Karyofyllis Panagiotis, Demerouti Eftychia, Tsetika Eleftheria-Garyfallia, Apostolopoulou Styliani, Tsiapras Panagiotis, Iakovou Ioannis, Tsiapras Dimitrios
Invasive Cardiology Department, Onassis Cardiac Surgery Center, 17674 Athens, Greece.
Non-Invasive Cardiology Department, Onassis Cardiac Surgery Center, 17674 Athens, Greece.
Life (Basel). 2024 Jul 14;14(7):876. doi: 10.3390/life14070876.
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.
溶血性贫血是肺动脉高压（PH）发生的一个危险因素，目前被归类为世界卫生组织第5组PH，而关于合适治疗策略的数据有限。本研究纳入了28例经右心导管检查确诊为PH的患者，其中85.7%为地中海贫血患者，14.3%为镰状细胞病患者。根据PH的血流动力学定义和总体诊断方法，将患者分为三组：42.9%患有毛细血管前PH（肺动脉高压-PAH组），25%患有毛细血管后PH，32.1%患有慢性血栓栓塞性PH（CTEPH）（β地中海贫血患者中占29%，镰状细胞病患者中占50%）。记录了每组的治疗方法及其对结局和血流动力学的影响。82.1%的患者接受了PAH特异性药物治疗，6例CTEPH患者接受了球囊肺动脉血管成形术（BPA）。CTEPH-溶血性贫血组与其他组之间的基线平均肺动脉压（mPAP）和肺血管阻力（PVR）值存在统计学显著差异。PAH特异性药物治疗使PAH组的血流动力学得到改善。接受BPA的患者肺血流动力学得到改善。中位生存时间为162个月，1年生存率为100%；2年、3年、4年、5年和6年生存率为96%；9年生存率为90%；13年生存率为78%。在溶血性贫血患者中，广泛的诱发性PH凸显了正确的主要诊断的重要性。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；然而，应在专业的PH中心根据患者的个体化方法提供管理。
