Is it truly chronic thromboembolic pulmonary hypertension? A case of pulmonary hypertension with proptosis

A 42-year-old man presented with an 11-month history of progressive exertional dyspnoea and leg swelling. His 6 min walk distance (6MWD) was 345 m. Three months before admission, blood tests revealed an elevated D-dimer level of 3.56 mg/L (<0.5 mg/L), B-type natriuretic peptide (BNP) level of 860 pg/mL (<100 pg/mL) and creatinine level of 251.70 µmol/L (35–106 μmol/L). No evidence of deep vein thrombosis was found on lower extremity ultrasonography. Echocardiography showed right heart enlargement with a right-to-left ventricular ratio of 2.1, right ventricular hypertrophy (wall thickness, 5.4 mm) and an increased estimated systolic pulmonary artery pressure (PAP) of 65 mmHg. A pulmonary ventilation–perfusion scan identified mismatched perfusion deficits, and CT pulmonary angiography (CTPA) showed filling defects primarily in the left pulmonary arterial trunk. Upon these findings, therapy with the anticoagulant rivaroxaban was immediately initiated. Despite a partial reduction in the D-dimer level to 1.67 mg/L, the patient showed no significant improvement on repeat CTPA (figure 1). One week before admission, a right heart catheterisation confirmed precapillary pulmonary hypertension (PH) with a mean PAP of 39 mmHg, pulmonary artery wedge pressure of 7 mmHg …