Pulmonary Hypertension in Hemolytic Disorders Pulmonary Vascular Disease: The Global Perspective
Pulmonary Hypertension in Hemolytic Disorders Pulmonary Vascular Disease: The Global Perspective
复制标题
DOI：
10.1378/chest.09-3057
复制
发表时间：
2010-06-01
期刊：
影响因子：
9.6
通讯作者：
Gladwin, Mark T.
中科院分区：
文献类型：
--
作者：
Machado, Roberto F.;Gladwin, Mark T.
遗传性血红蛋白疾病镰状细胞病和地中海贫血是世界上最常见的单基因疾病。肺动脉高压是镰状细胞病和地中海贫血成人患者发病和死亡的主要原因之一，溶血性疾病可能是肺动脉高压最常见的原因之一。溶血性疾病中肺动脉高压的发病机制可能是多因素的，包括溶血、一氧化氮（NO）生物利用度受损、慢性低氧血症、慢性血栓栓塞性疾病、慢性肝病和无脾。与传统形式的肺动脉高压患者相比，溶血性疾病患者的平均肺压轻度至中度升高，肺血管阻力轻度升高。这些患者肺动脉高压的血流动力学病因是多因素的，包括肺动脉高压、肺静脉高压和继发于高动力状态的肺动脉高压。目前，关于任何特定治疗方式对溶血性疾病患者肺动脉高压的影响的数据有限。在所有患者中最大限度地治疗原发性血红蛋白病以及在肺动脉高压患者中使用选择性肺血管扩张剂和抗增殖剂治疗可能是有益的。然而，对于这一患者人群的新型疗法的大型多国试验仍有很大的需求。胸部2010; 137（6）（增刊）：30 S-38 S
The inherited hemoglobin disorders sickle cell disease and thalassemia are the most common monogenetic disorders worldwide. Pulmonary hypertension is one of the leading causes of morbidity and mortality in adult patients with sickle cell disease and thalassemia, and hemolytic disorders are potentially among the most common causes of pulmonary hypertension. The pathogenesis of pulmonary hypertension in hemolytic disorders is likely multifactorial, including hemolysis, impaired nitric oxide (NO) bioavailability, chronic hypoxemia, chronic thromboembolic disease, chronic liver disease, and asplenia. In contrast to patients with traditional forms of pulmonary arterial hypertension, patients with hemolytic disorders have a mild-to-moderate degree of elevation in mean pulmonary pressures, with mild elevations in pulmonary vascular resistance. The hemodynamic etiology of pulmonary hypertension in these patients is multifactorial and includes pulmonary arterial hypertension, pulmonary venous hypertension, and pulmonary hypertension secondary to a hyperdynamic state. Currently, there are limited data on the effects of any specific treatment modality for pulmonary hypertension in patients with hemolytic disorders. It is likely that maximization of treatment of the primary hemoglobinopathy in all patients and treatment with selective pulmonary vasodilators and antiproliferative agents in patients with pulmonary arterial hypertension would be beneficial. However, there is still a major need for large multinational trials of novel therapies for this patient population. CHEST 2010; 137(6)(Suppl):30S-38S