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Recent advances in cardiac imaging allow the detection of cardiac amyloidosis. This study aimed to explore cardiomyopathy by cardiac imaging and its clinical correlates with polyneuropathy in late\u2010onset ATTRv\u2010PN.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Polyneuropathy was assessed by intraepidermal nerve fiber (IENF) density, nerve conduction study (NCS), autonomic function tests, quantitative sensory testing, and clinical questionnaires. Cardiomyopathy was evaluated by echocardiography,<jats:sup>99m<\/jats:sup>Tc\u2010pyrophosphate (PYP) single\u2010photon emission computed tomography (SPECT) imaging, cardiac magnetic resonance imaging (CMR), and serum Pro\u2010B\u2010type natriuretic peptide. Healthy controls and patients with Brugada syndrome were enrolled for comparison of CMR.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fifty late\u2010onset ATTRv\u2010PN patients (38 men, 46 with p. A117S mutation), aged 63.7\u2009\u00b1\u20095.5\u2009years, of polyneuropathy disability stage 1\u20134 were enrolled. All patients presented polyneuropathy in NCS, and 74.5% of patients had reduced IENF density in distal legs. All patients showed significant radiotracer uptake in the heart on<jats:sup>99m<\/jats:sup>Tc\u2010PYP SPECT imaging, and 87.8% of patients had abnormally increased left ventricular (LV) septum thickness on echocardiography. CMR showed longer myocardial native T1, larger extracellular volume, greater LV mass index, and higher LV mass to end\u2010diastolic volume ratio in ATTRv\u2010PN patients than healthy controls and patients with Brugada syndrome. These CMR parameters were associated with skin denervation, absent sympathetic skin responses, elevated thermal thresholds, worsened NCS profiles, and functional deficits of polyneuropathy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Late\u2010onset ATTRv\u2010PN coexisted with cardiomyopathy regardless of the clinical severity of polyneuropathy. The cardiac amyloid burden revealed by CMR was correlated with pathophysiology and clinical disability of nerve degeneration.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.51635","type":"journal-article","created":{"date-parts":[[2022,8,10]],"date-time":"2022-08-10T04:29:11Z","timestamp":1660105751000},"page":"1359-1369","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Cardiomyopathy correlates to nerve damage in p.<scp>A117S<\/scp>late\u2010onset transthyretin amyloid polyneuropathy"],"prefix":"10.1002","volume":"9","author":[{"given":"Yen\u2010Hung","family":"Lin","sequence":"first","affiliation":[{"name":"Department of Internal Medicine National Taiwan University Hospital Taipei 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