Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty

• Main Authors: Tomohiro Yamaguchi, Yasuhiro Izumiya, Hiroya Hayashi, Yoshitaka Ichikawa, Hirotoshi Ishikawa, Atsushi Shibata, Takanori Yamazaki, Minoru Yoshiyama
• Format: Article
• Language: English
• Published: Elsevier 2020-01-01
• Series: Respiratory Medicine Case Reports
• Subjects: Chronic thromboembolic pulmonary hypertension; Essential thrombocythemia; Balloon pulmonary angioplasty
• Online Access: http://www.sciencedirect.com/science/article/pii/S2213007120304494
• ISSN: 2213-0071

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.