Search Results - "PRIORI, S. G"

CaMKII inhibition rectifies arrhythmic phenotype in a patient-specific model of catecholaminergic polymorphic ventricular tachycardia by Di Pasquale, E, Lodola, F, Miragoli, M, Denegri, M, Avelino-Cruz, J E, Buonocore, M, Nakahama, H, Portararo, P, Bloise, R, Napolitano, C, Condorelli, G, Priori, S G

“…Induced pluripotent stem cells (iPSC) offer a unique opportunity for developmental studies, disease modeling and regenerative medicine approaches in humans…”
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Low penetrance in the long-QT Syndrome : Clinical impact by PRIORI, S. G, NAPOLITANO, C, SCHWARTZ, P. J

“…It is still currently held that most patients affected by the long-QT syndrome (LQTS) show QT interval prolongation or clinical symptoms. This is reflected by…”
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Effectiveness and limitations of β-blocker therapy in congenital long-QT syndrome by MOSS, A. J, ZAREBA, W, MEDINA, A, LI ZHANG, ROBINSON, J. L, TIMOTHY, K, TOWBIN, J. A, ANDREWS, M. L, HALL, W. J, SCHWARTZ, P. J, CRAMPTON, R. S, BENHORIN, J, VINCENT, G. M, LOCATI, E. H, PRIORI, S. G, NAPOLITANO, C

“…beta-blockers are routinely prescribed in congenital long-QT syndrome (LQTS), but the effectiveness and limitations of beta-blockers in this disorder have not…”
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Gene-specific response of dynamic ventricular repolarization to sympathetic stimulation in LQT1, LQT2 and LQT3 forms of congenital long QT syndrome by Noda, T., Takaki, H., Kurita, T., Suyama, K., Nagaya, N., Taguchi, A., Aihara, N., Kamakura, S., Sunagawa, K., Nakamura, K., Ohe, T., Horie, M., Napolitano, C., Towbin, J.A., Priori, S.G., Shimizu, W.

“…Aims Differences in the sensitivity of the genotype of the congenital long QT syndrome to sympathetic stimulation have been suggested. This study compared the…”
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Brugada syndrome and sudden cardiac death in children by Priori, Silvia G, Napolitano, Carlo, Giordano, Umberto, Collisani, Giuseppe, Memmi, Mirella

“…In five children from the same family who died after unexplained cardiac arrest, Brugada syndrome syndrome was suspected based on the transient manifestation…”
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Proposed Diagnostic Criteria for the Brugada Syndrome by Wilde, A.A.M., Antzelevitch, C., Borggrefe, M., Brugada, J., Brugada, R., Brugada, P., Corrado, D., Hauer, R.N.W., Kass, R.S., Nademanee, K., Priori, S.G., Towbin, J.A.

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A Molecular Link between the Sudden Infant Death Syndrome and the Long-QT Syndrome by Schwartz, Peter J, Priori, Silvia G, Dumaine, Robert, Napolitano, Carlo, Antzelevitch, Charles, Stramba-Badiale, Marco, Richard, Todd A, Berti, Maria Rosaria, Bloise, Raffaella

“…The sudden infant death syndrome (SIDS) remains the leading cause of death in the first year of life and has a devastating impact on the affected families. 1 –…”
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The elusive link between LQT3 and Brugada syndrome : The role of flecainide challenge by PRIORI, S. G, NAPOLITANO, C, SCHWARTZ, P. J, BLOISE, R, CROTTI, L, RONCHETTI, E

“…Defects of the SCN5A gene encoding the cardiac sodium channel are associated with both the LQT3 subtype of long-QT syndrome and Brugada syndrome (BS). The…”
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Spectrum of ST-T-wave patterns and repolarization parameters in congenital long-QT syndrome : ECG findings identify genotypes by LI ZHANG, TIMOTHY, Katherine W, YANOWITZ, Frank, BENHORIN, Jesaia, MOSS, Arthur J, SCHWARTZ, Peter J, ROBINSON, Jennifer L, QING WANG, ZAREBA, Wojciech, KEATING, Mark T, TOWBIN, Jeffrey A, NAPOLITANO, Carlo, VINCENT, G. Michael, MEDINA, Aharon, LEHMANN, Michael H, FOX, Jolene, GIULI, Lisa C, JIAXIANG SHEN, SPLAWSKI, Igor, PRIORI, Silvia G, COMPTON, Steven J

“…Congenital long-QT syndrome (LQTS) is caused by mutations of genes encoding the slow component of the delayed rectifier current (LQT1, LQT5), the rapid…”
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Influence of the Genotype on the Clinical Course of the Long-QT Syndrome by Zareba, Wojciech, Moss, Arthur J, Schwartz, Peter J, Vincent, G. Michael, Robinson, Jennifer L, Priori, Silvia G, Benhorin, Jesaia, Locati, Emanuela H, Towbin, Jeffrey A, Keating, Mark T, Lehmann, Michael H, Hall, W. Jackson, Andrews, Mark L, Napolitano, Carlo, Timothy, Katherine, Zhang, Li, Medina, Aharon, MacCluer, Jean W

