{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T12:22:22Z","timestamp":1756902142590,"version":"3.44.0"},"reference-count":15,"publisher":"The Pediatric Pharmacy Association","issue":"7","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023]]},"abstract":"<jats:p>We present a case of bidirectional ventricular tachycardia in a 15-year-old boy asymptomatic for arrhythmias, whose major complaint was muscle weakness. At our first evaluation he was receiving sotalol for his ventricular arrhythmias. In addition to bidirectional tachycardia, electrocardiogram during sinus rhythm showed prominent U waves and prolonged QT-U interval. These electrocardiographic signs, along with the evidence of clinodactyly and mild hypertelorism, led us to the diagnosis of Andersen-Tawil syndrome, confirmed by genetic analysis that revealed a \u201c<jats:italic>de novo<\/jats:italic>\u201d missense mutation of KCNJ2 gene. Monotherapy with flecainide was rapidly effective and almost eliminated ventricular arrhythmias. After a 4-year follow-up there were no adverse events, flecainide has been well tolerated without significant modification of the QRS or repolarization, and ventricular arrhythmias have not been relapsed to date. The case highlights the importance of a correct clinical diagnosis, which is crucial for the optimal selection of the most appropriate drug therapy, which is expected not to be harmful, before being beneficial.<\/jats:p>","DOI":"10.5863\/1551-6776-28.7.662","type":"journal-article","created":{"date-parts":[[2023,11,28]],"date-time":"2023-11-28T08:58:54Z","timestamp":1701161934000},"page":"662-666","source":"Crossref","is-referenced-by-count":0,"title":["Bidirectional Ventricular Tachycardia and Prominent U Waves: Look at Fingers and Muscles and Use Flecainide"],"prefix":"10.5863","volume":"28","author":[{"given":"Lilia","family":"Oreto","sequence":"first","affiliation":[{"name":"Mediterranean Pediatric Cardiology Center (LO, VGR, AD), Bambino Ges\u00f9 Children\u2019s Hospital, Taormina, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvana","family":"Briuglia","sequence":"additional","affiliation":[{"name":"Department of Biomedical, Dental, Morphological and Functional Imaging Sciences (SB, APC), University of Messina, Messina, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna Paola","family":"Capra","sequence":"additional","affiliation":[{"name":"Department of Biomedical, Dental, Morphological and Functional Imaging Sciences (SB, APC), University of Messina, Messina, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Victoria Garcia","family":"Ruiz","sequence":"additional","affiliation":[{"name":"Mediterranean Pediatric Cardiology Center (LO, VGR, AD), Bambino Ges\u00f9 Children\u2019s Hospital, Taormina, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alfredo","family":"Di Pino","sequence":"additional","affiliation":[{"name":"Mediterranean Pediatric Cardiology Center (LO, VGR, AD), Bambino Ges\u00f9 Children\u2019s Hospital, Taormina, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3883","reference":[{"key":"i2331-348X-28-7-662-b1","series-title":"Europace","first-page":"1337","article-title":"Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement","volume":"15","author":"Brugada","year":"2013","unstructured":"Brugada\n              J\n            , BlomN, Sarquella-BrugadaG,\n          . Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement. Europace2013;15:1337\u20131382."},{"issue":"(21)","key":"i2331-348X-28-7-662-b2","series-title":"Circulation","first-page":"2720","article-title":"Electrocardiographic features in Andersen-Tawil syndrome patients with KCNJ2 mutations: characteristic T-U-wave patterns predict the KCNJ2 genotype","volume":"111","author":"Zhang","year":"2005","unstructured":"Zhang\n              L\n            , BensonDW, Tristani-FirouziM,\n          . Electrocardiographic features in Andersen-Tawil syndrome patients with KCNJ2 mutations: characteristic T-U-wave patterns predict the KCNJ2 genotype. Circulation. 