{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T13:17:11Z","timestamp":1783516631435,"version":"3.55.0"},"reference-count":10,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2026,2,18]],"date-time":"2026-02-18T00:00:00Z","timestamp":1771372800000},"content-version":"vor","delay-in-days":48,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,1,1]],"date-time":"2026-01-01T00:00:00Z","timestamp":1767225600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["associationofanaesthetists-publications.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Anaesthesia Reports"],"published-print":{"date-parts":[[2026,1]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>Emery\u2013Dreifuss muscular dystrophy is a rare inherited neuromuscular disorder characterised by early joint contractures, slowly progressive humero\u2010peroneal weakness and cardiac conduction defects or cardiomyopathy. Although contractures and weakness usually begin in childhood, cardiac complications, such as atrioventricular block, arrhythmias and dilated cardiomyopathy, typically emerge in early adulthood and may cause sudden cardiac death, if unrecognised. Anaesthetic management is challenging due to potential airway complications from cervical contractures, restrictive respiratory physiology and cardiac instability. Pre\u2010operative cardiac and pulmonary assessment is essential. Depolarising neuromuscular blocking agents and volatile anaesthetics are not absolutely contraindicated but are preferably avoided due to the risk of rhabdomyolysis or malignant hyperthermia\u2010like reactions. Total intravenous anaesthesia is preferred to minimise these risks. We report a 27\u2010year\u2010old man with genetically confirmed Emery\u2013Dreifuss muscular dystrophy and severe multisystem involvement who underwent cardiac resynchronisation therapy pacemaker implantation under total intravenous anaesthetic technique with rocuronium and reversal with sugammadex. Anaesthetic management focused on malignant hyperthermia precautions, airway preparation for limited cervical mobility and minimising arrhythmia risk with readiness for external cardiac pacing. The procedure and recovery were uneventful, demonstrating that the total intravenous anaesthetic technique can be an effective technique for patients with Emery\u2013Dreifuss muscular dystrophy undergoing device implantation.<\/jats:p>","DOI":"10.1002\/anr3.70053","type":"journal-article","created":{"date-parts":[[2026,2,18]],"date-time":"2026-02-18T09:15:43Z","timestamp":1771406143000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Anaesthetic considerations for a patient with Emery\u2013Dreifuss muscular dystrophy undergoing cardiac resynchronisation therapy with pacemaker implantation"],"prefix":"10.1002","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-6225-6516","authenticated-orcid":false,"given":"B. S. M.","family":"Ng","sequence":"first","affiliation":[{"name":"Singapore General Hospital  Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Lim","sequence":"additional","affiliation":[{"name":"Singapore General Hospital  Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Valchanov","sequence":"additional","affiliation":[{"name":"Singapore General Hospital  Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,2,18]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000000526"},{"key":"e_1_2_5_3_1","volume-title":"GeneReviews\u00ae","author":"Ben Yaou R","year":"1993"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1097\/EJA.0000000000000672"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF03017922"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF03011813"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2018.21413"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1811697"},{"key":"e_1_2_5_9_1","unstructured":"OrphanAnesthesia.Emery\u2010Dreifuss muscular dystrophy\u2014anaesthesia recommendations.2020 https:\/\/www.orphananesthesia.eu\/rare\u2010diseases\/published\u2010guidelines\/emery\u2010dreifuss\u2010muscular\u2010dystrophy\/1698\u2010emery\u2010dreifuss\u2010muscular\u2010dystrophy\u20103\/file.html."},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/ACO.0000000000000466"},{"key":"e_1_2_5_11_1","doi-asserted-by":"crossref","DOI":"10.12659\/MSM.945675","article-title":"A review of muscle relaxants in anesthesia in patients with neuromuscular disorders including Guillain\u2010Barr\u00e9 syndrome, myasthenia gravis, Duchenne muscular dystrophy, Charcot\u2010Marie\u2010tooth disease, and inflammatory myopathies","volume":"30","author":"Radkowski P","year":"2024","journal-title":"Medical Science Monitor"}],"container-title":["Anaesthesia Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/associationofanaesthetists-publications.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/anr3.70053","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/associationofanaesthetists-publications.onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/anr3.70053","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/associationofanaesthetists-publications.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/anr3.70053","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T12:38:40Z","timestamp":1783514320000},"score":1,"resource":{"primary":{"URL":"https:\/\/associationofanaesthetists-publications.onlinelibrary.wiley.com\/doi\/10.1002\/anr3.70053"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,1]]},"references-count":10,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2026,1]]}},"alternative-id":["10.1002\/anr3.70053"],"URL":"https:\/\/doi.org\/10.1002\/anr3.70053","archive":["Portico"],"relation":{},"ISSN":["2637-3726","2637-3726"],"issn-type":[{"value":"2637-3726","type":"print"},{"value":"2637-3726","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,1]]},"assertion":[{"value":"2026-01-29","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-02-18","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70053"}}