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Geoffrey C. Colin, Anne-Catherine Pouleur, Chantal Lefebvre, Benoit Ghaye, Unexpected ‘pulmonary hypertension’ in a patient with granulomatosis with polyangiitis, European Heart Journal, Volume 35, Issue 44, 21 November 2014, Page 3141, https://doi.org/10.1093/eurheartj/ehu360
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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 ventricle and right atrium. Whereas pulmonary valve appeared normal, acceleration of flow with high peak velocities was depicted in the supra-valvular area (Supplementary Data) with a gradient pressure of 53 mmHg in regard of a parietal thickening, confirmed by a transoesophageal echocardiography (Panel A, asterisk and Panel B). MDCT angiography confirmed a short and severe stenosis of the main pulmonary artery (PA) 10 mm above the pulmonary valve (Panel C, arrows) focused on an asymmetric thickening of the PA right wall (Panel D, arrow). All c-ANCA titre tests remained within normal values during the 6 years of the disease. A PA localization of GPA was hypothesized. Provisional stenting of the PA with additional transcatheter pulmonary valve placement was considered a therapeutic option, but considering the worsening of the symptoms despite immunosuppressive treatment and the absence of definite exclusion of a tumoural origin, surgery was performed. An inflammatory arterial wall was resected (Panel E, arrows) and a stentless 27 mm bioprothesis was inserted. Histopathology confirmed the PA arteritis with oedema and diffuse lymphocytic and histocytic infiltration (Panel F, arrows).
