Unexpected 'pulmonary hypertension' in a patient with granulomatosis with polyangiitis.

A 62-year-old woman presented with increasing dyspnoea for 3 months. She had a known history of granulomatosis with polyangiitis (GPA) that was diagnosed upon biopsy of an interauricular septal mass revealed by a third-degree AV block 6 years earlier leading to implantation of a pacemaker. At current admission, transthoracic echocardiography showed a maximal systolic pressure gradient of 65 mmHg between the right …