“…The hereditary long-QT syndrome is a familial disorder characterized by prolonged ventricular repolarization on the electrocardiogram and a propensity for…”
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Genetics of sudden death: focus on inherited channelopathies by CERRONE, Marina, PRIORI, Silvia G

“…Since the discovery of the genetic bases of the long QT syndrome, several new genetically mediated arrhythmias have been described, defining a new group of…”
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Dispersion of the QT interval : a marker of therapeutic efficacy in the idiopathic long QT syndrome by PRIORI, S. G, NAPOLITANO, C, DIEHL, L, SCHWARTZ, P. J

“…QT interval dispersion, measured as interlead variability of QT, is a marker of dispersion of ventricular repolarization and, hence, of cardiac electrical…”
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Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome : Findings from the international LQTS registry by LOCATI, E. H, ZAREBA, W, ANDREWS, M, TIMOTHY, K, HALL, W. J, MOSS, A. J, SCHWARTZ, P. J, VINCENT, G. M, LEHMANN, M. H, TOWBIN, J. A, PRIORI, S. G, NAPOLITANO, C, ROBINSON, J. L

“…Unexplained female predominance is observed in long-QT syndrome (LQTS), a congenital autosomal disorder with prolonged repolarization and syncope or sudden…”
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Inherited Arrhythmogenic Diseases: The Complexity Beyond Monogenic Disorders by Priori, Silvia G

“…ABSTRACT—Twelve years after the identification of the molecular bases of the long-QT syndrome, it is now possible to express some considerations on the impact…”
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Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate : implications for gene-specific therapy by SCHWARTZ, P. J, PRIORI, S. G, COLATSKY, T. J, LOCATI, E. H, NAPOLITANO, C, CANTU, F, TOWBIN, J. A, KEATING, M. T, HICHAM HAMMOUDE, BROWN, A. M, CHEN, L.-S. K

“…The genes for the long QT syndrome (LQTS) linked to chromosomes 3 (LQT3) and 7 (LQT2) were identified as SCN5A, the cardiac Na+ channel gene, and as HERG, a K+…”
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Genetic and molecular basis of cardiac arrhythmias : Impact on clinical management parts I and II by PRIORI, S. G, BARHANIN, J, SCHWARTZ, P. J, TOWBIN, J. A, WILDE, A. M, HAUER, R. N. W, HAVERKAMP, W, JONGSMA, H. J, KLEBER, A. G, MCKENNA, W. J, RODEN, D. M, RUDY, Y, SCHWARTZ, K

“…Genetic approaches have succeeded in defining the molecular basis of an increasing array of heart diseases, such as hypertrophic cardiomyopathy and the long-QT…”
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Mechanisms underlying early and delayed afterdepolarizations induced by catecholamines by Priori, S G, Corr, P B

“…The relative influence of alpha- and beta-adrenergic receptor activation in eliciting early (EADs) and delayed (DADs) after depolarizations was assessed using…”
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Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome by Moss, Arthur J, Robinson, Jennifer L, Gessman, Laura, Gillespie, Robert, Zareba, Wojciech, Schwartz, Peter J, Vincent, G.Michael, Benhorin, Jesaia, Heilbron, Emanuela Locati, Towbin, Jeffrey A, Priori, Silvia G, Napolitano, Carlo, Zhang, Li, Medina, Aharon, Andrews, Mark L, Timothy, Katherine

“…Acute auditory stimuli and swimming activities are frequently associated with syncope, aborted cardiac arrest, and death in the long QT syndrome (LQTS). We…”
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Task Force on Sudden Cardiac Death of the European Society of Cardiology by Priori, S.G., Aliot, E., Blomstrom-Lundqvist, C., Bossaert, L., Breithardt, G., Brugada, P., Camm, A.J., Cappato, R., Cobbe, S.M., Di Mario, C., Maron, B.J., McKenna, W.J., Pedersen, A.K., Ravens, U., Schwartz, P.J., Trusz-Gluza, M., Vardas, P., Wellens, H.J.J., Zipes, D.P.

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Novel Arrhythmogenic Mechanism Revealed by a Long-QT Syndrome Mutation in the Cardiac Na+ Channel by Abriel, Hugues, Cabo, Candido, Wehrens, Xander H.T, Rivolta, Ilaria, Motoike, Howard K, Memmi, Mirella, Napolitano, Carlo, Priori, Silvia G, Kass, Robert S

“…Variant 3 of the congenital long-QT syndrome (LQTS-3) is caused by mutations in the gene encoding the α subunit of the cardiac Na channel. In the present…”
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