Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management (2nd Edition)

A Special Issue of Children (ISSN 2227-9067) belonging to the section "Pediatric Pulmonary and Sleep Medicine".

Deadline for manuscript submissions: closed (10 August 2026) | Viewed by 1327

Editor


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; pediatric cardiology
Special Issues, Collections and Topics in MDPI journals

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 cells in the neointima of the pulmonary veins, though the inciting etiology and cellular processes are 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.

Considering the success and popularity of the Special Issue “Pulmonary Vein Stenosis in Neonates and Infants: Etiology, Diagnosis and Management” previously published in the journal Children (https://www.mdpi.com/journal/children/special_issues/pulmonary_vein_stenosis) and the recent scientific progress, we now release a second edition that aims to focus 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, translational, 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 toward 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 250 words) can be sent to the Editorial Office for assessment.

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-anonymized 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
  • neoproliferation
  • congenital heart defect
  • prematurity
  • infant

Benefits of Publishing in a Special Issue

  • Ease of navigation: Grouping papers by topic helps scholars navigate broad scope journals more efficiently.
  • Greater discoverability: Special Issues support the reach and impact of scientific research. Articles in Special Issues are more discoverable and cited more frequently.
  • Expansion of research network: Special Issues facilitate connections among authors, fostering scientific collaborations.
  • External promotion: Articles in Special Issues are often promoted through the journal's social media, increasing their visibility.
  • Reprint: MDPI Books provides the opportunity to republish successful Special Issues in book format, both online and in print.

Further information on MDPI's Special Issue policies can be found here.

Related Special Issue

Published Papers (2 papers)

Order results
Result details
Select all
Export citation of selected articles as:

Research

Jump to: Review

22 pages, 24490 KB  
Article
Imatinib Mesylate and Sirolimus Inhibit Vascular Fibroproliferative Remodeling in Pulmonary Vein Stenosis
by Julia Gaal, Sophie C. Doettl, Kerstin Saraci, Cindy Zajac, Alexa von Mueffling, Peter E. Hammer, Magnus von Piechowski, Verena Gebert, Andrei-Antonio M. Caracioni, Daniel Diaz-Gil, Viktoria Weixler, Pedro J. Del Nido, Kathy Jenkins and Ingeborg Friehs
Children 2026, 13(9), 1280; https://doi.org/10.3390/children13091280 - 21 Sep 2026
Abstract
Background: Pulmonary vein stenosis (PVS) is a progressive disease characterized by neointimal hyperplasia with poor outcomes. Clinical observations show that adjunctive therapy with imatinib mesylate and sirolimus is associated with improved survival and reduced rates of reintervention. In this study, we aimed to [...] Read more.
Background: Pulmonary vein stenosis (PVS) is a progressive disease characterized by neointimal hyperplasia with poor outcomes. Clinical observations show that adjunctive therapy with imatinib mesylate and sirolimus is associated with improved survival and reduced rates of reintervention. In this study, we aimed to investigate the cellular mechanisms underlying PVS development and progression and to elucidate how these targeted therapies modulate these processes. Methods: We retrospectively analyzed cardiac catheterization and computed tomography, and lung scan data from three patients with PVS to estimate wall shear stress (WSS) at multiple time points. Tissue specimens resected during anatomical repair surgery were analyzed for tissue composition and fibrosis using hematoxylin and eosin staining, Masson’s trichrome staining, and flow cytometric analysis of endothelial cell (ECs) and fibroblast markers to determine endothelial-to-mesenchymal transition (EndMT). The safety and efficacy of imatinib mesylate and sirolimus were evaluated in healthy and PVS-derived ECs using cellular viability and proliferation assays, and by assessing fibrotic marker expression indicative of EndMT. Results: Patients exhibited severely elevated WSS estimates at multiple time points throughout disease progression. Disproportionate fibrotic remodeling driven by ECs transitioning into mesenchymal cells through EndMT was the cellular substrate of resected tissue in PVS. Targeted therapy with clinically relevant concentrations of imatinib mesylate and sirolimus was safe and significantly inhibited fibroproliferation and EndMT. At follow-up, all patients were alive and free of surgical and stent-based reintervention after anatomic repair surgery and adjunctive imatinib mesylate and sirolimus therapy. Conclusions: Fibroproliferative ECs undergoing EndMT are likely the cellular substrate underlying PVS development and progression. A multimodal approach consisting of anatomically focused surgical repair with adjunctive imatinib mesylate and sirolimus therapy may attenuate fibroproliferative remodeling. Full article
Show Figures

Figure 1

Review

Jump to: Research

16 pages, 2295 KB  
Review
Therapeutic Targets for Pediatric Pulmonary Vein Stenosis: Insights from Animal Models
by Siqi She, Debao Li, Qi Sun and Lincai Ye
Children 2026, 13(5), 677; https://doi.org/10.3390/children13050677 - 14 May 2026
Viewed by 806
Abstract
Pulmonary vein stenosis (PVS) is a rare and devastating condition affecting infants and children, characterized by progressive intimal hyperplasia, myofibroblast proliferation, and extracellular matrix deposition, leading to pulmonary hypertension and right heart failure. Despite multimodal interventions including surgery and catheter-based approaches, long-term outcomes [...] Read more.
Pulmonary vein stenosis (PVS) is a rare and devastating condition affecting infants and children, characterized by progressive intimal hyperplasia, myofibroblast proliferation, and extracellular matrix deposition, leading to pulmonary hypertension and right heart failure. Despite multimodal interventions including surgery and catheter-based approaches, long-term outcomes remain poor due to high rates of restenosis and disease progression. The development of representative animal models has been instrumental in unraveling the complex pathophysiology of PVS and identifying potential therapeutic targets. This review comprehensively examines the evolution of PVS animal models—from large animals to recently established rodent models—and synthesizes insights gained regarding key pathogenic pathways and their therapeutic implications in guiding associated clinical trials in pediatric patients. We discuss evidence supporting mammalian target of rapamycin (mTOR) inhibition, TGF-β, platelet-derived growth factor (PDGF) and vascular endothelial growth factor (VEGF) targeting, and emerging strategies including fibroblast activation protein (FAP) inhibition and YAP/β-catenin pathway modulation. The recent development of neonatal rat PVS models has accelerated translational research by enabling cost-effective, high-throughput evaluation of candidate therapies. We propose a mechanistic framework integrating these pathways and discuss future directions for precision medicine approaches in PVS. Full article
Show Figures

Figure 1

Back to TopTop