@article{3139630, title = "Pulmonary capillary endothelial metabolic function in chronic thromboembolic pulmonary hypertension", author = "Orfanos, S. E. and Hirsch, A. M. and Giovinazzo, M. and Armaganidis, A. and and Catravas, J. D. and Langleben, D.", journal = "Journal of Thrombosis and Haemostasis", year = "2008", volume = "6", number = "8", pages = "1275-1280", publisher = "Wiley-Blackwell", issn = "1538-7933, 1538-7836", doi = "10.1111/j.1538-7836.2008.03046.x", keywords = "angiotensin converting enzyme; endothelium; pulmonary hypertension; pulmonary metabolism; thromboembolic disease", abstract = "Background: Chronic thromboembolic pulmonary hypertension (CTEPH) causes physical plugging of large pulmonary arteries as well as a distal micro-vasculopathy. Pulmonary endothelium is an active metabolic tissue in normal humans. The effects of CTEPH on pulmonary endothelial metabolism are unknown. Objectives: We studied pulmonary capillary endothelium-bound angiotensin converting enzyme (ACE) activity as an index of endothelial metabolism in patients with CTEPH. Patients/methods: We measured single-pass transpulmonary per cent metabolism (%M) and hydrolysis of an ACE synthetic substrate and calculated functional capillary surface area (FCSA), normalized to body surface area (BSA), in 13 patients with CTEPH and 23 controls. Results: Mean %M for CTEPH (71.6 +/- 4.0% SE) was similar to controls (74.7 +/- 2.7%). Substrate hydrolysis (v) was similar for CTEPH (1.47 +/- 0.22) and controls (1.51 +/- 0.11). However, FCSA/BSA was reduced (P < 0.01) for CTEPH (1530 +/- 218 mL min(-1)*m(-2)) as compared with controls (2948 +/- 245). Conclusions: The metabolically functional pulmonary capillary bed is reduced in CTEPH. However, because %M and hydrolysis are preserved, this points to a reduction in functional capillary surface area rather than reduced ACE activity on the pulmonary capillary endothelial cell. The reduction in functional capillary surface area may just be a result of decreased capillary recruitment because of upstream vascular plugging by chronic organized thrombus." }