Article Type: Studies of Vascular Biology and Mechanism of Disease

In Vitro Models to Study Fibroelastic Remodeling of the Endocardium in Congenital Heart Disease

Julia Gaal, Cindy Zajac, Sophie C. Doettl, Magnus von Piechowski, Kerstin Saraci, Viktoria Weixler, Daniel Diaz-Gil, Pedro J. del Nido, Ingeborg Friehs Boston Children’s Hospital. Charité University. University Hospital of Giessen and Marburg and Justus Liebig University Giessen. Harvard Medical School.United States and Germany Pediatric CardiologyPediatr Cardiol 2026;DOI: 10.1007/s00246-026-04503-w AbstractCongenital heart defects (CHD) are characterized

In Vitro Models to Study Fibroelastic Remodeling of the Endocardium in Congenital Heart Disease Read More »

Imatinib Mesylate and Sirolimus Inhibit Vascular Fibroproliferative Remodeling in Pulmonary Vein Stenosis

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, Ingeborg Friehs Boston Children’s Hospital and Harvard Medical School. German Heart Center of Charité-Universitätsmedizin Berlin.United States and Germany ChildrenChildren 2026; 13:DOI: 10.3390/children13091280

Imatinib Mesylate and Sirolimus Inhibit Vascular Fibroproliferative Remodeling in Pulmonary Vein Stenosis Read More »

Losartan attenuates upstream vasculopathy in a modified piglet model of pulmonary vein stenosis: contribution of the Hippo pathway

Wenhui Zeng, Siming Liu, Zhen Xu, Fangbao Ding, Ju Mei, Jiaquan Zhu Xinhua Hospital, Shanghai Jiaotong University School of Medicine.China Annals of Translational MedicineAnn Transl Med 2022; DOI: 10.21037/atm-22-2621 AbstractBackground: Diffuse pulmonary vein stenosis (PVS) is an intractable congenital heart disease for which the underlying mechanism remains unclear. In this study, we investigated the effect of losartan

Losartan attenuates upstream vasculopathy in a modified piglet model of pulmonary vein stenosis: contribution of the Hippo pathway Read More »

Insights into the molecular mechanism of pulmonary vein stenosis in pediatric patients with congenital heart disease

Alyssa B. Kalustian, Pengfei Ji, Lalita Wadhwa, Christopher A. Caldarone, Manish Bansal, Athar M. Qureshi, Jeffrey S. Heinle, Ravi K. Birla Texas Children’s Hospital and Baylor College of Medicine. United States Journal of Thoracic and Cardiovascular Surgery OpenJTCVS Open 2025; 26: 166-181DOI: 10.1016/j.xjon.2025.04.012 AbstractObjective: Pediatric pulmonary vein stenosis (PVS) is associated with substantial morbidity and mortality for

Insights into the molecular mechanism of pulmonary vein stenosis in pediatric patients with congenital heart disease Read More »

Evaluation of Treatment Effect of Sirolimus on Pediatric Pulmonary Vein Stenosis Using a Neonatal Rat Model

Debao Li, Yingying Xiao, Peisen Ruan, Zunmin Wan, Yuqing Hu, Sijuan Sun, Zheng Wang, Sixie Zheng, Yi Yan, Hao Chen, Hao Zhang, Chun Shen, Qi Sun, Lincai Ye Shanghai Children’s Medical Center and Shanghai Jiao Tong University School of Medicine. Children’s Hospital of Fudan University and National Children’s Medical Center. Women and Children’s Hospital of

Evaluation of Treatment Effect of Sirolimus on Pediatric Pulmonary Vein Stenosis Using a Neonatal Rat Model Read More »

A neonatal rat model of pulmonary vein stenosis

Debao Li, Lisheng Qiu, Haifa Hong, Hao Chen, Peibin Zhao, Yingying Xiao, Hao Zhang, Qi Sun, Lincai Ye Shanghai Children’s Medical Center and Shanghai Jiao Tong University. Second Affiliated Hospital and Yuying Children’s Hospital and Wenzhou Medical University. China Cell and BioscienceCell Biosci 2023; 13:DOI: 10.1186/s13578-023-01058-8 AbstractObjectives: Pulmonary vein stenosis (PVS), one of the most challenging clinical

A neonatal rat model of pulmonary vein stenosis Read More »

Targeted Rapamycin Delivery via Magnetic Nanoparticles to Address Stenosis in a 3D Bioprinted in Vitro Model of Pulmonary Veins

Liqun Ning, Stefano Zanella, Martin M. Tomov, Medhi Salar Amoli, Linqi Jin, Bouen Hwang, Maher Saadeh, Huang Chen, Sunder Neelakantan, Lakshmi Prasad Dasi, Reza Avazmohammadi, Morteza Mahmooudi, Holly D. Bauser-Heaton, Vahid Serpooshan Emory University School of Medicine, Children’s Healthcare of Atlanta and Georgia Institute of Technology. Cleveland State University. Texas A&M University. Michigan State University.United

Targeted Rapamycin Delivery via Magnetic Nanoparticles to Address Stenosis in a 3D Bioprinted in Vitro Model of Pulmonary Veins Read More »

Mechanism of pulmonary vein stenosis in infants with normally connected veins

Iran M. Sadr, Puay E. Tan, Mark W. Kieran, Kathy J. Jenkins Children’s Hospital, Boston.United States American Journal of CardiologyAm J Cardiol 2000; 86: 577-579DOI: 10.1016/s0002-9149(00)01022-5 AbstractWe used microscopy, immunohistochemistry, and cell culture to identify the mechanism of restenosis in 4 infants with isolated pulmonary vein stenosis. Recurrent obstruction appears to be due to myofibroblastic proliferation

Mechanism of pulmonary vein stenosis in infants with normally connected veins Read More »

Metakaryotic cells linked to pediatric pulmonary vein stenosis

Edward C. Kirkpatrick, Michael E. Mitchell, William G. Thilly, Pip Hidestrand, Aoy Tomita-Mitchell, Mats Hidestrand, Elena V. Gostjeva Children’s Hospital of Wisconsin and Medical College of Wisconsin. Massachusetts Institute of Technology. Eastern Maine Medical Center.United States Cardiovascular PathologyCardiovasc Pathol 2019; 39: 51-53DOI: 10.1016/j.carpath.2018.12.005 AbstractNo Abstract AvailableMethods: Pulmonary vein tissue from five patients with PVS was collected

Metakaryotic cells linked to pediatric pulmonary vein stenosis Read More »

Paucicellular Fibrointimal Proliferation Characterizes Pediatric Pulmonary Vein Stenosis: Clinicopathologic Analysis of 213 Samples From 97 Patients

Alexandra E. Kovach, Philip M. Magcalas, Christina Ireland, Kerry McEnany, Andre M. Oliveira, Mark W. Kieran, Christopher W. Baird, Kathy Jenkins, MD, Sara O. Vargas Boston Children’s Hospital. Dana Farber Cancer Institute. Mayo Clinic.United States American Journal of Surgical PathologyAm J Surg Pathol 2017; 41: 1198-1204DOI: 10.1097/PAS.0000000000000892 AbstractPulmonary vein stenosis (PVS) is a luminal narrowing of

Paucicellular Fibrointimal Proliferation Characterizes Pediatric Pulmonary Vein Stenosis: Clinicopathologic Analysis of 213 Samples From 97 Patients Read More »