TY - JOUR T1 - Pulmonary endarterectomy in sickle cell haemoglobin C disease JF - European Respiratory Journal JO - Eur Respir J SP - 735 LP - 737 DO - 10.1183/09031936.00192910 VL - 38 IS - 3 AU - A. Jerath AU - P. Murphy AU - M. Madonik AU - D. Barth AU - J. Granton AU - M. de Perrot Y1 - 2011/09/01 UR - http://erj.ersjournals.com/content/38/3/735.abstract N2 - To the Editors:Pulmonary hypertension (PH) is a common complication of sickle cell disease (SCD), with a prevalence of 10–20%, and is often a cause of death in this patient population [1]. PH can be related to two different mechanisms: 1) chronic haemolysis with endothelial dysfunction, reduction in nitric oxide bioavailability and subsequent proliferative vasculopathy; or 2) vaso-occlusive complications resulting from erythrocyte sickling and hyperviscosity [2]. According to a recent report, up to 23% of SCD patients with PH have evidence of perfusion mismatch on ventilation/perfusion scan and 11.5% have evidence of chronic thromboembolic disease on computed tomography (CT) pulmonary angiograms [3]. Although pulmonary endarterectomy (PEA) is the best option for patients with PH related to chronic thromboembolic disease, chronic haemolysis with subsequent proliferative vasculopathy in the distal vessels put patients with SCD at increased risk of residual PH after PEA. In addition, SCD patients may be at increased risk of developing in situ thrombosis in the proximal vessels which can be difficult to differentiate from true thromboembolic events on radiological imaging.Herein, we present the case of a patient with SCD due to sickle cell haemoglobin (Hb) C disease (HbSC) and chronic thromboembolic pulmonary hypertension (CTEPH) who underwent successful PEA with complete normalisation of the pulmonary … ER -