Search

Your search keyword '"Benhorin J."' showing total 161 results
Start Over Author "Benhorin J."
161 results on '"Benhorin J."'

3. Interventional treatment in diabetics in the era of drug-eluting stents and compliance to the ESC guidelines: lessons learned from the Euro Heart Survey Programme

Author

Onuma Y., Kukreja N., Ramcharitar S., Hochadel M., Gitt A., Serruys P., Marco J., Vahanian A., Weidinger F., Wijns W., Zeymer U., Silber S., Seabra-Gomez R., Eberli F., Manini M., Bramley C., Laforest V., Taylor C., Huber K., Backer G. D., Sirakova V., Cerbak R., Thayssen P., Aziz O. A., Tammam K., Lehto S., Delahaye F., Kobulia B., Cokkinos D., Kremastinos D., Karlocai K., Shelley E., Behar S., Maggioni A., Grabauskiene V., Deckers J., Asmussen I., Stepinska J., Goncalves L., Fonseca C., Mareev V., Vasilijevic Z., Riecansky M. I., Kenda M. F., Lopez-Sendon J. L., Rosengren A., Buser P., Okay T., Sychov O., Schofield P., Gitt A. K., Tavazzi L., Gomes R. S., de la Iglesia J. M., Wallentin L., Kearney P., McGregor K., Simoons M. L., Squibb B. -M., Lilly E., Margaryan K., Khachatryan S., Doerler J., Stocker E. -M., Altenberger I. J., Heigert M., Pichler M., Christ S. G., Glogar H., Lang I., Ingerle S., De Wilde P., de Marneffe M., Vrolix B. M., Dens J., Lierde J. V., De Wagter G. X., Carlier G. M., Weyne G. A., Legrand K. V., Doneux P., Gach O., Davin L., Mievis L. E., Massart P. -E., Holvoet N. G., Giunio L., Glavas D., Vukovic I., Markovic B., Duplancic D., Runjic F., Galic S. E., Mirat J., Kala P., Semenka J., Hlinomaz O., Petrikovits E., Widimsky B. P., Tousek P., Varvarovsky P. I., Cappelen H., Helqvist O. S., Kelbaek H., Jorgensen E., Engstrom T., Saunamaki K., Kastrup J., Clemmensen P., Hansen H., Al Abbadi M., Razek H. A., Aboul el Nasr G., Ragi H., Ibrihim B., Zarif B., el Banhawy N., Sorour K., Meguid M. A., Mahrous A., Al Khashab K. A., Ahmed Abd Elmoniem F., El Emry M., El Naggar A., Saad B. A., Laanmets P., Voitk J., Lutter P., Jarvekulg S., Jalakas M., Reinmets J., Marandi T., Peeba M., Serka T., Syvannne M., Kaihovirta E., Korpilahti H. K., Vaittinen M. -A., Bassand J. -P., Espinosa D. P., Cottin B. Y., Lhuillier I., Buffet P., Lorgis L., Machecourt D. J., Bertrand B., Serrano D., Bonnet G. J. -L., Steg M. P. G., Juliard J. -M., Farnoud R., Delarche P. N., Marco P. J., Petit F., Farah B., Carrie D., Galinier M., Puel J., Cahuzac J., Roncalli J., Tauzin S., Elbaz M., Schachinger V., Gitt F. A., am Rhein Ralf Zahn L., Fraiture B., Haetinger S., Klepzig N. H., Girth E., Hauber A., Firschke O. C., Widmaier J., Hofbauer F., Huttl S., Sechtem P. U., Parade U., Linnartz S. G., Andrianidis S., Tsiavou N., Papaioannou G., Deliargyris E., Attikis M., Alexopoulos D., Davlouros P., Tsikaderis D., Dardas P., Mezilis N., Istvan E., Zoltan B., Turgeman Y., Khaled S., Feldman A., Jafari J., Manevich I., Cafri C., Ilia R., Abu-Ful A., Yaroslavslev S., Wainstain J. M., Rosenchtein G., Sheva B., Krakover R., Yakov B., Halon D., Gruberg L., Markiewicz W., Grenadier E., Boulos M., Roguin A., Kerner A., Amikam S., Ben-Tzvi M., Rezmovitz J., Mosseri H. M., Lotan H., Varshizky B., Nassar H., Daninberg H., Rot D., Vais T., Benhorin J., Keren A., Medina A., Huri Z., Brandis J. S., Schoenmann G., Kornowski N. R., Assali A., Fuch S., Hasdai D., Brosh D., Sela O., Teplitski I., Tikva P., Eisenberg O., Banai S., Finkelstein A., Hasin Y., Aboud M., Nahir M., Qarwani D., Diab G., Meloni L., Lai G., Cadeddu M., Pirisi R., Bonechi F., Nassi F., Nieri M., Taiti A., Naldoni A., Calabro F., Achilli F., Maggiolini S., Piatti L., Tiberti G., Addamiano P., Berti S., Ravani M., Palmieri C., Trianni G., Cardullo S., Cioppa A., Rubino P., Ambrosini V., Salemme L., Sorropago G., Tesorio T., Geraci G., Scalise F., Mazzeti S., Auguadro C., Esposito G., Canali G., Caccia M. E., Ruggieri C., Benedetta B., de Cesare N., De Benedictis M., Coco T., Manzotti S., Fraz O. S., Marraccini P., Danesi A., Ricci R., Ferraironi A., Olivieri E., Chiera A., Garducci S., Grasseli D., McFadden E., Cahill N., Quinn M., Crean P., Caroll E., Foley D., O'Connor S., O'Hanlon R., Lynch B., O'Donnell S., Roy J., O'Brien D., Krastina A., Erglis A., Lawand S., Dorniak W., Klaudel J., Pawlowski K., Trenkner W., Janion M., Sadowski M., Janion-Sadowska A., Skorupa I., Bystryk L., Kern A., Janiak B., Szelemej R., Ruzyllo W., Witkowski A., Deptuch T., Maczynska-Mazuruk R., Budaj A., Cegieska K. L., Opolski G., Wilczyska J., Roik M., Kochman J., Martins D., Goncalves I. M. F. J., Pereira H., Faria H., Calisto J., Matos V., Leitao-Marques A., Costa M., Oliveira H., Mota P., Santos W., Brandao V., Caires F. G., Silva B., Teles F. R. C., Almeida M., Goncalves P., Raposo L., Mourao L., Bernardes L., Pedro P. G., Ferreira R., Conduto R., Quininha J., Patricio L., Cacela D., Goncalves J. M., de Sousa L., Adao M., Carvalho L. H. C., Romeira H., Sousa J. P., Garcia J. M. M., Silva J. C., Magalhaes D., Santos P. R., Mendes S. P. G., Pipa J., Nunes L., Ferreira P., Vinereanu D., Udroiu C., Florescu N., Parvu O., Stoicescu C., Dorobantu M., Balanescu S. M., Niculescu R., Calmac L., Marinescu M., Olinic B. D., Ober M., Homorodean C., Budurea C., Hij A., Anton F., Cluj-Napoca, Ortan F., Suciu C., Ursu M., Baba C., Targu-Mures, Dragulescu S. I., Petrescu L., Slovenski M., Gavrilescu D., Dina C., Mut B., Babic R., Colic M., Topic D., Vilarrasa J. B., Pont M. P., Martorell R. M., Rohlfs I., Moreno R. M., Irurita M., Irurita J., de Gran Canaria L. P., Cervantes C. E., Galvan T., Navarro J., Franco D., Rodriguez I. S., Ramirez V. H., Fernandes-Aviles F., Revilla A., Masson N., Dupertuis V., Kachboura S., Iyisoy A., Erol M. K., Ongen Z., Babalik E., Oskan M., Ozdemir N., Oto A., Aytemir K., Yavuz B., Sahin M., Durna K., Aytekin V., Demiroglu C., Gulbaran M., Aytekin S., Catakoglu A. B., Ozme B., Gemici G., Feray H., Schofield P. M., Kahn S., Clarke S., Millington H., Di Mario C., Dempster D., Henderson R. A., Burton J., Falcon-Lang