2005;111(21):2720\u20132726."},{"issue":"(3)","key":"i2331-348X-28-7-662-b3","series-title":"Curr Cardiol Rev","first-page":"222","article-title":"Electrocardiogram in Andersen-Tawil syndrome: new electrocardiographic criteria for diagnosis of type-1 Andersen-Tawil syndrome","volume":"10","author":"Kukla","year":"2014","unstructured":"Kukla\n              P\n            , BiernackaEK, BaranchukA,\n          . Electrocardiogram in Andersen-Tawil syndrome: new electrocardiographic criteria for diagnosis of type-1 Andersen-Tawil syndrome. Curr Cardiol Rev. 2014;10(3):222\u2013228."},{"issue":"(3)","key":"i2331-348X-28-7-662-b4","series-title":"Heart Rhythm","first-page":"596","article-title":"Efficacy and safety of flecainide for ventricular arrhythmias in patients with Andersen-Tawil syndrome with KCNJ2 mutations","volume":"12","author":"Miyamoto","year":"2015","unstructured":"Miyamoto\n              K\n            , AibaT, KimuraH,\n          . Efficacy and safety of flecainide for ventricular arrhythmias in patients with Andersen-Tawil syndrome with KCNJ2 mutations. Heart Rhythm. 2015;12(3):596\u2013603."},{"issue":"(4)","key":"i2331-348X-28-7-662-b5","series-title":"Heart Rhythm","first-page":"508","article-title":"Flecainide for recurrent malignant ventricular arrhythmias in two siblings with Andersen-Tawil syndrome","volume":"4","author":"B\u00f6kenkamp","year":"2007","unstructured":"B\u00f6kenkamp\n              R\n            , WildeAA, SchalijMJ, BlomNA. Flecainide for recurrent malignant ventricular arrhythmias in two siblings with Andersen-Tawil syndrome. Heart Rhythm. 2007;4(4):508\u2013511."},{"issue":"(1)","key":"i2331-348X-28-7-662-b6","series-title":"J Cardiovasc Electrophysiol","first-page":"95","article-title":"Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome","volume":"19","author":"Pellizz\u00f3n","year":"2008","unstructured":"Pellizz\u00f3n\n              OA\n            , KalaizichL, Pt\u00e1cekLJ,\n          . Flecainide suppresses bidirectional ventricular tachycardia and reverses tachycardia-induced cardiomyopathy in Andersen-Tawil syndrome. J Cardiovasc Electrophysiol. 2008;19(1):95\u201397."},{"issue":"(15)","key":"i2331-348X-28-7-662-b7","series-title":"J Am Coll Cardiol","first-page":"1772","article-title":"Natural history and risk stratification in Andersen-Tawil syndrome type 1","volume":"75","author":"Mazzanti","year":"2020","unstructured":"Mazzanti\n              A\n            , GuzD, TrancuccioA,\n          . Natural history and risk stratification in Andersen-Tawil syndrome type 1. J Am Coll Cardiol. 2020;75(15):1772\u20131784."},{"key":"i2331-348X-28-7-662-b8","series-title":"Int J Cardiol","first-page":"473","article-title":"Rarity and phenotypic heterogeneity provide challenges in the diagnosis of Andersen-Tawil syndrome: two cases presenting with ECGs mimicking catecholaminergic polymorphic ventricular tachycardia (CPVT)","volume":"201","author":"Tully","year":"2015","unstructured":"Tully\n              I\n            , AthertonJ, HuntL,\n          . Rarity and phenotypic heterogeneity provide challenges in the diagnosis of Andersen-Tawil syndrome: two cases presenting with ECGs mimicking catecholaminergic polymorphic ventricular tachycardia (CPVT). Int J Cardiol. 2015;201:473\u2013475."},{"issue":"(15)","key":"i2331-348X-28-7-662-b9","series-title":"J Am Coll Cardiol","first-page":"1785","article-title":"Time to Redefine the natural history and clinical management of type 1 Andersen-Tawil syndrome?","volume":"75","author":"Ackerman","year":"2020","unstructured":"Ackerman\n              MJ\n            , GiudicessiJR. Time to Redefine the natural history and clinical management of type 1 Andersen-Tawil syndrome?J Am Coll Cardiol. 2020;75(15):1785\u20131787."