Cardiac manifestation of polyarteritis nodosa

Benjamin Peters, Jochen von Spiczak, Frank Ruschitzka, Oliver Distler, Robert Manka, Hatem Alkadhi
2018 European Heart Journal  
A 24-year-old woman patient presented with an 18-month history of fatigue, weight loss, transient exanthema, fever, and occasional palpitations. Dyspnoea, orthopnoea, and chest pain were denied. Clinical examination revealed tachycardia with a heart rate up to 122 b.p.m. Chest X-ray showed left-sided pleural effusion. Laboratory examination indicated increased acute-phase reactants (erythrocyte sedimentation rate 102 mm/h, c-reactive protein 65 mg/dL), normal cardiac biomarkers, and negative
more » ... umatologic antibodies (ANCA, ANA, and rheumatoid factor). Magnetic resonance (MR) angiography revealed multiple renovisceral aneurysms. Based on these findings the patient was diagnosed with polyarteritis nodosa (PAN). Transoesophageal echocardiography showed a decreased ejection fraction (35%), hypokinesia in the mid-ventricular anterolateral segment, and akinesia in anteroseptal and inferoseptal mid-ventricular to apical segments. Cardiac MR confirmed the wall motion abnormalities (see Supplementary material online, Video S1), showed myocardial oedema suggestive of acute inflammation (Panel A) and transmural late gadolinium enhancement (LGE) corresponding to the vascular distribution of the left anterior descending artery (LAD) (Panel B). Coronary computed tomography (CT) angiography showed aneurysms of the coronary arteries (Panels C-F) and of the right internal mammary artery (Panel F). Due to the extensive cardiac disease, Lisinopril, acetylsalicylic acid, and rivaroxaban were added to the immunosuppressive treatment. The patient is currently clinically stable and has a normal ejection fraction. Polyarteritis nodosa is a systemic inflammatory necrotizing vasculitis of medium-sized arteries. Congestive heart failure in PAN can be caused by renal artery vasculitis and nephropathy or by coronary involvement and myocardial infarction. The distribution of LGE in our patient suggests thromboembolic infarctions from the coronary aneurysms as the most likely aetiology. Cardiac MR imaging: short-axis T2w black blood image (Panel A) shows myocardial oedema anterior to anterolateral mid-ventricular (arrow). Short-axis LGE T1w image obtained 10 min after administration of intravenous gadolinium (Panel B) shows transmural late enhancement anteroseptal and anterior mid-ventricular (arrows). Coronary CT angiography: curved planar reformation shows multiple aneurysms of the LAD (Panel C) and D1 (Panel D). Three-dimensional cinematic rendering of the cardiac CT (Panels E and F). Panel E illustrates the multiple aneurysms of the LAD (white arrow) and D1 (black arrow). Panel F shows aneurysm of the RCA (black arrows) and of the right internal mammary artery (white arrow). D1, first diagonal branch; LAD, left anterior descending artery; RCA, right coronary artery.
doi:10.1093/eurheartj/ehy090 pmid:29481598 fatcat:hhbxsxwaz5h4lele2bmm5ei6wa