Yamashita Shuhei, Hiraide Takahiro, Shiraishi Yasuyuki, Katsumata Yoshinori, Kataoka Masaharu, Fukui Shogo, Kawakami Michiyuki, Yuasa Shinsuke, Okamoto Shinichiro, Fukuda Keiichi, Ieda Masaki
Department of Cardiology, Keio University School of Medicine, Shinanomachi 35, Shinjuku-ku, Tokyo, 160-8582, Japan.
Institute for Integrated Sports Medicine, Keio University School of Medicine, Tokyo, Japan.
J Med Case Rep. 2025 May 6;19(1):209. doi: 10.1186/s13256-025-05221-2.
Dasatinib-related pulmonary arterial hypertension is a rare complication of chronic therapy for hematological malignancies. Pulmonary hypertension often persists despite drug discontinuation and might require vasodilators. Normalizing pulmonary hemodynamics and avoiding the long-term use of vasodilators is challenging.
Patient was a 55-year-old Japanese man complaining of progressive dyspnea on effort and fatigue. He had a history of hypertension and chronic myeloid leukemia treated with dasatinib. He was diagnosed with dasatinib-related pulmonary arterial hypertension by a right heart catheterization at rest, demonstrating a mean pulmonary artery pressure of 31 mmHg and a normal pulmonary arterial wedge pressure of 6 mmHg. Symptoms and hemodynamics significantly improved after the discontinuation of dasatinib and the initiation of upfront combination therapy of vasodilators. An exercise right heart catheterization, performed more than 2 years after the initiation of vasodilators, showed a mean pulmonary artery pressure of 15 mmHg at rest and 29 mmHg at peak exercise (normal reference value, < 30 mmHg), suggesting normal pulmonary microcirculation. On the basis of these findings, pulmonary vasodilators were discontinued. Notably, a repeat exercise right heart catheterization demonstrated preserved pulmonary microcirculation, and the patient has remained asymptomatic for more than 2 years after discontinuing pulmonary-arterial-hypertension-targeted therapy.
The evaluation of pulmonary microcirculation by exercise right heart catheterization can be useful for withdrawing pulmonary vasodilators safely in the management of patients with dasatinib-related pulmonary arterial hypertension.
达沙替尼相关的肺动脉高压是血液系统恶性肿瘤慢性治疗中一种罕见的并发症。尽管停用药物，肺动脉高压仍常持续存在，可能需要使用血管扩张剂。使肺血流动力学正常化并避免长期使用血管扩张剂具有挑战性。
患者为一名55岁的日本男性，主诉进行性劳力性呼吸困难和疲劳。他有高血压病史，曾用达沙替尼治疗慢性粒细胞白血病。静息状态下经右心导管检查诊断为达沙替尼相关的肺动脉高压，平均肺动脉压为31 mmHg，肺动脉楔压正常为6 mmHg。停用达沙替尼并开始早期联合使用血管扩张剂治疗后，症状和血流动力学显著改善。在开始使用血管扩张剂2年多后进行的运动右心导管检查显示，静息时平均肺动脉压为15 mmHg，运动峰值时为29 mmHg（正常参考值，<30 mmHg），提示肺微循环正常。基于这些发现，停用了肺血管扩张剂。值得注意的是，重复进行的运动右心导管检查显示肺微循环得以保留，并且在停用肺动脉高压靶向治疗后，患者已保持无症状超过2年。
通过运动右心导管检查评估肺微循环对于在达沙替尼相关肺动脉高压患者的管理中安全停用肺血管扩张剂可能是有用的。
