A 40-year-old woman was diagnosed as having Takayasu arteritis with pulmonary hypertension (TA-PH) and administered prednisolone 12 years ago. Despite treatment with oxygen, PH-specific, and antiplatelet therapy, she presented with progressive right-sided heart failure of World Health Organization functional class (WHO-FC) III and pretibial edema. Echocardiography and magnetic resonance revealed a dilated right-sided heart with tricuspid regurgitation (Figure A,B). Cardiac catheterization revealed mean pulmonary arterial pressure of 40 mmHg, right atrial pressure of 29 mmHg, and reduced mixed venous oxygen saturation (SVO2, 32.4%). Computed tomography (CT) and angiography showed diffuse stenoses/occlusions and aneurysms in the pulmonary arteries (Figure C,D), representing TA-PH. We escalated the PH-specific therapies and prednisolone with subcutaneous tocilizumab under inotropic agents. After confirming no accumulation on 18F-fluorodeoxyglucose positron emission tomography-CT (FDG-PET/CT) (Figure E) and switching antiplatelet to anticoagulation therapy, she underwent 8 balloon pulmonary angioplasty (BPA) sessions using intravascular ultrasound and the Navvus®II micro-Catheter (ACIST Corporation) (Figure F–L) to avoid overdilation of the external elastic membrane, resulting in hemodynamic and symptomatic amelioration (mPAP, 31 mmHg, SVO2, 71.1%, WHO-FC II). Multimodality imaging was useful to evaluate advanced HF and diffusely affected TA-PH lesions, and to guide successful BPA in long-standing TA-PH.
