Karyofyllis Panagiotis, Tsiapras Dimitrios, Demerouti Eftychia, Armenis Iakovos, Papadopoulou Varvara, Voudris Vassilis
Department of Interventional Cardiology, Onassis Cardiac Surgery Center, Syggrou 356 Avenue, Kallithea, 17674, Athens, Attiki, Greece.
Department of Non-Invasive Diagnostics, Onassis Cardiac Surgery Center, Athens, Attiki, Greece.
J Thromb Thrombolysis. 2022 Feb;53(2):467-470. doi: 10.1007/s11239-021-02483-7. Epub 2021 May 27.
Sickle cell disease (SCD), a haemoglobinopathy characterized by chronic haemolysis with acute exacerbations and vascular occlusion episodes, may be complicated by pulmonary hypertension. The latter may be caused by chronic thromboembolic disease of pulmonary artery branches and its management is not well-defined. Herein, we present a case of SCD complicated by chronic thromboembolic pulmonary hypertension of subsegmental pulmonary artery branches successfully treated with endothelin receptor antagonists, orally administered prostacyclin analogs and balloon pulmonary angioplasty. This challenging case highlights the need for clinical awareness of chronic thromboembolic pulmonary hypertension as a specific and potentially curable form of pulmonary hypertension complicating SCD course that may necessitate combined pharmacologic and interventional management.
镰状细胞病（SCD）是一种血红蛋白病，其特征为慢性溶血伴急性加重和血管闭塞发作，可能并发肺动脉高压。后者可能由肺动脉分支的慢性血栓栓塞性疾病引起，其治疗方法尚不明确。在此，我们报告一例SCD并发亚段肺动脉分支慢性血栓栓塞性肺动脉高压的病例，该病例通过内皮素受体拮抗剂、口服前列环素类似物和球囊肺血管成形术成功治疗。这一具有挑战性的病例凸显了临床认识慢性血栓栓塞性肺动脉高压的必要性，它是SCD病程中一种特殊的、可能可治愈的肺动脉高压形式，可能需要药物和介入联合治疗。
