TY - T1的微血管疾病慢性thromboembolic pulmonary hypertension: a role for pulmonary veins and systemic vasculature JF - European Respiratory Journal JO - Eur Respir J SP - 1275 LP - 1288 DO - 10.1183/09031936.00169113 VL - 44 IS - 5 AU - Dorfmüller, Peter AU - Günther, Sven AU - Ghigna, Maria-Rosa AU - Thomas de Montpréville, Vincent AU - Boulate, David AU - Paul, Jean-François AU - Jaïs, Xavier AU - Decante, Benoit AU - Simonneau, Gérald AU - Dartevelle, Philippe AU - Humbert, Marc AU - Fadel, Elie AU - Mercier, Olaf Y1 - 2014/11/01 UR - //www.qdcxjkg.com/content/44/5/1275.abstract N2 - Limited numbers of operated patients with chronic thromboembolic pulmonary hypertension (CTEPH) are refractory to pulmonary endarterectomy (PEA) and experience persistent pulmonary hypertension (PH). We retrospectively assessed lung histology available from nine patients with persistent PH (ineffective PEA (inPEA) group) and from eight patients transplanted for distal CTEPH inaccessible by PEA (noPEA group). Microscopically observed peculiarities were compared with the histology of a recently developed CTEPH model in piglets. Pre-interventional clinical/haemodynamic data and medical history of patients from the inPEA and noPEA groups were collected and analysed. Conspicuous remodelling of small pulmonary arteries/arterioles, septal veins and pre-septal venules, including focal capillary haemangiomatosis, as well as pronounced hypertrophy and enlargement of bronchial systemic vessels, were the predominant pattern in histology from both groups. Most findings were reproduced in our porcine CTEPH model. Ink injection experiments unmasked abundant venular involvement in so-called small vessel or microvascular disease, as well as post-capillary bronchopulmonary shunting in human and experimental CTEPH. Microvascular disease is partly due to post-capillary remodelling in human and experimental CTEPH and appears to be related to bronchial-to-pulmonary venous shunting. Further studies are needed to clinically assess the functional importance of this finding. Pulmonary veins and systemic vasculature contribute to microvascular disease in CTEPH http://ow.ly/yGK4U ER -