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

Hayashi, Hiroya ; Izumiya, Yasuhiro ; et al.

In: Respiratory Medicine Case Reports, Jg. 31 (2020)

Online unknown

Zugriff:

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.

Titel:	Successful treatment for a patient with chronic thromboembolic pulmonary hypertension comorbid with essential thrombocythemia with the JAK2 V617F mutation by balloon pulmonary angioplasty
Autor/in / Beteiligte Person:	Hayashi, Hiroya ; Izumiya, Yasuhiro ; Ichikawa, Yoshitaka ; Yamaguchi, Tomohiro ; Shibata, Atsushi ; Yoshiyama, Minoru ; Ishikawa, Hirotoshi ; Yamazaki, Takanori
Link:	View record at OpenAIRE (Volltext) http://www.sciencedirect.com/science/article/pii/S2213007120304494
Zeitschrift:	Respiratory Medicine Case Reports, Jg. 31 (2020)
Veröffentlichung:	Elsevier, 2020
Medientyp:	unknown
ISSN:	2213-0071 (print)
Schlagwort:	Pulmonary and Respiratory Medicine medicine.medical_specialty ET, essential thrombocythemia BPA, balloon pulmonary angioplasty PEA, pulmonary endarterectomy mPAP, mean pulmonary arterial pressure medicine.medical_treatment PH, pulmonary hypertension Chronic thromboembolic pulmonary hypertension Hemodynamics Case Report RHC, right heart catheterization Essential thrombocythemia Balloon 6MWT, 6-min walking test Riociguat 03 medical and health sciences 0302 clinical medicine Angioplasty Internal medicine medicine Blood test BNP, Brain natriuretic peptide Balloon pulmonary angioplasty Platelet lcsh:RC705-779 CTEPH, chronic thromboembolic pulmonary hypertension medicine.diagnostic_test business.industry Anagrelide lcsh:Diseases of the respiratory system medicine.disease 030228 respiratory system 030220 oncology & carcinogenesis Cardiology business medicine.drug
Sonstiges:	Nachgewiesen in: OpenAIRE Sprachen: English Language: English Rights: OPEN

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