Pathologic assessment of vasculopathies in pulmonary hypertension
Giuseppe G. Pietra, MD*,*,
Frederique Capron, MD ,
Susan Stewart, MD ,
Ornella Leone, MD ,
Marc Humbert, MD¶,
Ivan M. Robbins, MD,
Lynne M. Reid, MD|| and
R. M. Tuder, MD**
* Department of Pathology, University of Pennsylvania, School of Medicine, Philadelphia, Pennsylvania, USA
Service d'Anatomie Pathologique 1, Hôpital de la Pitié, 83, bd de l'Hôpital 75651 Paris Cedex, 13, France
Papworth Hospital, Papworth Everard, Cambridge, United Kingdom
Department of Pathology, Azienda Ospedaliera S. Orsola-Malpighi of Bologna, Bologna, Italy
¶ Service de Pneumologie et Réanimation Respiratoire, Hôpital Antoine Béclère, Assistance Publique, Hôpitaux de Paris, Université Paris-Sud, Clamart, France
Vanderbilt University Medical Center, Adult Pulmonary Hypertension Center, Vanderbilt University, Nashville, Tennessee, USA
|| Department of Pathology, Harvard Medical School, Boston, MassachusettsBaltimore, Maryland, USA
** Division of Cardiopulmonary Pathology, The Johns Hopkins University, School of Medicine, Baltimore, Maryland, USA
Manuscript received November 26, 2003;
accepted February 3, 2004.
*
Reprint requests and correspondence: Dr. Giuseppe G. Pietra, Via San Giorgio 23, CH 6976 Castagnola, Switzerland.
gkpietra{at}freesurf.ch
Pulmonary arterial hypertension (PAH) includes various forms of pulmonary hypertension of different etiology but similar clinical presentation and functional derangement. Histopathological vascular changes in all forms of PAH are qualitatively similar but with quantitative differences in the distribution and prevalence of pathological changes in various portions of the pulmonary vascular bed. The documentation of these topographic variations in the response of the pulmonary vasculature to injury may be important to understand the pathogenesis of the various subsets of PAH. To standardize the precise histopathological documentation of the pulmonary vasculopathy in PAH we propose a histopathological classification that includes both the predominant segment of the pulmonary vasculature affected and the possible coexistence of pathological changes in other vascular segments.
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Abbreviations and Acronyms
| | BMPR2 | = Bone morphometric protein receptor-2 | | HIV | = human immunodeficiency virus | | PAH | = pulmonary arterial hypertension | | PCH | = pulmonary capillary hemangiomatosis | | PM | = pulmonary microvasculopathy | | POV | = pulmonary occlusive venopathy | | PPH | = primary pulmonary hypertension | | PVOD | = pulmonary veno-occlusive disease |
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