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J Am Coll Cardiol, 2009; 54:3-9, doi:10.1016/j.jacc.2009.04.009
© 2009 by the American College of Cardiology Foundation
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STATE-OF-THE-ART PAPER

Development and Pathology of Pulmonary Hypertension

Rubin M. Tuder, MD*,*, Steven H. Abman, MD{dagger}, Thomas Braun, MD, PhD{ddagger}, 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
{dagger} Pediatric Heart Lung Center, University of Colorado, Denver, Colorado
{ddagger} 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

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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