George A Alba1, Deepak Atri2, Sriranjani Darbha3, Inderjit Singh4, Victor F Tapson5, Michael I Lewis5, Hyung J Chun6, Yen-Rei Yu7, Bradley A Maron2,8, Sudarshan Rajagopal9. 1. Division of Pulmonary and Critical Care, Massachusetts General Hospital, Boston, MA, USA. 2. Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, Boston, MA, USA. 3. College of Natural Sciences, The University of Texas, Austin, TX, USA. 4. Division of Pulmonary, Critical Care, and Sleep Medicine, Yale New Haven Hospital and Yale School of Medicine, New Haven, CT, USA. 5. Division of Pulmonary and Critical Care Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA. 6. Section of Cardiovascular Medicine, Department of Internal Medicine, Yale Cardiovascular Research Center, Yale School of Medicine, New Haven, CT, USA. 7. Division of Pulmonary, Allergy and Critical Care Medicine, Department of Medicine, Duke University Medical Center, Durham, NC, USA. 8. Section of Cardiology, Veterans Affairs Boston Healthcare System, Boston, MA, USA. 9. Division of Cardiology, Department of Medicine, Duke University Medical Center, Durham, NC, USA. sudarshan.rajagopal@duke.edu.
Abstract
PURPOSE OF REVIEW: Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon complication of acute pulmonary embolism (PE), in which the red, platelet-rich thrombus does not resolve but forms into an organized yellow, fibrotic scar-like obstruction in the pulmonary vasculature. Here we review the pathobiology of CTEPH. RECENT FINDINGS: Our current knowledge has predominantly been informed by studies of human samples and animal models that are inherently limited in their ability to recapitulate all aspects of the disease. These studies have identified alterations in platelet biology and inflammation in the formation of a scar-like thrombus that comprised endothelial cells, myofibroblasts, and immune cells, along with a small vessel pulmonary arterial hypertension-like vasculopathy. The development of CTEPH-specific therapies is currently hindered by a limited knowledge of its pathobiology. The development of new CTEPH medical therapies will require new insights into its pathobiology that bridge the gap from bench to bedside.
PURPOSE OF REVIEW: Chronic thromboembolic pulmonary hypertension (CTEPH) is an uncommon complication of acute pulmonary embolism (PE), in which the red, platelet-rich thrombus does not resolve but forms into an organized yellow, fibrotic scar-like obstruction in the pulmonary vasculature. Here we review the pathobiology of CTEPH. RECENT FINDINGS: Our current knowledge has predominantly been informed by studies of human samples and animal models that are inherently limited in their ability to recapitulate all aspects of the disease. These studies have identified alterations in platelet biology and inflammation in the formation of a scar-like thrombus that comprised endothelial cells, myofibroblasts, and immune cells, along with a small vessel pulmonary arterial hypertension-like vasculopathy. The development of CTEPH-specific therapies is currently hindered by a limited knowledge of its pathobiology. The development of new CTEPH medical therapies will require new insights into its pathobiology that bridge the gap from bench to bedside.
Authors: Joanna Pepke-Zaba; Marion Delcroix; Irene Lang; Eckhard Mayer; Pavel Jansa; David Ambroz; Carmen Treacy; Andrea M D'Armini; Marco Morsolini; Repke Snijder; Paul Bresser; Adam Torbicki; Bent Kristensen; Jerzy Lewczuk; Iveta Simkova; Joan A Barberà; Marc de Perrot; Marius M Hoeper; Sean Gaine; Rudolf Speich; Miguel A Gomez-Sanchez; Gabor Kovacs; Abdul Monem Hamid; Xavier Jaïs; Gérald Simonneau Journal: Circulation Date: 2011-10-03 Impact factor: 29.690
Authors: D Bonderman; H Wilkens; S Wakounig; H-J Schäfers; P Jansa; J Lindner; I Simkova; A M Martischnig; J Dudczak; R Sadushi; N Skoro-Sajer; W Klepetko; I M Lang Journal: Eur Respir J Date: 2008-09-17 Impact factor: 16.671