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Page 1
Late sodium current associated cardiac electrophysiological and mechanical dysfunction.
Yu S, Li G, Huang CL, Lei M, Wu L. Yu S, et al. Pflugers Arch. 2018 Mar;470(3):461-469. doi: 10.1007/s00424-017-2079-7. Epub 2017 Nov 10. Pflugers Arch. 2018. PMID: 29127493 Review.
Late sodium current (I(NaL)) is a small sustained inward current observed during the cardiac action potential plateau phase following decay of the early peak I(Na). The endogenous I(NaL) is relatively small in normal hearts but exerts functionally significant effects on ca …
Late sodium current (I(NaL)) is a small sustained inward current observed during the cardiac action potential plateau phase following …
Genetic testing in Polynesian long QT syndrome probands reveals a lower diagnostic yield and an increased prevalence of rare variants.
Winbo A, Earle N, Marcondes L, Crawford J, Prosser DO, Love DR, Merriman TR, Cadzow M, Stiles R, Donoghue T, Stiles MK, Hayes I, Skinner JR. Winbo A, et al. Heart Rhythm. 2020 Aug;17(8):1304-1311. doi: 10.1016/j.hrthm.2020.03.015. Epub 2020 Mar 27. Heart Rhythm. 2020. PMID: 32229296
METHODS: A 15-year retrospective review of clinical CIDRNZ LQTS probands with a Schwartz score of 2 who had undergone genetic testing was performed. RESULTS: Of the 264 included LQTS probands, 160 (61%) reported as European, 79 (30%) NZ Maori and Pacific peoples (Polynesia …
METHODS: A 15-year retrospective review of clinical CIDRNZ LQTS probands with a Schwartz score of 2 who had undergone genetic testing …
Systematic ajmaline challenge in patients with long QT 3 syndrome caused by the most common mutation: a multicentre study.
Hohmann S, Rudic B, Konrad T, Duncker D, König T, Tülümen E, Rostock T, Borggrefe M, Veltmann C. Hohmann S, et al. Europace. 2017 Oct 1;19(10):1723-1729. doi: 10.1093/europace/euw214. Europace. 2017. PMID: 27915266
Following ajmaline, a significant shortening of the rate-corrected JT (JTc) interval was observed in mutation carriers. The baseline JTc interval was significantly longer in mutation carriers with a positive ajmaline challenge compared with those with a negative one. ...
Following ajmaline, a significant shortening of the rate-corrected JT (JTc) interval was observed in mutation carriers. The baseline …
Gene-Specific Therapy With Mexiletine Reduces Arrhythmic Events in Patients With Long QT Syndrome Type 3.
Mazzanti A, Maragna R, Faragli A, Monteforte N, Bloise R, Memmi M, Novelli V, Baiardi P, Bagnardi V, Etheridge SP, Napolitano C, Priori SG. Mazzanti A, et al. J Am Coll Cardiol. 2016 Mar 8;67(9):1053-1058. doi: 10.1016/j.jacc.2015.12.033. J Am Coll Cardiol. 2016. PMID: 26940925 Free PMC article.
METHODS: The endpoint of this retrospective cohort study, which studied consecutive LQT3 patients who were referred to our center and treated with mexiletine, was to evaluate the antiarrhythmic efficacy of mexiletine by comparing the number of arrhythmic events per patient and th …
METHODS: The endpoint of this retrospective cohort study, which studied consecutive LQT3 patients who were referred to our center and treate …
Cardiomyocytes obtained from induced pluripotent stem cells with long-QT syndrome 3 recapitulate typical disease-specific features in vitro.
Malan D, Friedrichs S, Fleischmann BK, Sasse P. Malan D, et al. Circ Res. 2011 Sep 30;109(8):841-7. doi: 10.1161/CIRCRESAHA.111.243139. Epub 2011 Jul 28. Circ Res. 2011. PMID: 21799153
Patch-clamp measurements of LQTS 3-specific cardiomyocytes showed the biophysical effects of the mutation on the Na(+) current, with faster recovery from inactivation and larger late currents than observed in controls. Moreover, LQTS 3-specific cardiomyocytes had prolonged …
Patch-clamp measurements of LQTS 3-specific cardiomyocytes showed the biophysical effects of the mutation on the Na(+) current, with faster …
Delayed sodium channel inactivation mimics long QT syndrome 3.
