STATE-OF-THE-ART PAPER
Development and Pathology of Pulmonary Hypertension
Rubin M. Tuder, MD*,*,
Steven H. Abman, MD ,
Thomas Braun, MD, PhD ,
Frédérique Capron, MD, PhD ,
Troy Stevens, PhD||,
Patricia A. Thistlethwaite, MD, PhD¶ and
Sheila G. Haworth, MD#
* Divisions of Pulmonary Sciences and Critical Care Medicine, University of Colorado, Denver, Colorado
Pediatric Heart Lung Center, University of Colorado, Denver, Colorado
Max Planck Institute for Heart Lung Research, Bad Neuheim, Germany
Department of Pathology, Hôpital de la Pitié, Paris, France
|| Center for Lung Biology, University of South Alabama College of Medicine, Mobile, Alabama
¶ Division of Cardiothoracic Surgery, University of California, San Diego, San Diego, California
# Vascular Biology and Pharmacology Unit, Institute of Child Health, UCL, London, United Kingdom
Manuscript received February 6, 2009;
accepted April 15, 2009.
* Reprint requests and correspondence: Dr. Rubin M. Tuder, Program in Translational Lung Research, Division of Pulmonary Sciences and Critical Care Medicine, Department of Medicine, University of Colorado at Denver, School of Medicine, Anschutz Medical Campus Research 2, Room 9001, 12700 East 19th Avenue, Aurora, Colorado 80045 (Email: Rubin.Tuder{at}UCdenver.edu).
The Development and Pathology working group was charged with reviewing the present knowledge, gaps in understanding, and areas for further studies in a broad range of themes. These themes in pulmonary vascular biology and pathobiology involved: 1) pulmonary vascular development; 2) pulmonary vascular disease accompanying fetal development and perinatal life; 3) properties of pulmonary vascular endothelial cells; 4) role of bone marrow cells in pulmonary vascular disease; 5) insights into pulmonary thromboembolic disease; 6) role of pathology in the assessment of pulmonary vascular disease; and 7) considerations of tissue banking for research in pulmonary hypertension. These important goals provide a blueprint for future research that may significantly impact our present and future understanding of pulmonary hypertension.
Key Words: pulmonary hypertension • pulmonary circulation • angiogenesis • endothelial cell progenitor • pathology
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Abbreviations and Acronyms
| | BMP = bone morphogenetic protein | | BMPR = bone morphogenetic protein receptor | | BPD = bronchopulmonary dysplasia | | CDH = congenital diaphragmatic hernia | | CTEPH = chronic thromboembolic pulmonary hypertension | | eNOS = endothelial nitric oxide synthase | | EPC = endothelial progenitor cell | | IPAH = idiopathic pulmonary arterial hypertension | | PAH = pulmonary arterial hypertension | | PCH = pulmonary capillary hemangiomatosis | | PH = pulmonary hypertension | | PPHN = persistent pulmonary hypertension of the newborn | | PVOD = pulmonary veno-occlusive disease | | TGF = transforming growth factor | | WHO = World Health Organization |
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