D., Cardiology, Onuma, Y., Kukreja, N., Ramcharitar, S., Hochadel, M., Gitt, A., Serruys, P., Marco, J., Vahanian, A., Weidinger, F., Wijns, W., Zeymer, U., Silber, S., Seabra-Gomez, R., Eberli, F., Manini, M., Bramley, C., Laforest, V., Taylor, C., Huber, K., Backer, G. D., Sirakova, V., Cerbak, R., Thayssen, P., Aziz, O. A., Tammam, K., Lehto, S., Delahaye, F., Kobulia, B., Cokkinos, D., Kremastinos, D., Karlocai, K., Shelley, E., Behar, S., Maggioni, A., Grabauskiene, V., Deckers, J., Asmussen, I., Stepinska, J., Goncalves, L., Fonseca, C., Mareev, V., Vasilijevic, Z., Riecansky, M. I., Kenda, M. F., Lopez-Sendon, J. L., Rosengren, A., Buser, P., Okay, T., Sychov, O., Schofield, P., Gitt, A. K., Tavazzi, L., Gomes, R. S., de la Iglesia, J. M., Wallentin, L., Kearney, P., Mcgregor, K., Simoons, M. L., Squibb, B. -M., Lilly, E., Margaryan, K., Khachatryan, S., Doerler, J., Stocker, E. -M., Altenberger, I. J., Heigert, M., Pichler, M., Christ, S. G., Glogar, H., Lang, I., Ingerle, S., De Wilde, P., de Marneffe, M., Vrolix, B. M., Dens, J., Lierde, J. V., De Wagter, G. X., Carlier, G. M., Weyne, G. A., Legrand, K. V., Doneux, P., Gach, O., Davin, L., Mievis, L. E., Massart, P. -E., Holvoet, N. G., Giunio, L., Glavas, D., Vukovic, I., Markovic, B., Duplancic, D., Runjic, F., Galic, S. E., Mirat, J., Kala, P., Semenka, J., Hlinomaz, O., Petrikovits, E., Widimsky, B. P., Tousek, P., Varvarovsky, P. I., Cappelen, H., Helqvist, O. S., Kelbaek, H., Jorgensen, E., Engstrom, T., Saunamaki, K., Kastrup, J., Clemmensen, P., Hansen, H., Al Abbadi, M., Razek, H. A., Aboul el Nasr, G., Ragi, H., Ibrihim, B., Zarif, B., el Banhawy, N., Sorour, K., Meguid, M. A., Mahrous, A., Al Khashab, K. A., Ahmed Abd Elmoniem, F., El Emry, M., El Naggar, A., Saad, B. A., Laanmets, P., Voitk, J., Lutter, P., Jarvekulg, S., Jalakas, M., Reinmets, J., Marandi, T., Peeba, M., Serka, T., Syvannne, M., Kaihovirta, E., Korpilahti, H. K., Vaittinen, M. -A., Bassand, J. -P., Espinosa, D. P., Cottin, B. Y., Lhuillier, I., Buffet, P., Lorgis, L., Machecourt, D. J., Bertrand, B., Serrano, D., Bonnet, G. J. -L., Steg, M. P. G., Juliard, J. -M., Farnoud, R., Delarche, P. N., Marco, P. J., Petit, F., Farah, B., Carrie, D., Galinier, M., Puel, J., Cahuzac, J., Roncalli, J., Tauzin, S., Elbaz, M., Schachinger, V., Gitt, F. A., am Rhein Ralf Zahn, L., Fraiture, B., Haetinger, S., Klepzig, N. H., Girth, E., Hauber, A., Firschke, O. C., Widmaier, J., Hofbauer, F., Huttl, S., Sechtem, P. U., Parade, U., Linnartz, S. G., Andrianidis, S., Tsiavou, N., Papaioannou, G., Deliargyris, E., Attikis, M., Alexopoulos, D., Davlouros, P., Tsikaderis, D., Dardas, P., Mezilis, N., Istvan, E., Zoltan, B., Turgeman, Y., Khaled, S., Feldman, A., Jafari, J., Manevich, I., Cafri, C., Ilia, R., Abu-Ful, A., Yaroslavslev, S., Wainstain, J. M., Rosenchtein, G., Sheva, B., Krakover, R., Yakov, B., Halon, D., Gruberg, L., Markiewicz, W., Grenadier, E., Boulos, M., Roguin, A., Kerner, A., Amikam, S., Ben-Tzvi, M., Rezmovitz, J., Mosseri, H. M., Lotan, H., Varshizky, B., Nassar, H., Daninberg, H., Rot, D., Vais, T., Benhorin, J., Keren, A., Medina, A., Huri, Z., Brandis, J. S., Schoenmann, G., Kornowski, N. R., Assali, A., Fuch, S., Hasdai, D., Brosh, D., Sela, O., Teplitski, I., Tikva, P., Eisenberg, O., Banai, S., Finkelstein, A., Hasin, Y., Aboud, M., Nahir, M., Qarwani, D., Diab, G., Meloni, L., Lai, G., Cadeddu, M., Pirisi, R., Bonechi, F., Nassi, F., Nieri, M., Taiti, A., Naldoni, A., Calabro, F., Achilli, F., Maggiolini, S., Piatti, L., Tiberti, G., Addamiano, P., Berti, S., Ravani, M., Palmieri, C., Trianni, G., Cardullo, S., Cioppa, A., Rubino, P., Ambrosini, V., Salemme, L., Sorropago, G., Tesorio, T., Geraci, G., Scalise, F., Mazzeti, S., Auguadro, C., Esposito, G., Canali, G., Caccia, M. E., Ruggieri, C., Benedetta, B., de Cesare, N., De Benedictis, M., Coco, T., Manzotti, S., Fraz, O. S., Marraccini, P., Danesi, A., Ricci, R., Ferraironi, A., Olivieri, E., Chiera, A., Garducci, S., Grasseli, D., Mcfadden, E., Cahill, N., Quinn, M., Crean, P., Caroll, E., Foley, D., O'Connor, S., O'Hanlon, R., Lynch, B., O'Donnell, S., Roy, J., O'Brien, D., Krastina, A., Erglis, A., Lawand, S., Dorniak, W., Klaudel, J., Pawlowski, K., Trenkner, W., Janion, M., Sadowski, M., Janion-Sadowska, A., Skorupa, I., Bystryk, L., Kern, A., Janiak, B., Szelemej, R., Ruzyllo, W., Witkowski, A., Deptuch, T., Maczynska-Mazuruk, R., Budaj, A., Cegieska, K. L., Opolski, G., Wilczyska, J., Roik, M., Kochman, J., Martins, D., Goncalves, I. M. F. J., Pereira, H., Faria, H., Calisto, J., Matos, V., Leitao-Marques, A., Costa, M., Oliveira, H., Mota, P., Santos, W., Brandao, V., Caires, F. G., Silva, B., Teles, F. R. C., Almeida, M., Goncalves, P., Raposo, L., Mourao, L., Bernardes, L., Pedro, P. G., Ferreira, R., Conduto, R., Quininha, J., Patricio, L., Cacela, D., Goncalves, J. M., de Sousa, L., Adao, M., Carvalho, L. H. C., Romeira, H., Sousa, J. P., Garcia, J. M. M., Silva, J. C., Magalhaes, D., Santos, P. R., Mendes, S. P. G., Pipa, J., Nunes, L., Ferreira, P., Vinereanu, D., Udroiu, C., Florescu, N., Parvu, O., Stoicescu, C., Dorobantu, M., Balanescu, S. M., Niculescu, R., Calmac, L., Marinescu, M., Olinic, B. D., Ober, M., Homorodean, C., Budurea, C., Hij, A., Anton, F., Cluj-Napoca, Ortan, F., Suciu, C., Ursu, M., Baba, C., Targu-Mures, Dragulescu, S. I., Petrescu, L., Slovenski, M., Gavrilescu, D., Dina, C., Mut, B., Babic, R., Colic, M., Topic, D., Vilarrasa, J. B., Pont, M. P., Martorell, R. M., Rohlfs, I., Moreno, R. M., Irurita, M., Irurita, J., de Gran Canaria, L. P., Cervantes, C. E., Galvan, T., Navarro, J., Franco, D., Rodriguez, I. S., Ramirez, V. H., Fernandes-Aviles, F., Revilla, A., Masson, N., Dupertuis, V., Kachboura, S., Iyisoy, A., Erol, M. K., Ongen, Z., Babalik, E., Oskan, M., Ozdemir, N., Oto, A., Aytemir, K., Yavuz, B., Sahin, M., Durna, K., Aytekin, V., Demiroglu, C., Gulbaran, M., Aytekin, S., Catakoglu, A. B., Ozme, B., Gemici, G., Feray, H., Schofield, P. M., Kahn, S., Clarke, S., Millington, H., Di Mario, C., Dempster, D., Henderson, R. A., Burton, J., and Falcon-Lang, D.