Kühlkamp V, Mewis C, Bosch R, Seipel L. Kühlkamp V, et al. J Cardiovasc Pharmacol. 2003 Jul;42(1):113-7. doi: 10.1097/00005344-200307000-00017. J Cardiovasc Pharmacol. 2003. PMID: 12827035 Clinical Trial.
Acute administration of DPI 201-106 prolonged the QT interval, provoked spontaneous torsades de pointes in one patient, and facilitated stimulation-induced polymorphic ventricular tachyarrhythmias in two patients. Similar to the observations in animal studies, delaying sod …
Acute administration of DPI 201-106 prolonged the QT interval, provoked spontaneous torsades de pointes in one patient, and facilitated stim …
Pronounced Shortening of QT Interval With Mexiletine Infusion Test in Patients With Type 3 Congenital Long QT Syndrome.
Funasako M, Aiba T, Ishibashi K, Nakajima I, Miyamoto K, Inoue Y, Okamura H, Noda T, Kamakura S, Anzai T, Noguchi T, Yasuda S, Miyamoto Y, Fukushima Kusano K, Ogawa H, Shimizu W. Funasako M, et al. Circ J. 2016;80(2):340-5. doi: 10.1253/circj.CJ-15-0984. Epub 2015 Dec 3. Circ J. 2016. PMID: 26632536 Free article.
While QTc interval was shortened with mexiletine in both groups (P<0.0001 vs. baseline), degree of QTc shortening (QTc) was significantly larger in LQT3 than in LQT1/LQT2 patients (99 39 vs. 48 32 ms; P=0.0004). The sensitivity, specificity and predictive accuracy of me …
While QTc interval was shortened with mexiletine in both groups (P<0.0001 vs. baseline), degree of QTc shortening (QTc) was significantly …
Further Insights in the Most Common SCN5A Mutation Causing Overlapping Phenotype of Long QT Syndrome, Brugada Syndrome, and Conduction Defect.
Veltmann C, Barajas-Martinez H, Wolpert C, Borggrefe M, Schimpf R, Pfeiffer R, Cáceres G, Burashnikov E, Antzelevitch C, Hu D. Veltmann C, et al. J Am Heart Assoc. 2016 Jul 5;5(7):e003379. doi: 10.1161/JAHA.116.003379. J Am Heart Assoc. 2016. PMID: 27381756 Free PMC article.
BACKGROUND: Phenotypic overlap of type 3 long QT syndrome (LQT3), Brugada syndrome (BrS), cardiac conduction disease (CCD), and sinus node dysfunction (SND) is observed with SCN5A mutations. SCN5A-E1784K is the most common mutation associated with BrS and LQTS3. ...Co-expr …
BACKGROUND: Phenotypic overlap of type 3 long QT syndrome (LQT3), Brugada syndrome (BrS), cardiac conduction disease (CCD), and sinus node d …
Cardiac evaluation of pediatric relatives in sudden arrhythmic death syndrome: a 2-center experience.
Wong LC, Roses-Noguer F, Till JA, Behr ER. Wong LC, et al. Circ Arrhythm Electrophysiol. 2014 Oct;7(5):800-6. doi: 10.1161/CIRCEP.114.001818. Epub 2014 Sep 6. Circ Arrhythm Electrophysiol. 2014. PMID: 25194972
A probable diagnosis was made in 18 (29.5%) families: Brugada syndrome, 13/18 (72%); long QT syndrome, 3/18 (17%); and catecholaminergic polymorphic ventricular tachycardia, 2/18 (11%). ...
A probable diagnosis was made in 18 (29.5%) families: Brugada syndrome, 13/18 (72%); long QT syndrome, 3/18 (17% …
15 results