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dc.contributor.authorMazzanti, Andrea
dc.contributor.authorMaragna, Riccardo
dc.contributor.authorVacanti, Gaetano
dc.contributor.authorKostopoulou, Anna
dc.contributor.authorMarino, Maira
dc.contributor.authorMonteforte, Nicola
dc.contributor.authorBloise, Raffaella
dc.contributor.authorUnderwood, Katherine
dc.contributor.authorTibollo, Valentina
dc.contributor.authorPagan, Eleonora
dc.contributor.authorNapolitano, Carlo
dc.contributor.authorBellazzi, Riccardo
dc.contributor.authorBagnardi, Vincenzo
dc.contributor.authorPriori, Silvia G. 
dc.date.accessioned2020-06-19T08:59:31Z
dc.date.available2020-06-19T08:59:31Z
dc.date.issued2017-12-19
dc.identifier.citationJ Am Coll Cardiol. 2017; 70(24):3010-3015es_ES
dc.identifier.urihttp://hdl.handle.net/20.500.12105/10497
dc.description.abstractShort QT syndrome (SQTS) is a rare and life-threatening arrhythmogenic syndrome characterized by abbreviated repolarization. Hydroquinidine (HQ) prolongs the QT interval in SQTS patients, although whether it reduces cardiac events is currently unknown. This study investigated whether long-term treatment with HQ reduces the occurrence of life-threatening arrhythmic events (LAE) (cardiac arrest or sudden cardiac death) in SQTS patients. In this cohort study on consecutive SQTS patients, 2 analyses were performed: 1) a matched-period analysis for the occurrence of LAE in 17 SQTS patients who received long-term HQ; and 2) a comparison of the annual incidence of LAE off- and on-HQ in 16 SQTS patients who survived a cardiac arrest. A total of 17 patients (82% male, age 29 ± 3 years, QTc before treatment 331 ± 3 ms) received HQ therapy (584 ± 53 mg/day). Therapy was stopped in 2 cases (12%) due to gastrointestinal intolerance, and 15 patients continued treatment for 6 ± 1 year. QTc prolongation was observed in all patients (by 60 ± 6 ms; p < 0.001). We compared the occurrence of LAE during 6 ± 1 years before and after HQ, observing that patients on HQ experienced a reduction in both the rate of LAE from 40% to 0% (p = 0.03) and the number of LAE per patient from 0.73 ± 0.3 to 0 (p = 0.026). Furthermore, the annual rate of LAE in the 16 patients with a previous cardiac arrest dropped from 12% before HQ to 0 on therapy (p = 0.028). We demonstrated for the first time that treatment with HQ was associated with a lower incidence of LAE in SQTS patients. These data point to the importance that quinidine, that in several countries has been removed from the market, remains available worldwide for patients with SQTS. In the present study, therapy with HQ has been proven to be safe, with a relatively low rate of side effects.es_ES
dc.description.sponsorshipThis work was supported by the Ricerca Corrente Funding scheme of the Italian Ministry of Health. Dr. Bellazzi is a shareholder of Biomeris; and is a stakeholder of Engenome. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Pedro Brugada, MD, served as Guest Editor for this paper.es_ES
dc.language.isoenges_ES
dc.publisherElsevier es_ES
dc.type.hasVersionAMes_ES
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subject.meshAdolescent es_ES
dc.subject.meshAdult es_ES
dc.subject.meshAnti-Arrhythmia Agents es_ES
dc.subject.meshArrhythmias, Cardiaces_ES
dc.subject.meshDeath, Sudden, Cardiac es_ES
dc.subject.meshElectrocardiography es_ES
dc.subject.meshFemale es_ES
dc.subject.meshFollow-Up Studies es_ES
dc.subject.meshHeart Conduction System es_ES
dc.subject.meshHeart Rate es_ES
dc.subject.meshHumans es_ES
dc.subject.meshIncidence es_ES
dc.subject.meshItaly es_ES
dc.subject.meshMale es_ES
dc.subject.meshQuinidine es_ES
dc.subject.meshSurvival Rate es_ES
dc.subject.meshVentricular Fibrillation es_ES
dc.subject.meshYoung Adult es_ES
dc.titleHydroquinidine Prevents Life-Threatening Arrhythmic Events in Patients With Short QT Syndrome.es_ES
dc.typejournal articlees_ES
dc.rights.licenseAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.identifier.pubmedID29241489es_ES
dc.format.volume70es_ES
dc.format.number24es_ES
dc.format.page3010-3015es_ES
dc.identifier.doi10.1016/j.jacc.2017.10.025es_ES
dc.contributor.funderMinistero della Salute (Italia) 
dc.description.peerreviewedes_ES
dc.identifier.e-issn1558-3597es_ES
dc.relation.publisherversionhttps://doi.org/10.1016/j.jacc.2017.10.025es_ES
dc.identifier.journalJournal of the American College of Cardiologyes_ES
dc.repisalud.orgCNICCNIC::Grupos de investigación::Cardiología Moleculares_ES
dc.repisalud.institucionCNICes_ES
dc.rights.accessRightsopen accesses_ES


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Attribution-NonCommercial-NoDerivatives 4.0 Internacional
Este Item está sujeto a una licencia Creative Commons: Attribution-NonCommercial-NoDerivatives 4.0 Internacional