Department of Cardiology, Faculty of Medicine, Pamukkale University, Denizli, Turkey,
Department of Cardiology, Faculty of Medicine, Pamukkale University, Denizli, Turkey.
Med Princ Pract. 2020;29(5):486-491. doi: 10.1159/000506596. Epub 2020 Feb 19.
Pulmonary hypertension (PH) can complicate the course of myeloproliferative neoplasms (MPNs). Echocardiography is a useful noninvasive screening test for PH in populations at risk. We aimed to investigate the echocardiographic evidence of PH and clinical characteristics of patients with MPNs.
This study included 197 patients with MPNs (mean age, 59 ± 14 years; females, 53%; mean disease duration, 3.4 ± 2.8 years). Clinical and laboratory characteristics, including JAK2V617F mutation status, were obtained. All participants underwent a comprehensive transthoracic echocardiographic examination. The echocardiographic evidence of PH was defined as systolic pulmonary artery pressure (SPAP) ≥40 mm Hg.
Overall, 11 patients (5.5%) with SPAP ≥40 mm Hg had echocardiographic evidence of PH. Patients with myelofibrosis had echocardiographic evidence of PH more often than patients with other MPNs (p < 0.001). Disease duration since the diagnosis of MPNs was 6.7 ± 4.6 years in the PH group and 3.1 ± 2.5 years in the non-PH group (p < 0.001). There was a weak positive correlation between SPAP values and time since diagnosis (r = 0.236, p =0.001). JAK2V617F mutation was not associated with PH. In multivariate logistic regression analysis, the presence of myelofibrosis (odds ratio [OR]: 22.177, 95% CI: 4.480-109.790, p < 0.001), long disease duration (OR: 1.217, 95% CI: 1.024-1.447, p = 0.026), and high uric acid levels (OR: 1.868, 95% CI: 1.049-3.328, p = 0.034) were found to be related with the echocardiographic evidence of PH. Survival was worse in the PH group (p = 0.0001).
Our results suggest that patients with myelofibrosis are more likely to develop PH than other MPNs patients. Disease duration may predict the development of PH in MPN patients.
肺动脉高压（PH）可使骨髓增殖性肿瘤（MPN）患者的病程复杂化。超声心动图是一种用于检测高危人群 PH 的有用的非侵入性筛查试验。我们旨在研究 MPN 患者的 PH 超声心动图证据和临床特征。
本研究纳入了 197 例 MPN 患者（平均年龄 59±14 岁；女性占 53%；平均疾病病程为 3.4±2.8 年）。获取了包括 JAK2V617F 突变状态在内的临床和实验室特征。所有参与者均接受了全面的经胸超声心动图检查。将收缩期肺动脉压（SPAP）≥40mmHg 定义为存在 PH 的超声心动图证据。
总体而言，11 例（5.5%）患者的 SPAP≥40mmHg，存在 PH 的超声心动图证据。与其他 MPN 患者相比，骨髓纤维化患者更常出现 PH 的超声心动图证据（p＜0.001）。PH 组的 MPN 诊断后病程为 6.7±4.6 年，而非 PH 组为 3.1±2.5 年（p＜0.001）。SPAP 值与诊断后时间之间存在弱正相关（r=0.236，p=0.001）。JAK2V617F 突变与 PH 无关。在多变量逻辑回归分析中，骨髓纤维化的存在（比值比[OR]：22.177，95%置信区间[CI]：4.480-109.790，p＜0.001）、疾病病程长（OR：1.217，95%CI：1.024-1.447，p=0.026）和高尿酸水平（OR：1.868，95%CI：1.049-3.328，p=0.034）与 PH 的超声心动图证据相关。PH 组的生存率更差（p=0.0001）。
我们的结果表明，骨髓纤维化患者比其他 MPN 患者更易发生 PH。疾病病程可能预测 MPN 患者 PH 的发生。