Subjects
Registrie, Male, medicine.medical_treatment, Angiotensin-Converting Enzyme Inhibitors, Comorbidity, Coronary Artery Disease, Severity of Illness Index, Cardiovascular Disease, Hospital Mortality, Registries, Angioplasty, Balloon, Coronary, Drug-Eluting Stents, Middle Aged, Clopidogrel, Europe, Treatment Outcome, Drug-eluting stent, Cardiovascular Diseases, Practice Guidelines as Topic, Female, Guideline Adherence, Inpatient, Cardiology and Cardiovascular Medicine, Human, medicine.drug, medicine.medical_specialty, Diabetic Angiopathie, Adrenergic beta-Antagonists, Diabetic, SDG 3 - Good Health and Well-being, Internal medicine, Diabetes mellitus, Angioplasty, medicine, Humans, Drug eluting stent, cardiovascular diseases, Risk factor, Aged, European Heart Survey, Inpatients, Clinical Audit, business.industry, Platelet Aggregation Inhibitor, Adrenergic beta-Antagonist, Angiotensin-Converting Enzyme Inhibitor, Guideline, medicine.disease, Surgery, Health Care Survey, Health Care Surveys, Conventional PCI, Hydroxymethylglutaryl-CoA Reductase Inhibitor, Hydroxymethylglutaryl-CoA Reductase Inhibitors, business, Diabetic Angiopathies, Platelet Aggregation Inhibitors
Abstract

