Abstract
Pulmonary hypertension (PH) is one of the main cardiovascular complications in haemoglobinopathies and is considerably implicated in patients’ morbidity and mortality. In thalassemia intermedia, PH is found in about 60% of traditionally managed patients and represents the main cause of heart failure. In sickle cell anemia, PH is encountered in one third of patients and has been found to be a strong and independent predictor of mortality, while in sickle thalassemia, PH is generally less frequent and severe. The pathophysiology of PH in haemoglobinopathies is multifactorial and several mechanisms seem to be implicated, including a complex vasculopathy, hypercoagulability and elastic tissue defects, all associated with chronic hemolysis, high output state due to chronic anemia, as well as left heart dysfunction, pulmonary disorders and thromboembolic complications. Echocardiography is the most useful tool for patients’ screening on a regular basis, while the diagnosis of PH should always be confirmed by right cardiac catheterization. The proper management of the disease itself with haematological modalities such as blood transfusions combined with iron chelation or hydroxyurea, is the most effective approach for the prevention and treatment of haemoglobinopathy-associated PH. Antithrombotic agents should also be considered while the value of novel agents used in the treatment of pulmonary arterial hypertension, including endothelin antagonists or phosphodiesterase-5 inhibitors, is not yet established in patients with haemoglobinopathies.肺动脉高压（PH）是血红蛋白病患者中主要心脏血管并发症之一，与患者的发病和死亡有着密切的关系。 在患有地中海贫血中间的病人体内，PH仅为传统护理病人的60%，这是导致心脏衰竭的主要原因。 在患有镰状细胞贫血的病人体内，PH仅为传统护理病人的三分之一，这已经成为死亡强有力的独立指标。然而，在患有镰状细胞的中海贫血的病人体内，PH的频率通常更低而且严重。 血红蛋白病的PH病理生理学是多元的并涉及多个发病机制，包括复杂血管病变、高凝状态和弹性组织缺损，相关的所有慢性溶血现象、慢性贫血引起的高输出状态，以及左心室功能障碍、肺部異常和血栓栓塞并发症。 超声心动图仪是定期筛查病人情况最有效的工具，而PH的诊断应使用正确的心导管插入术来确认。 采用血液物理疗法正确控制肺动脉高压，比如结合使用输血和铁螯合疗法，是预防和治疗血红蛋白病关联PH最有效的疗法。 尽管使用新药剂（包括内皮素拮抗剂和磷酸二酯酶5型抑制剂）治疗血红蛋白病患者的肺动脉高压作用还不确定，但也应考虑使用抗血栓形成剂 。