},{"issue":"(2)","key":"i2331-348X-28-7-662-b10","series-title":"Heart Rhythm","first-page":"255","article-title":"Inhibition of Na+ channels ameliorate arrhythmias in a drug-induced model of Andersen-Tawil syndrome","volume":"10","author":"Radwa\u0144ski","year":"2013","unstructured":"Radwa\u0144ski\n              PB\n            , Greer-ShortA, PoelzingS. Inhibition of Na+ channels ameliorate arrhythmias in a drug-induced model of Andersen-Tawil syndrome. Heart Rhythm. 2013;10(2):255\u2013263."},{"issue":"(2)","key":"i2331-348X-28-7-662-b11","series-title":"Heart Rhythm","first-page":"318","article-title":"Combined inhibition of Na+ and Ca2+ channels: a novel paradigm for the treatment of incessant ventricular arrhythmias in Andersen-Tawil syndrome","volume":"11","author":"Janson","year":"2014","unstructured":"Janson\n              CM\n            , PoelzingS, ShahMJ. Combined inhibition of Na+ and Ca2+ channels: a novel paradigm for the treatment of incessant ventricular arrhythmias in Andersen-Tawil syndrome. Heart Rhythm. 2014;11(2):318\u2013320."},{"issue":"(3)","key":"i2331-348X-28-7-662-b12","series-title":"Europace","first-page":"162","article-title":"Sudden cardiac death in Andersen-Tawil syndrome","volume":"9","author":"Peters","year":"2007","unstructured":"Peters\n              S\n            , Schulze-BahrE, EtheridgeSP,\n          . Sudden cardiac death in Andersen-Tawil syndrome. Europace. 2007;9(3):162\u2013166."},{"issue":"(4)","key":"i2331-348X-28-7-662-b13","series-title":"Pacing Clin Electrophysiol","first-page":"429","article-title":"Response to flecainide test in Andersen-Tawil syndrome with incessant ventricular tachycardia","volume":"41","author":"Fern\u00e1ndez","year":"2018","unstructured":"Fern\u00e1ndez\n              M\n            , Mar\u00ednMDR, Fern\u00e1ndez-ArmentaJ,\n          . Response to flecainide test in Andersen-Tawil syndrome with incessant ventricular tachycardia. Pacing Clin Electrophysiol. 2018;41(4):429\u2013432."},{"issue":"(2)","key":"i2331-348X-28-7-662-b14","series-title":"Heart Rhythm Case Rep","first-page":"151","article-title":"Flecainide treats a novel KCNJ2 mutation associated with Andersen-Tawil syndrome","volume":"3","author":"Van Ert","year":"2016","unstructured":"Van Ert\n              HA\n            , McCuneEC, OrlandKM,\n          . Flecainide treats a novel KCNJ2 mutation associated with Andersen-Tawil syndrome. Heart Rhythm Case Rep. 2016;3(2):151\u2013154."},{"key":"i2331-348X-28-7-662-b15","series-title":"J Electrocardiol","first-page":"37","article-title":"Therapeutic management of ventricular arrhythmias in Andersen-Tawil syndrome","volume":"58","author":"Maff\u00e8","year":"2020","unstructured":"Maff\u00e8\n              S\n            , PaffoniP, BergamascoL,\n          . Therapeutic management of ventricular arrhythmias in Andersen-Tawil syndrome. J Electrocardiol. 2020;58:37\u201342."}],"container-title":["The Journal of Pediatric Pharmacology and Therapeutics"],"original-title":[],"link":[{"URL":"https:\/\/jppt.kglmeridian.com\/view\/journals\/jppt\/28\/7\/article-p662.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/meridian.allenpress.com\/jppt\/article-pdf\/28\/7\/662\/3292976\/i2331-348x-28-7-662.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/jppt.kglmeridian.com\/downloadpdf\/journals\/jppt\/28\/7\/article-p662.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/jppt.kglmeridian.com\/downloadpdf\/journals\/jppt\/28\/7\/article-p662.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,16]],"date-time":"2025-05-16T00:49:00Z","timestamp":1747356540000},"score":1,"resource":{"primary":{"URL":"https:\/\/jppt.kglmeridian.com\/view\/journals\/jppt\/28\/7\/article-p662.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023]]},"references-count":15,"journal-issue":{"issue":"7"},"URL":"https:\/\/doi.org\/10.5863\/1551-6776-28.7.662","relation":{},"ISSN":["2331-348X","1551-6776"],"issn-type":[{"type":"electronic","value":"2331-348X"},{"type":"print","value":"1551-6776"}],"subject":[],"published":{"date-parts":[[2023]]}}}