Aims: The objective of the study is to determine the demographics and the in-hospital outcome of diabetic and non-diabetic patients treated with percutaneous coronary interventions (PCI) in Europe, to report the type of equipment and technology used for PCI procedures in diabetics and to clarify whether the treatment of diabetic patients complies with current European Society of Cardiology (ESC) guidelines. Methods and results: A total of 14,458 patients treated with PCI were enrolled from 29 member countries of the ESC between June 2005 and January 2006. Data were collected on patient characteristics and treatment, using new Cardiology Audit and Registration Data standards. In total, 3,603 patients (24.9%) were diabetic. Diabetics were older, more often female and had a higher body mass index than non-diabetics. Diabetics had higher rates of hypercholesterolaemia and hypertension, while current smokers were more frequent in the non-diabetics. Diabetics also had significantly higher rates of previous cardiovascular events. Clopidogrel was administered only in 48.1% of diabetic patients before PCI, while IIb/IIIa inhibitors were 22.9% during PCI. At discharge, there was a major adjustment of treatment with increases in the use of Beta-blocker (80.4%), angiotensin converting enzyme inhibitor (ACEI, 71.3%) and statins (89.8%) compared with on admission (Beta-blocker 60.9%, ACEI 55.0%, statin 63.1%). Inhospital mortality was higher in diabetics (1.8% vs 1.2%) although the in-hospital MACCE rate was not significantly different (3.6% vs 3.0%, p=0.09). Conclusions: Diabetic patients treated with PCI were older with more comorbidity. According to ESC guideline, the under-usage of clopidogrel, GP IIb/IIIa inhibitors should be improved. PCI is now taken as a good opportunity to adjust the use of appropriate medication. © Europa Edition. All rights reserved.

