Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty.
Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty.
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DOI：
10.1016/j.rmcr.2020.101235
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发表时间：
2020
影响因子：
1.1
通讯作者：
Yoshiyama M
中科院分区：
文献类型：
--
作者：
Yamaguchi T;Izumiya Y;Hayashi H;Ichikawa Y;Ishikawa H;Shibata A;Yamazaki T;Yoshiyama M
一名71岁女性被诊断为慢性血栓栓塞性肺动脉高压（CTEPH）伴原发性血小板增多症（ET），JAK 2 V617 F突变。血液检查显示血小板计数显著增加（132.9 × 10^4/μL），血浆BNP水平升高（125.1pg/mL）。右心导管检查（RHC）显示平均肺动脉压（mPAP）显著升高，为43 mmHg。我们给予她7.5 mg利奥西呱、口服抗凝剂、吸氧治疗CTEPH，并给予阿那格雷治疗ET。我们在9个月内进行了4次球囊肺血管成形术（BPA），RHC显示最终BPA手术后（未使用利奥西呱）血流动力学成功改善（mPAP = 21 mmHg）。最终BPA手术后6个月，RHC显示mPAP（21 mmHg）稳定改善，无需利奥西呱和吸氧。她没有吸氧和PH靶向治疗，生活得很好。这是BPA成功治疗1例伴有JAK 2 V617 F突变的CTEPH合并ET患者的首次报告。
A-71-year-old woman was diagnosed as chronic thromboembolic pulmonary hypertension (CTEPH) accompanied by essential thrombocythemia (ET) with JAK2 V617F mutation. Blood test showed remarkable increase of platelet counts (132.9 × 10^4/μL) and elevated plasma BNP level (125.1pg/mL). Right heart catheterization (RHC) revealed remarkably high mean pulmonary arterial pressure (mPAP) of 43 mmHg. We gave her riociguat of 7.5mg, oral anticoagulants, oxygen inhalation for CTEPH, and anagrelide for ET. We performed 4 sessions of balloon pulmonary angioplasty (BPA) in 9 months RHC revealed successful hemodynamic improvement (mPAP = 21 mmHg) after final BPA procedure without riociguat. At six month later after final BPA procedure, RHC showed steadily improvement of mPAP (21 mmHg) without riociguat and oxygen inhalation. She lives well without oxygen inhalation and PH targeted therapy. This is the first report of successful treatment for a patient with CTEPH comorbid with ET with JAK2 V617F mutation by BPA.
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