{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T12:58:28Z","timestamp":1785502708424,"version":"3.56.0"},"reference-count":17,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","license":[{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Friedreich\u2019s ataxia (FA) is a hereditary neuromuscular disorder with cardiac involvement as the leading cause of death. This study examines the progression of cardiac magnetic resonance imaging (CMR) biomarkers in FA-associated cardiomyopathy and their relationships with clinical outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Materials and Methods:<\/jats:title>\n                    <jats:p>This retrospective, single-center study included FA patients with at least one CMR. CMR assessment included segmental late gadolinium enhancement (LGE), left ventricle (LV) ejection fraction (EF), LV wall thickness, native T1 mapping, and extracellular volume (ECV) fraction. Linear mixed-effects regression models were used to assess CMR parameters in relation to each other and time.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      Fifteen patients (mean age: 22.7 \u00b1 7.6 years) and 37 CMRs were evaluated over an average of 11.0 \u00b1 5.0 years from neuromuscular symptom onset. LV thickness was most notable along the septum, while LGE was localized to the LV free wall. Native T1 and ECV were globally elevated. Six (40%) patients demonstrated LGE, and those negative at baseline remained negative. LGE increased by 0.5 segments per year (\n                      <jats:bold>P<\/jats:bold>\n                      = 0.25), and LVEF declined by 0.73%\/year (\n                      <jats:bold>P<\/jats:bold>\n                      = 0.06); however, neither reached statistical significance. LV thickness, native T1, and ECV did not change significantly. Maximal LV thickness correlated with cardiac symptoms (\n                      <jats:bold>P<\/jats:bold>\n                      = 0.04) and cardiac medications (\n                      <jats:bold>P<\/jats:bold>\n                      = 0.024).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>In this exploratory study, CMR demonstrated patterns of septal hypertrophy, lateral wall fibrosis, and trends toward declining ventricular function. Findings were largely nonsignificant and should be considered hypothesis-generating. Larger, multicenter studies are needed to clarify the role of CMR in disease progression and clinical management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/apc.apc_22_26","type":"journal-article","created":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T11:00:18Z","timestamp":1785495618000},"page":"364-373","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Characterizing Friedreich\u2019s ataxia cardiomyopathy with serial cardiac magnetic resonance imagings"],"prefix":"10.4103","volume":"19","author":[{"given":"Megan E.","family":"Gunsaulus","sequence":"first","affiliation":[{"name":"The Heart Center, Nationwide Children\u2019s Hospital, Columbus, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajesh","family":"Krishnamurthy","sequence":"additional","affiliation":[{"name":"The Heart Center, Nationwide Children\u2019s Hospital, Columbus, OH, USA"},{"name":"Department of Radiology, Nationwide Children\u2019s Hospital, Columbus, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chance","family":"Alvarado","sequence":"additional","affiliation":[{"name":"The Heart Center, Nationwide Children\u2019s Hospital, Columbus, OH, USA"},{"name":"Biostatistics Resource, Nationwide Children\u2019s Hospital, Columbus, OH, USA"},{"name":"Center for Biostatistics, The Ohio State University College of Medicine, Columbus, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deipanjan","family":"Nandi","sequence":"additional","affiliation":[{"name":"The Heart Center, Nationwide Children\u2019s Hospital, Columbus, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda H.","family":"Cripe","sequence":"additional","affiliation":[{"name":"The Heart Center, Nationwide Children\u2019s Hospital, Columbus, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan A.","family":"Waldrop","sequence":"additional","affiliation":[{"name":"Center for Gene Therapy, Nationwide Children\u2019s Hospital, Columbus, OH, USA"},{"name":"Department of Pediatrics and Neurology, Ohio State University, Columbus, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily A.","family":"Hayes","sequence":"additional","affiliation":[{"name":"The Heart Center, Nationwide Children\u2019s Hospital, Columbus, OH, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2026,7,31]]},"reference":[{"key":"R1-20260731","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1093\/brain\/104.3.589","article-title":"Friedreich\u2019s ataxia: A clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical 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A statement for healthcare professionals from the cardiac imaging committee of the council on clinical cardiology of the American Heart Association","volume":"105","author":"Cerqueira","year":"2002","journal-title":"Circulation"},{"key":"R12-20260731","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1148\/radiol.2018180226","article-title":"Native T1 and extracellular volume measurements by cardiac MRI in healthy adults: A meta-analysis","volume":"290","author":"Gottbrecht","year":"2019","journal-title":"Radiology"},{"key":"R13-20260731","doi-asserted-by":"crossref","first-page":"e0303969","DOI":"10.1371\/journal.pone.0303969","article-title":"Insights into the effects of Friedreich ataxia on the left ventricle using T1 mapping and late gadolinium enhancement","volume":"19","author":"Peverill","year":"2024","journal-title":"PLoS One"},{"key":"R14-20260731","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.ijcard.2015.05.074","article-title":"The cardiomyopathy in Friedreich\u2019s ataxia-new biomarker for staging cardiac involvement","volume":"194","author":"Weidemann","year":"2015","journal-title":"Int J Cardiol"},{"key":"R15-20260731","doi-asserted-by":"crossref","first-page":"305","DOI":"10.14503\/THIJ-14-4198","article-title":"Serum versus imaging biomarkers in Friedreich ataxia to indicate left ventricular remodeling and outcomes","volume":"43","author":"Mehta","year":"2016","journal-title":"Tex Heart Inst J"},{"issue":"Suppl 1","key":"R16-20260731","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1111\/jnc.12217","article-title":"Cardiomyopathy of Friedreich ataxia","volume":"126","author":"Weidemann","year":"2013","journal-title":"J Neurochem"},{"key":"R17-20260731","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1002\/mds.22864","article-title":"Analysis of the factors influencing the cardiac phenotype in Friedreich\u2019s ataxia","volume":"25","author":"Rajagopalan","year":"2010","journal-title":"Mov Disord"}],"container-title":["Annals of Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_22_26","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T12:00:45Z","timestamp":1785499245000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/apc.apc_22_26"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7]]},"references-count":17,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.4103\/apc.apc_22_26","relation":{},"ISSN":["0974-2069","0974-5149"],"issn-type":[{"value":"0974-2069","type":"print"},{"value":"0974-5149","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,7]]},"assertion":[{"value":"2026-01-10","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-05-04","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}