Published
2009

5. Mutation-Specific Risk in Two Genetic Forms of Type 3 Long QT Syndrome

Author

Liu, J, Moss, A, Jons, C, Benhorin, J, Schwartz, P, Spazzolini, C, Crotti, L, Ackerman, M, Mcnitt, S, Robinson, J, Qi, M, Goldenberg, I, Zareba, W, Liu JF, Moss AJ, Jons C, Benhorin J, Schwartz PJ, Spazzolini C, Crotti L, Ackerman MJ, McNitt S, Robinson JL, Qi M, Goldenberg I, Zareba W, Liu, J, Moss, A, Jons, C, Benhorin, J, Schwartz, P, Spazzolini, C, Crotti, L, Ackerman, M, Mcnitt, S, Robinson, J, Qi, M, Goldenberg, I, Zareba, W, Liu JF, Moss AJ, Jons C, Benhorin J, Schwartz PJ, Spazzolini C, Crotti L, Ackerman MJ, McNitt S, Robinson JL, Qi M, Goldenberg I, and Zareba W

Abstract

The clinical course of patients with 2 relatively common long QT syndrome type 3 mutations has not been well described. In the present study, we investigated the mutational-specific risk in patients with deletional (DeltaKPQ) and missense (D1790G) mutations involving the SCN5A gene. The study population involved 50 patients with the DeltaKPQ mutation and 35 patients with the D1790G mutation. The cumulative probability of a first cardiac event (syncope, aborted cardiac arrest, or long QT syndrome-related sudden death) was evaluated using the Kaplan-Meier method. The Cox proportional hazards survivorship model was used to determine the independent contribution of clinical and genetic factors to the first occurrence of cardiac events from birth through 40 years of age. The Andersen-Gill proportional intensity regression model was used to analyze the factors associated with recurrent syncope. Patients with a DeltaKPQ mutation had a significantly greater probability of a first cardiac event from birth through 40 years of age (34%) than those with the D1790G mutation (20%; p <0.001). Multivariate analysis demonstrated an increased risk of cardiac events among DeltaKPQ carriers compared to D1790G carriers (hazard ratio 2.42, p <0.0001) after adjustment for gender and QTc duration. Patients with DeltaKPQ mutations also had an increased risk of recurrent syncope (hazard ratio 5.20, p <0.001). In conclusion, the clinical course of patients with long QT syndrome type with DeltaKPQ mutations was shown to be more virulent than those with D1790G mutations, and this effect was independent of QTc duration. The findings highlight the importance of knowing the specific mutation in risk stratification of patients with long QT syndrome type 3

Published
2010

6. Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy

Author

Spazzolini, C, Mullally, J, Schwartz, P, Moss, A, Mcnitt, S, Ouellet, G, Fugate, T, Goldenberg, I, Jons, C, Zareba, W, Robinson, J, Ackerman, M, Benhorin, J, Crotti, L, Kaufman, E, Locati, E, Ming, Q, Napolitano, C, Priori, S, Towbin, J, Vincent, G, Spazzolini C, Mullally J, Schwartz PJ, Moss AJ, McNitt S, Ouellet G, Fugate T, Goldenberg I, Jons C, Zareba W, Robinson JL, Ackerman MJ, Benhorin J, Crotti L, Kaufman ES, Locati EH, Ming Q, Napolitano C, Priori SG, Towbin JA, Vincent GM., Spazzolini, C, Mullally, J, Schwartz, P, Moss, A, Mcnitt, S, Ouellet, G, Fugate, T, Goldenberg, I, Jons, C, Zareba, W, Robinson, J, Ackerman, M, Benhorin, J, Crotti, L, Kaufman, E, Locati, E, Ming, Q, Napolitano, C, Priori, S, Towbin, J, Vincent, G, Spazzolini C, Mullally J, Schwartz PJ, Moss AJ, McNitt S, Ouellet G, Fugate T, Goldenberg I, Jons C, Zareba W, Robinson JL, Ackerman MJ, Benhorin J, Crotti L, Kaufman ES, Locati EH, Ming Q, Napolitano C, Priori SG, Towbin JA, and Vincent GM.

Abstract

Objectives: This study was designed to evaluate the clinical and prognostic aspects of long QT syndrome (LQTS)-related cardiac events that occur in the first year of life (infancy). Background: The clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously. Methods: The study population of 3,323 patients with QT interval corrected for heart rate (QTc) ≥450 ms enrolled in the International LQTS Registry involved 20 patients with sudden cardiac death (SCD), 16 patients with aborted cardiac arrest (ACA), 34 patients with syncope, and 3,253 patients who were asymptomatic during the first year of life. Results: The risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc ≥500 ms, heart rate ≤100 beats/min, and female sex. An ACA before age 1 year was associated with a hazard ratio of 23.4 (p < 0.01) for ACA or SCD during ages 1 to 10 years. During the 10-year follow-up after infancy, beta-blocker therapy was associated with a significant reduction in ACA/SCD only in those with a syncopal episode within 2 years before ACA/SCD but not for those who survived ACA in infancy. Conclusions: Patients with LQTS who experience ACA during the first year of life are at very high risk for subsequent ACA or death during their next 10 years of life, and beta-blockers might not be effective in preventing fatal or near-fatal cardiac events in this small but high-risk subset

