{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T05:21:49Z","timestamp":1780636909295,"version":"3.54.1"},"reference-count":56,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2025,2,19]],"date-time":"2025-02-19T00:00:00Z","timestamp":1739923200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Background\/Objectives: Hereditary transthyretin-mediated amyloidosis (ATTRv) is a rare disease characterized by the deposition of amyloid in the heart and peripheral nerves, particularly affecting small fibers. This study aims to evaluate autonomic cardiac involvement in ATTRv. Methods: Twelve patients with ATTRv and twelve sex- and age-matched healthy subjects underwent 123I-mIBG scintigraphy to evaluate the early and late heart-to-mediastinum ratio (eH\/M and lH\/M), 99mTc-HDP bone scan scintigraphy, and neurophysiological assessments. Data were analyzed in relation to functional cardiac and neurologic scales (NYHA and FAP scales). Results: Patients with ATTRv exhibited significant cardiac denervation, as demonstrated by the reduction in early and late H\/M ratios compared to the control group (eH\/M: 1.48 \u00b1 0.08 vs. 1.89 \u00b1 0.05, p &lt; 0.001; lH\/M: 1.39 \u00b1 0.08 vs. 2.01 \u00b1 0.05, p &lt; 0.001). Values of eH\/M and lH\/M &lt; 1.6 effectively differentiated patients with ATTRv from the healthy controls. Cardiac denervation correlated with interventricular septal thickness and the Perugini score but was not related to neurophysiological assessments or NYHA and FAP scales. Conclusions: Ultimately, 123I-mIBG scintigraphy is an effective tool for assessing cardiac denervation in patients with ATTRv.<\/jats:p>","DOI":"10.3390\/diagnostics15040508","type":"journal-article","created":{"date-parts":[[2025,2,19]],"date-time":"2025-02-19T11:49:27Z","timestamp":1739965767000},"page":"508","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Exploring Cardiac Sympathetic Denervation in Transthyretin-Mediated Hereditary Amyloidosis (ATTRv): Insights from 123I-mIBG Scintigraphy"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3216-4838","authenticated-orcid":false,"given":"Maria Silvia De","family":"Feo","sequence":"first","affiliation":[{"name":"Department of Radiological Sciences, Oncology and Anatomo Pathology, Sapienza University of Rome, 00151 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9424-7730","authenticated-orcid":false,"given":"Chiara","family":"Cambieri","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Galosi","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3497-4236","authenticated-orcid":false,"given":"Viviana","family":"Frantellizzi","sequence":"additional","affiliation":[{"name":"Department of Radiological Sciences, Oncology and Anatomo Pathology, Sapienza University of Rome, 00151 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Chimenti","sequence":"additional","affiliation":[{"name":"Department of Clinical, Anesthesiological and Cardiovascular Sciences, I School of Medicine, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7539-505X","authenticated-orcid":false,"given":"Marco","family":"Luigetti","sequence":"additional","affiliation":[{"name":"Dipartimento di Neuroscienze, Universit\u00e0 Cattolica del Sacro Cuore, 00168 Rome, Italy"},{"name":"Dipartimento di Neuroscienze, Organi di Senso e Torace, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1490-0320","authenticated-orcid":false,"given":"Maria Ausilia","family":"Sciarrone","sequence":"additional","affiliation":[{"name":"Dipartimento di Neuroscienze, Universit\u00e0 Cattolica del Sacro Cuore, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Graziani","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Medicine, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1267-864X","authenticated-orcid":false,"given":"Luca","family":"Leonardi","sequence":"additional","affiliation":[{"name":"Neuromuscular and Rare Disease Centre, Neurology Unit, Sant\u2019Andrea Hospital, 00189 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4872-7981","authenticated-orcid":false,"given":"Beatrice","family":"Musumeci","sequence":"additional","affiliation":[{"name":"Department of Clinical and Molecular Medicine, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4323-3598","authenticated-orcid":false,"given":"Laura","family":"Libonati","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Federica","family":"Moret","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edoardo","family":"D\u2019Andrea","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matteo","family":"Di Giulio","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1830-2886","authenticated-orcid":false,"given":"Matteo","family":"Garibaldi","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mental Health and Sensory Organs (NESMOS), Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-2572-5801","authenticated-orcid":false,"given":"Francesca","family":"Forcina","sequence":"additional","affiliation":[{"name":"Department of Neurology, Mental Health and Sensory Organs (NESMOS), Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Truini","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4690-1528","authenticated-orcid":false,"given":"Giuseppe","family":"De Vincentis","sequence":"additional","affiliation":[{"name":"Department of Radiological Sciences, Oncology and Anatomo Pathology, Sapienza University of Rome, 00151 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5365-3417","authenticated-orcid":false,"given":"Maurizio","family":"Inghilleri","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"},{"name":"IRCCS Neuromed, 86077 Pozzilli, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8897-3736","authenticated-orcid":false,"given":"Marco","family":"Ceccanti","sequence":"additional","affiliation":[{"name":"Department of Human Neuroscience, Sapienza University of Rome, 00185 Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,2,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1080\/13506129.2020.1794807","article-title":"ATTRv Amyloidosis Italian Registry: Clinical and Epidemiological Data","volume":"27","author":"Russo","year":"2020","journal-title":"Amyloid"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1212\/01.WNL.0000132966.36437.12","article-title":"Pathology of Early- vs Late-Onset TTR Met30 Familial Amyloid Polyneuropathy","volume":"63","author":"Koike","year":"2004","journal-title":"Neurology"},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Luigetti, M., Guglielmino, V., Antonini, G., Casali, C., Ceccanti, M., Chiappini, M.G., De Giglio, L., Di Lazzaro, V., Di Muzio, A., and Goglia, M. 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