Extract

A 68-year-old woman with a history of pulmonary hypertension associated with systemic lupus erythematosus was referred to our department for worsening dyspnoea and back pain. Her oxygen saturation was 84% on room air and physical examination showed decreased pulmonary sounds in the left lower lung. Chest radiograph showed an increased cardiothoracic ratio, remarkable left second arc protrusion, and right pulmonary artery (PA) dilation (Panel A-1). Transthoracic echocardiogram revealed preserved left ventricular ejection fraction, severe pulmonary regurgitation, and dilation of the main PA (Panels B and C, see Supplementary data online, Movies S1 and S2). Chest computed tomography revealed a giant PA aneurysm (106 mm in minimum diameter) compressing the left pulmonary vein and left main bronchial tube (Panels D and E). Her mean PA pressure was 35 mmHg and pulmonary capillary wedge pressure was 20 mmHg.

She was treated with non-invasive positive pressure ventilation and diuretics, but her back pain worsened and the PA aneurysm was exacerbated on chest X-ray after admission (Panel A-2). She underwent emergent aneurysm repair with synthetic grafts and pulmonary valve replacement. Her post-operative course was uneventful and without any complications.

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