Published
2009

12. Cardiac events in genotyped long QT syndrome patients

Author

Zareba, W., primary, Moss, A.J., additional, Robinson, J., additional, Schwart, P.J., additional, Vincent, G.M., additional, Priori, S.G., additional, Benhorin, J., additional, Locati, E.H., additional, Towbin, J.A., additional, Keating, M.T., additional, Lehmann, M.H., additional, Hall, W.J., additional, Napolitano, C., additional, and Andrews, M., additional

Published
1998
Full Text
View/download PDF

16. The phenomenon of "QT stunning": the abnormal QT prolongation provoked by standing persists even as the heart rate returns to normal in patients with long QT syndrome.

Author

Adler A, van der Werf C, Postema PG, Rosso R, Bhuiyan ZA, Kalman JM, Vohra JK, Guevara-Valdivia ME, Marquez MF, Halkin A, Benhorin J, Antzelevitch C, Wilde AA, Viskin S, Adler, Arnon, van der Werf, Christian, Postema, Pieter G, Rosso, Raphael, Bhuiyan, Zahir A, and Kalman, Jonathan M

Abstract

Background: Patients with long QT syndrome (LQTS) have inadequate shortening of the QT interval in response to the sudden heart rate accelerations provoked by standing-a phenomenon of diagnostic value. We now validate our original observations in a cohort twice as large. We also describe that this abnormal QT-interval response persists as the heart rate acceleration returns to baseline.Objectives: To describe a novel observation, termed "QT stunning" and to validate previous observations regarding the "QT-stretching" phenomenon in patients with LQTS by using our recently described "standing test."Methods: The electrocardiograms of 108 patients with LQTS and 112 healthy subjects were recorded in the supine position. Subjects were then instructed to stand up quickly and remain standing for 5 minutes during continuous electrocardiographic recording. The corrected QT interval was measured at baseline (QTc(base)), when heart rate acceleration without appropriate QT-interval shortening leads to maximal QT stretching (QTc(stretch)) and upon return of heart rate to baseline (QTc(return)).Results: QTc(stretch) lengthened significantly more in patients with LQTS (103 ± 80 ms vs 66 ± 40 ms in controls; P <.001) and so did QTc(return) (28 ± 48 ms for patients with LQTS vs -3 ± 32 ms for controls; P <.001). Using a sensitivity cutoff of 90%, the specificity for diagnosing LQTS was 74% for QTc(base), 84% for QTc(return), and 87% for QTc(stretch).Conclusions: The present study extends our previous findings on the abnormal response of the QT interval in response to standing in patients with LQTS. Our study also shows that this abnormal response persists even after the heart rate slows back to baseline. [ABSTRACT FROM AUTHOR]

Published
2012
Full Text
View/download PDF

19. Risk of death in the long QT syndrome when a sibling has died.

Author

Kaufman ES, McNitt S, Moss AJ, Zareba W, Robinson JL, Hall WJ, Ackerman MJ, Benhorin J, Locati ET, Napolitano C, Priori SG, Schwartz PJ, Towbin JA, Vincent GM, Zhang L, Kaufman, Elizabeth S, McNitt, Scott, Moss, Arthur J, Zareba, Wojciech, and Robinson, Jennifer L

Abstract

Background: Sudden death of a sibling is thought to be associated with greater risk of death in long QT syndrome (LQTS). However, there is no evidence of such an association.Objective: This study sought to test the hypothesis that sudden death of a sibling is a risk factor for death or aborted cardiac arrest (ACA) in patients with LQTS.Methods: We examined all probands and first-degree and second-degree relatives in the International Long QT Registry from birth to age 40 years with QTc >/= 0.45 s. Covariates included sibling death, QTc, gender by age, syncope, and implantable cardioverter-defibrillator (ICD) and beta-blocker treatment. End points were (1) severe events (ACA, LQTS-related death) and (2) any cardiac event (syncope, ACA, or LQTS-related death).Results: Of 1915 subjects, 270 had a sibling who died. There were 213 severe events and 829 total cardiac events. More subjects with history of sibling death received beta-blocker therapy. Sibling death was not significantly associated with risk of ACA or LQTS-related death, but was associated with increased risk of syncope. QTc >/= 0.53 s (hazard ratio 2.5, P <.01), history of syncope (hazard ratio 6.1, P <.01), and gender were strongly associated with risk of ACA or LQTS-related death.Conclusion: Sudden death of a sibling prompted more aggressive treatment but did not predict risk of death or ACA, whereas QTc >/= 0.53 s, gender, and syncope predicted this risk. All subjects should receive appropriate beta-blocker therapy. The decision to implant an ICD should be based on an individual's own risk characteristics (QTc, gender, and history of syncope). [ABSTRACT FROM AUTHOR]

Published
2008
Full Text
View/download PDF

21. Long-QT syndrome after age 40.

Author

Goldenberg I, Moss AJ, Bradley J, Polonsky S, Peterson DR, McNitt S, Zareba W, Andrews ML, Robinson JL, Ackerman MJ, Benhorin J, Kaufman ES, Locati EH, Napolitano C, Priori SG, Qi M, Schwartz PJ, Towbin JA, Vincent GM, and Zhang L

Published
2008
Full Text
View/download PDF

23. Clinical, genetic, and electrophysiologic characteristics of a new PAS-domain HERG mutation (M124R) causing Long QT syndrome.

