溶血性贫血相关性肺动脉高压

Life Pub Date : 2024-07-14 DOI:10.3390/life14070876
P. Karyofyllis, E. Demerouti, Eleftheria-Garyfallia Tsetika, Styliani Apostolopoulou, Panagiotis Tsiapras, Ioannis Iakovou, Dimitrios Tsiapras
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摘要

溶血性贫血是肺动脉高压(PH)的危险因素之一,目前被世界卫生组织列为第 5 类肺动脉高压,但有关适当治疗策略的数据却很有限。研究共纳入了 28 名经右心导管检查确诊为 PH 的患者,其中 85.7% 患有地中海贫血症,14.3% 患有镰状细胞病。根据 PH 血流动力学定义和整体诊断方法,患者被分为三组:42.9%的患者患有毛细血管前 PH(肺动脉高压-PAH 组),25%的患者患有毛细血管后 PH,32.1%的患者患有慢性血栓栓塞性 PH(CTEPH)(29%的地中海贫血患者和 50%的 SCD 患者)。记录了各组的治疗方法及其对预后和血液动力学的影响。82.1%的患者接受了针对 PAH 的药物治疗,6 名 CTEPH 患者接受了球囊肺血管成形术(BPA)。溶血性贫血 CTEPH 组与其他组的 mPAP 和 PVR 基线值在统计学上存在显著差异。PAH 特异性药物治疗使 PAH 组的血流动力学得到改善。接受BPA治疗的患者肺血流动力学得到改善。中位生存时间为 162 个月,生存率为 1 年-100%;2、3、4、5 和 6 年-96%;9 年-90%;13 年-78%。在溶血性贫血患者中,诱发 PH 的范围很广,这凸显了正确诊断的重要性。CTEPH患者的BPA和PAH患者的特异性PAH药物治疗是潜在的治疗策略;但是,应在PH专家中心根据患者的个体化情况进行治疗。
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Haemolytic Anaemia-Related Pulmonary Hypertension
Haemolytic anaemia represents a risk factor for the development of pulmonary hypertension (PH), currently classified as World Health Organization group 5 PH, and data regarding appropriate therapeutic strategy are limited. A total of 28 patients, 85.7% with thalassaemia and 14.3% with sickle cell disease, with a diagnosis of PH confirmed by right heart catheterization were included in the study. The patients were divided into three groups according to the PH haemodynamic definition and overall diagnostic approach: 42.9% had precapillary PH (pulmonary arterial hypertension—PAH group), 25% had post-capillary PH, and 32.1% had chronic thromboembolic PH (CTEPH) (29% of b-thalassemia and 50% of SCD patients). The therapeutic approach in each group and its impact on the outcome and haemodynamics were recorded. PAH-specific drug therapy received 82.1% of patients, and balloon pulmonary angioplasty (BPA) was performed in six patients with CTEPH. There were statistically significant differences in baseline mPAP and PVR values between the CTEPH-haemolytic anaemia group and other groups. PAH-specific drug therapy resulted in haemodynamic improvement for the PAH group. Patients who underwent BPA had improved pulmonary haemodynamics. The median survival time was 162 months, and the survival rate was 1 year—100%; 2, 3, 4, 5, and 6 years—96%; 9 years—90%; and 13 years—78%. In patients with haemolytic anaemia, the wide spectrum of induced PH highlighted the importance of a correct predominant diagnosis. BPA in CTEPH patients and specific-PAH drug therapy for PAH patients represent potential therapeutic strategies; however, the management should be offered in expert PH centres under individualized approaches for patients.
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