other

6 pages, 629 KiB  
Case Report
Combination Chemotherapy in Severe Pulmonary Vein Stenosis—A Case Series
by Gabriel Krivenko, Karen Iacono, David Nykanen, Robyn Keen, Robert Sutphin and Michael Farias
Children 2023, 10(2), 364; https://0-doi-org.brum.beds.ac.uk/10.3390/children10020364 - 11 Feb 2023
Viewed by 1327
Abstract
Pulmonary vein stenosis results from a proliferative process that leads to the progressive obstruction of venous return to the left atrium. It is often resistant to catheterization and surgical based interventions and is frequently fatal when encountered in its severe form. Here, we [...] Read more.
Pulmonary vein stenosis results from a proliferative process that leads to the progressive obstruction of venous return to the left atrium. It is often resistant to catheterization and surgical based interventions and is frequently fatal when encountered in its severe form. Here, we describe three patients with severe, primary pulmonary vein stenosis that was progressing despite aggressive conventional management strategies. All three patients were initiated on combination chemotherapy with imatinib and sirolimus, drugs which have been previously shown to independently have potential benefit against PVS. Soon after the initiation of these therapies, all three patients experienced a stabilization of their disease process and clinical improvement. All three patients remain alive, with tolerable side effects from the medications. Although early in our experience and with only a small number of patients, combination chemotherapy with imatinib and sirolimus shows promise and merits further investigation as a therapeutic option for this aggressive disease. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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6 pages, 228 KiB  
Concept Paper
Progress in Pulmonary Vein Stenosis: Lessons from Success in Treating Pulmonary Arterial Hypertension
by Kathy J. Jenkins and Jeffrey R. Fineman
Children 2022, 9(6), 799; https://0-doi-org.brum.beds.ac.uk/10.3390/children9060799 - 29 May 2022
Cited by 1 | Viewed by 1557
Abstract
Pulmonary vein stenosis (PVS) is a rare and poorly understood condition that can be classified as primary, acquired, status-post surgical repair of PVS, and/or associated with developmental lung disease. Immunohistochemical studies demonstrate that obstruction of the large (extrapulmonary) pulmonary veins is associated with [...] Read more.
Pulmonary vein stenosis (PVS) is a rare and poorly understood condition that can be classified as primary, acquired, status-post surgical repair of PVS, and/or associated with developmental lung disease. Immunohistochemical studies demonstrate that obstruction of the large (extrapulmonary) pulmonary veins is associated with the neointimal proliferation of myofibroblasts. This rare disorder is likely multifactorial with a spectrum of pathobiology. Treatments have been historically surgical, with an increasing repetitive interventional approach. Understanding the biology of these disorders is in its infancy; thus, medical management has lagged behind. Throughout medical history, an increased understanding of the underlying biology of a disorder has led to significant improvements in care and outcomes. One example is the treatment of pulmonary arterial hypertension (PAH). PAH shares several common themes with PVS. These include the spectrum of disease and biological alterations, such as vascular remodeling and vasoconstriction. Over the past two decades, an exponential increase in the understanding of the pathobiology of PAH has led to a dramatic increase in medical therapies that have changed the landscape of the disease. We believe that a similar approach to PVS can generate novel medical therapeutic targets that will markedly improve the outcome of these vulnerable patients. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
7 pages, 1049 KiB  
Case Report
Pulmonary Vein Stenosis Associated with Germline PIK3CA Mutation
by Delphine Yung, Kaitlyn Freeman and Ghayda Mirzaa
Children 2022, 9(5), 671; https://0-doi-org.brum.beds.ac.uk/10.3390/children9050671 - 05 May 2022
Cited by 2 | Viewed by 1744
Abstract
Pulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include surgery, interventional cardiac catheterization, and more recently, medications targeting cell proliferation, which are not uniformly effective. [...] Read more.
Pulmonary vein stenosis is a rare and frequently lethal childhood disease. There are few known genetic associations, and the pathophysiology is not well known. Current treatments include surgery, interventional cardiac catheterization, and more recently, medications targeting cell proliferation, which are not uniformly effective. We present a patient with PVS and a PIK3CA mutation, who demonstrated a good response to the targeted inhibitor, alpelisib. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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7 pages, 675 KiB  
Perspective
Pulmonary Vein Stenosis in Children: A Programmatic Approach Employing Primary and Anatomic Therapy
by James A. Kuo and Christopher J. Petit
Children 2021, 8(8), 663; https://0-doi-org.brum.beds.ac.uk/10.3390/children8080663 - 30 Jul 2021
Cited by 4 | Viewed by 1968
Abstract
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 [...] Read more.
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. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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8 pages, 1377 KiB  
Opinion
Pulmonary Vein Stenosis: Incremental Knowledge Gains to Improve Outcomes
by Rachel D. Vanderlaan and Christopher A. Caldarone
Children 2021, 8(6), 481; https://0-doi-org.brum.beds.ac.uk/10.3390/children8060481 - 07 Jun 2021
Cited by 5 | Viewed by 3762
Abstract
Pulmonary vein stenosis remains a considerable clinical challenge, with high mortality still present in children with progressive disease. In this review, we discuss the clinical spectrum of pulmonary vein stenosis and what is known about the etiology and potential modifying and contributing factors [...] Read more.
Pulmonary vein stenosis remains a considerable clinical challenge, with high mortality still present in children with progressive disease. In this review, we discuss the clinical spectrum of pulmonary vein stenosis and what is known about the etiology and potential modifying and contributing factors in progressive pulmonary vein stenosis. Full article
(This article belongs to the Special Issue Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management)
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