Author

Shushi L, Kerem B, Goldmit M, Peretz A, Attali B, Medina A, Towbin JA, Kurokawa J, Kass RS, Benhorin J, Shushi, Liat, Kerem, Batsheva, Goldmit, Maya, Peretz, Asher, Attali, Bernard, Medina, Aron, Towbin, Jeffrey A, Kurokawa, Junko, Kass, Robert S, and Benhorin, Jesaia

Abstract

Objectives: To describe the clinical, genetic, and electrophysiologic characteristics of a new PAS-domain HERG mutation (M124R) that has been identified in a single large Jewish family with Long QT syndrome (LQTS).Background: Many previously reported HERG mutations causing LQTS are located either in the C-terminus, or in the pore region. Relatively fewer clinical data are available on N-terminus (PAS-domain) mutation carriers.Methods: Clinical data were available in 76 family members (aged 1-93 years, 69 alive) over 18 years of follow-up, while electrocardiographic data were available in 57, and genetic data in 45 family members. Cellular electrophysiology was assessed in transfected Chinese Hamster Ovary (CHO) cells using the whole-cell patch-clamp technique.Results: Thirty-six family members were phenotypically categorized as nonaffected, 3 as equivocal, and 20 as affected. Mean QTc was 410+/-23, 440+/-10, and 498+/-41 ms, respectively, in these three subgroups. Eight out of 20 affected family members were symptomatic: five had only syncope, two had aborted cardiac arrest, and one sudden death. Genetic analyses identified the M124R point mutation in all affected members tested (n=16), while all those tested with nonaffected (n=26) and equivocal (n=3) phenotype did not carry the mutation. The M124R mutation reduced the HERG tail-current density by 65%, significantly accelerated the deactivation kinetics, and caused a negative shift in the voltage dependence of activation.Conclusions: A new PAS-domain HERG mutation (M124R) was identified as causing LQTS in a large Jewish family, with high penetrance and frequent disease-related symptoms. This mutation markedly decreased the tail-current density and accelerated the deactivation kinetics of the HERG channel in transfected CHO cells. [ABSTRACT FROM AUTHOR]

Published
2005

24. ISHNE guidelines for electrocardiographic evaluation of drug-related QT prolongation and other alterations in ventricular repolarization: task force summary. A report of the Task Force of the International Society for Holter and Noninvasive Electrocardiology (ISHNE), Committee on Ventricular Repolarization.

Author

Moss, Arthur J., Wojciech Zareba, Chair, Benhorin, Jesaia, Couderc, Jean-Philippe, Kennedy, Harold, Emanuela Locati-Heilbron, Maison-Blanche, Pierre, Moss, A J, Zareba, W, Benhorin, J, Couderc, J P, Kennedy, H, Locati-Heilbron, E, and Maison-Blanche, P

Published
2001
Full Text
View/download PDF

41. Isolated Huge Aneurysm of the Non-coronary Sinus of Valsalva.

Author

Chenzbraun, A., Benhorin, J., Milgarter, E., and Keren, A.

Subjects
VALSALVA'S maneuver, ANEURYSMS, ECHOCARDIOGRAPHY, SAPHENOUS vein
Abstract

The article presents a case study of a 40-year-old male with acute anterior myocardial infarction. The Valsalva's non-coronary sinus was found with isolated aneurysms through transoesophageal echocardiography. The diagnosis was made following the presentation of an acute myocardial infarction. The patient went through replacement of composite graft aortic root and saphenous vein graft.

Published
2001
Full Text
View/download PDF

42. Prospective study of bacteremia after cardiac catheterization.

Author

Banai S, Selitser V, Keren A, Benhorin J, Ben Shitrit O, Yalon S, Halperin E, Banai, Shmuel, Selitser, Vera, Keren, Andre, Benhorin, Jesaia, Shitrit, Orly Ben, Yalon, Shaul, and Halperin, Efraim

Abstract

Nine hundred sixty consecutive cardiac catheterization procedures were studied prospectively for the presence of periprocedural bacteremia. Overall, among 960 procedures, only 4 were associated with clinically significant bacteremia. All 4 were related to the intravenous line and none to the cardiac procedure itself. Clinically nonsignificant bacteremias were correlated with procedural duration, multiple skin punctures, use of multiple balloons, and obesity. [ABSTRACT FROM AUTHOR]

Published
2003
Full Text
View/download PDF

43. Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with long QT syndrome.

