{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T16:42:49Z","timestamp":1786725769703,"version":"build-2736575974"},"reference-count":8,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/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":[[2026,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Erdheim\u2013Chester disease (ECD) is a rare systemic non-Langerhans cell histiocytosis of unknown etiology characterized by multiorgan xanthomatous infiltration by foamy histiocytes. Cardiovascular involvement in ECD is frequent at the level of the right atrium and superior vena cava. We present the case of a 67-year-old woman with a history of ECD and cardiac involvement, referred to our attention following syncopal episodes and paroxysmal atrial fibrillation alternating with phases of marked sinus bradycardia and asystolic pauses. Transesophageal echocardiogram and cardiac magnetic resonance imaging confirmed the increased wall thickness and the marked reduction of the internal diameter of the superior vena cava and of the right atrium. This can be a major obstacle to the implantation of a bicameral pacemaker, necessary for the sick sinus syndrome. Despite this problem, we decided to try an implant with a traditional approach from the left axillary vein; we followed the procedure with fluoroscopic and ultrasound guidance that was very useful in crossing the stenotic superior vena cava and in the positioning of the active fixation electrode in the right atrial appendage. This case confirms how a multimedial imaging approach is crucial to organize and best perform electrostimulation procedures in particular anatomical contexts such as in ECD with cardiac involvement, minimizing the risks.<\/jats:p>","DOI":"10.4103\/jcecho.jcecho_163_25","type":"journal-article","created":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T09:02:08Z","timestamp":1778144528000},"page":"322-324","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Challenging Pacemaker Implant in Sinus Node Dysfunction and Cardiac Involvement in Erdheim\u2013Chester Disease: A Multimodality Imaging-guided Procedure"],"prefix":"10.4103","volume":"36","author":[{"given":"Stefano","family":"Maff\u00e8","sequence":"first","affiliation":[{"name":"Division of Cardiology, SS Trinita\u2019 Hospital, ASL NO, Borgomanero (Novara), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Paffoni","sequence":"additional","affiliation":[{"name":"Division of Cardiology, SS Trinita\u2019 Hospital, ASL NO, Borgomanero (Novara), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Prenna","sequence":"additional","affiliation":[{"name":"Division of Cardiology, SS Trinita\u2019 Hospital, ASL NO, Borgomanero (Novara), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierfranco","family":"Dellavesa","sequence":"additional","affiliation":[{"name":"Division of Cardiology, SS Trinita\u2019 Hospital, ASL NO, Borgomanero (Novara), Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2026,5,6]]},"reference":[{"key":"R1-20260720","doi-asserted-by":"crossref","first-page":"1929","DOI":"10.1182\/blood.2019003507","article-title":"Erdheim-Chester disease: Consensus recommendations for evaluation, diagnosis, and treatment in the molecular era","volume":"135","author":"Goyal","year":"2020","journal-title":"Blood"},{"key":"R2-20260720","first-page":"1","article-title":"Cardiac involvement in Erdheim-Chester disease: MRI findings and literature revision","volume":"4","author":"Ponsiglione","year":"2015","journal-title":"Acta Radiol Open"},{"key":"R3-20260720","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1097\/00005792-199605000-00005","article-title":"Erdheim-chester disease. Clinical and radiologic characteristics of 59 cases","volume":"75","author":"Veyssier-Belot","year":"1996","journal-title":"Medicine (Baltimore)"},{"key":"R4-20260720","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1097\/01.md.0000145368.17934.91","article-title":"Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: Report of 6 new cases and a literature review","volume":"83","author":"Haroche","year":"2004","journal-title":"Medicine (Baltimore)"},{"key":"R5-20260720","doi-asserted-by":"crossref","first-page":"1959","DOI":"10.1016\/j.jaccas.2020.08.014","article-title":"Erdheim-Chester disease: A case of right atrial involvement and superior vena cava stenosis","volume":"2","author":"Tao","year":"2020","journal-title":"JACC Case Rep"},{"key":"R6-20260720","doi-asserted-by":"crossref","first-page":"e412","DOI":"10.1161\/CIRCULATIONAHA.106.656439","article-title":"Images in cardiovascular medicine. Magnetic resonance imaging guiding pacemaker implantation for severe sinus node dysfunction due to cardiac involvement in Erdheim-Chester disease","volume":"115","author":"Elgeti","year":"2007","journal-title":"Circulation"},{"key":"R7-20260720","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1111\/pace.14520","article-title":"Leadless pacemakers as a new alternative for pacemaker lead-related superior vena cava syndrome: A case report","volume":"45","author":"Curnis","year":"2022","journal-title":"Pacing Clin Electrophysiol"},{"key":"R8-20260720","article-title":"A challenging pacemaker implantation in the presence of a giant right coronary aneurysm compressing the right atrium","volume":"92","author":"Maff\u00e8","year":"2021","journal-title":"Monaldi Arch Chest Dis"}],"container-title":["Journal of Cardiovascular Echography"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/jcecho.jcecho_163_25","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,14]],"date-time":"2026-08-14T16:25:20Z","timestamp":1786724720000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/jcecho.jcecho_163_25"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5]]},"references-count":8,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.4103\/jcecho.jcecho_163_25","relation":{},"ISSN":["2211-4122","2347-193X"],"issn-type":[{"value":"2211-4122","type":"print"},{"value":"2347-193X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5]]},"assertion":[{"value":"2025-11-09","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-27","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}