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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

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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