{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T02:18:51Z","timestamp":1790302731384,"version":"4.1.0"},"reference-count":14,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2008,10,13]],"date-time":"2008-10-13T00:00:00Z","timestamp":1223856000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cardiovasc electrophysiol"],"published-print":{"date-parts":[[2009,3]]},"abstract":"<jats:p> <jats:bold>Adult\u2010Onset KWD and Arrhythmia.<\/jats:bold> We present a case of a 43\u2010year\u2010old male patient with adult onset of spinal muscular atrophy (SMA). The patient first came to our attention with atrioventricular (AV) block. A dual\u2010chamber pacemaker (DDD\u2010PM) was implanted. Four years later, the PM data log showed occurrence of frequent episodes of nonsustained ventricular tachycardia (NSVT). The episodes progressed in duration and frequency. An electrophysiological study revealed prolonged His\u2010ventricular (HV) interval duration and induction of sustained ventricular tachycardia. The patient was successfully upgraded to a prophylactic dual\u2010chamber cardioverter defibrillator. Our case is the first description of a patient with adult\u2010onset SMA (Kugelberg\u2013Welander disease [KWD]) and malignant ventricular arrhythmias. <jats:italic>(J Cardiovasc Electrophysiol, Vol. 20, pp. 342\u2010344, March 2009)<\/jats:italic><\/jats:p>","DOI":"10.1111\/j.1540-8167.2008.01327.x","type":"journal-article","created":{"date-parts":[[2008,10,13]],"date-time":"2008-10-13T19:18:37Z","timestamp":1223925517000},"page":"342-344","source":"Crossref","is-referenced-by-count":15,"title":["Malignant Ventricular Arrhythmia in a Case of Adult Onset of Spinal Muscular Atrophy (Kugelberg\u2013Welander Disease)"],"prefix":"10.1111","volume":"20","author":[{"given":"MARKUS","family":"ROOS","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"ANDREA","family":"SARKOZY","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"GIAN BATTISTA","family":"CHIERCHIA","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"PHILIPPE","family":"DE WILDE","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"ERIC","family":"SCHMEDDING","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"PEDRO","family":"BRUGADA","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,2,20]]},"reference":[{"key":"e_1_2_3_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90460-3"},{"key":"e_1_2_3_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90461-1"},{"key":"e_1_2_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(06)80015-5"},{"key":"e_1_2_3_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90461-1"},{"key":"e_1_2_3_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(99)00016-4"},{"key":"e_1_2_3_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-1004(200003)15:3<228::AID-HUMU3>3.0.CO;2-9"},{"key":"e_1_2_3_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(99)00016-4"},{"key":"e_1_2_3_9_2","doi-asserted-by":"crossref","first-page":"1458","DOI":"10.1136\/bmj.1.5501.1458","article-title":"Kugelberg\u2010Welander syndrome (hereditary proximal spinal muscular atrophy)","volume":"1","author":"Garvie JM","year":"1966","journal-title":"BMJ"},{"key":"e_1_2_3_10_2","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1001\/archneurpsyc.1956.02330230050005","article-title":"Heredofamilial juvenile muscular atrophy simulating muscular dystrophy","volume":"75","author":"Kugelberg E","year":"1956","journal-title":"AMA Arch Neurol Psychiatry"},{"key":"e_1_2_3_11_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.1.5501.1458"},{"key":"e_1_2_3_12_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000441-200612000-00009"},{"key":"e_1_2_3_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9149(76)90473-2"},{"key":"e_1_2_3_14_2","doi-asserted-by":"publisher","DOI":"10.1097\/00000441-200612000-00009"},{"key":"e_1_2_3_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9149(76)90473-2"}],"container-title":["Journal of Cardiovascular Electrophysiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1540-8167.2008.01327.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/j.1540-8167.2008.01327.x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,10]],"date-time":"2023-10-10T22:31:43Z","timestamp":1696977103000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1540-8167.2008.01327.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,2,20]]},"references-count":14,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2009,3]]}},"alternative-id":["10.1111\/j.1540-8167.2008.01327.x"],"URL":"https:\/\/doi.org\/10.1111\/j.1540-8167.2008.01327.x","archive":["Portico"],"relation":{},"ISSN":["1045-3873","1540-8167"],"issn-type":[{"value":"1045-3873","type":"print"},{"value":"1540-8167","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,2,20]]}}}