An 46-year-old congenitally deaf woman was presented to our hospital with chest tightness and edema of both lower extremities 5 days ago. Except for systolic ejection murmur in the auscultation area of pulmonary valve, her physical examination was unremarkable. The laboratory results indicated N-terminal pro-B-type natriuretic peptide of 1060.00 pg/ml (< 125 pg/ml), a D-dimer of 0.91 mg/L (< 0.55 mg/L), an erythrocyte sedimentation rate in first hour (ESR) of 60 mm/h (< 26 mm/h) and a C-reactive protein concentration (CRP) of 15.77 mg/dL (< 5 mg/dL). M. Tuberculosis interferon-γ release assay (TB-IGRA) and Treponema pallidum (TPPA) are negative. Electrolytes, infectious markers, and autoantibodies were normal. Pulmonary computed tomography angiography (CTA) was scanned on GE Light Speed VCT with injection 3.0 ml/s contrast. It revealed limited thick walls of both sides proximal pulmonary artery and severe stenosis of pulmonary artery lumen, with the normal images of pulmonary parenchymal. The left side pulmonary artery stenosis was even worse than the right side (5.4 mm vs. 6.3 mm). The images of the aortic vessel and head and neck were unremarkable. On echocardiogram revealed stenosis of the left and right proximal pulmonary artery with narrowest diameter of 5.4 mm and 6.3 mm, respectively, with severe pulmonary hypertension about 100 mmHg. The maximum flow velocity of left and right pulmonary artery was about 3.7 m/s and 3.5 m/s. The maximum pressure difference of left and right pulmonary artery was about 55.7 mmHg and 48.0 mmHg. The patient undergone pulmonary endarterectomy and pulmonary angioplasty. Then, about a month later, she died of heart failure. Postoperative pathology assessment revealed vitreous collagen fibers, mucoid degeneration and inflammatory cell such as lymphocytes infiltration could be seen in the interstitium, which indicated Takayasu arteritis.