{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T09:46:33Z","timestamp":1785836793656,"version":"3.56.0"},"reference-count":24,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T00:00:00Z","timestamp":1777248000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T00:00:00Z","timestamp":1777248000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Arrhythmias affect approximately half of patients with Costello syndrome (CS, OMIM # 218040), with non\u2010reentrant atrial tachycardia being the most common. This case describes an infant with Costello syndrome carrying the pathogenic\n                    <jats:italic>HRAS<\/jats:italic>\n                    c.34G&gt;A (p.G12S) variant who developed early\u2010onset, drug\u2010refractory multifocal atrial tachycardia (MAT). Despite multiple antiarrhythmic therapies, rhythm control remained inadequate until trametinib was initiated, resulting in rapid resolution of MAT and allowing stepwise discontinuation of adjunct antiarrhythmics. Although MAT did not recur after the MEK inhibitor was stopped, the patient subsequently developed hypertrophic cardiomyopathy only after trametinib was discontinued, suggesting a potential disease\u2010modifying cardioprotective effect during active treatment. This case supports MEK inhibition as a targeted antiarrhythmic strategy in Costello syndrome, with benefits that may extend beyond acute rhythm stabilization to include prevention of cardiac hypertrophy. While spontaneous resolution of MAT can occur in Costello syndrome, the rapid and sustained response in this patient strengthens the hypothesis that trametinib exerts a direct antiarrhythmic effect.\n                  <\/jats:p>","DOI":"10.1002\/ajmg.a.70180","type":"journal-article","created":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T07:52:09Z","timestamp":1777276329000},"page":"2151-2156","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Resolution of Refractory Multifocal Atrial Tachycardia in\n                    <scp>C<\/scp>\n                    ostello Syndrome Using Trametinib: A Case Supporting\n                    <scp>MEK<\/scp>\n                    Inhibitors as Targeted, Specific Antiarrhythmic"],"prefix":"10.1002","volume":"200","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6226-3576","authenticated-orcid":false,"given":"Vanina","family":"Taliercio","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annabelle","family":"Wilcox","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stacey","family":"Cole","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9548-4188","authenticated-orcid":false,"given":"Josue Flores","family":"Daboub","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Medical Genetics University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jos\u00e9 E.","family":"Morales Moreno","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of General Pediatrics University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kasey G.","family":"Andrews","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of General Pediatrics University of Utah  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. 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