Author

Ali, R H, Zareba, W, Moss, A J, Schwartz, P J, Benhorin, J, Vincent, G M, Locati, E H, Priori, S, Napolitano, C, Towbin, J A, Hall, W J, Robinson, J L, Andrews, M L, Zhang, L, Timothy, K, and Medina, A

Abstract

In patients with the long QT syndrome (LQTS), the occurrence of cardiac events (syncope or cardiac arrest) is frequently associated with acute arousal caused by exercise, swimming, emotion, or noise. However, cardiac events may also occur during sleep or with ordinary daily activities. The purpose of this study was to determine whether there are differential clinical, electrocardiographic, and genetic features among LQTS patients who experienced cardiac events with and without acute arousal. We identified 1,325 patients with cardiac events from the International LQTS Registry. Based on the precipitating conditions of the first event, 427 patients were classified as arousal, 345 as nonarousal, and the remaining 553 were unknown (not classifiable). Gene linkage was known in 78 of the 772 patients with classifiable first events. The age at first cardiac event was significantly younger in the arousal than the nonarousal group (11.7 vs. 15.5 years, respectively; p<0.001). The arousal-type patients had a higher rate of subsequent cardiac events during follow-up after the index event than the nonarousal-type patients (p = 0.02). Arousal-related cardiac events occurred in 85% of LQT1, 67% of LQT2, and 33% of LQT3 patients (p = 0.008). This study provides evidence that the genotype is an important determinant of the LQTS phenotype in terms of arousal and nonarousal-related cardiac events. [ABSTRACT FROM AUTHOR]

Published
2000
Full Text
View/download PDF

44. Clinical Aspects of Type 3 Long-QT Syndrome

Author

Derick R. Peterson, Marielle Alders, Connie R. Bezzina, Wataru Shimizu, Takeshi Aiba, Michael J. Ackerman, Bronislava Polonsky, Coeli M. Lopes, Yoshihiro Miyamoto, Arthur J. Moss, David J. Tester, Jørgen K. Kanters, Carla Spazzolini, Jennifer L. Robinson, Ming Qi, Jeffrey A. Towbin, Arthur A.M. Wilde, Wojciech Zareba, Ilan Goldenberg, Elizabeth S. Kaufman, Jesaia Benhorin, Nynke Hofman, Mark L. Andrews, Lia Crotti, Peter J. Schwartz, Scott McNitt, Shiro Kamakura, Cardiology, Human Genetics, Wilde, A, Moss, A, Kaufman, E, Shimizu, W, Peterson, D, Benhorin, J, Lopes, C, Towbin, J, Spazzolini, C, Crotti, L, Zareba, W, Goldenberg, I, Kanters, J, Robinson, J, Qi, M, Hofman, N, Tester, D, Bezzina, C, Alders, M, Aiba, T, Kamakura, S, Miyamoto, Y, Andrews, M, Mcnitt, S, Polonsky, B, Schwartz, P, and Ackerman, M

Subjects
Male, Pediatrics, cardiac event, risk stratification, 030204 cardiovascular system & hematology, Sodium Channels, Electrocardiography, 0302 clinical medicine, Cardiac Conduction System Disease, Registries, Young adult, Child, Sex Characteristics, medicine.diagnostic_test, clinical manifestation, Long QT Syndrome, Child, Preschool, Risk stratification, Female, long qt syndrome type 3, Cardiology and Cardiovascular Medicine, Risk assessment, Adult, medicine.medical_specialty, Adolescent, medicine.drug_class, Long QT syndrome, Adrenergic beta-Antagonists, Risk Assessment, Syncope, Article, Young Adult, 03 medical and health sciences, Physiology (medical), medicine, Humans, In patient, Intensive care medicine, Beta blocker, Genetic testing, therapy, business.industry, Infant, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, Heart Arrest, Scn5a, Genetic Testing, Long Qt Syndrome, Risk Stratification, Sudden Cardiac Death, Arrhythmia, beta blocker, Multicenter study, business, 030217 neurology & neurosurgery
Abstract

Background: Risk stratification in patients with type 3 long-QT syndrome (LQT3) by clinical and genetic characteristics and effectiveness of β-blocker therapy has not been studied previously in a large LQT3 population. Methods: The study population included 406 LQT3 patients with 51 sodium channel mutations; 391 patients were known to be event free during the first year of life and were the focus of our study. Clinical, electrocardiographic, and genetic parameters were acquired for patients from 7 participating LQT3 registries. Cox regression analysis was used to evaluate the independent contribution of clinical, genetic, and therapeutic factors to the first occurrence of time-dependent cardiac events (CEs) from age 1 to 41 years. Results: Of the 391 patients, 118 (41 males, 77 females) patients (30%) experienced at least 1 CE (syncope, aborted cardiac arrest, or long-QT syndrome–related sudden death), and 24 (20%) suffered from LQT3-related aborted cardiac arrest/sudden death. The risk of a first CE was directly related to the degree of QTc prolongation. Cox regression analysis revealed that time-dependent β-blocker therapy was associated with an 83% reduction in CEs in females ( P =0.015) but not in males (who had many fewer events), with a significant sex × β-blocker interaction ( P =0.04). Each 10-ms increase in QTc duration up to 500 ms was associated with a 19% increase in CEs. Prior syncope doubled the risk for life-threatening events ( P Conclusions: Prolonged QTc and syncope predispose patients with LQT3 to life-threatening CEs. However, β-blocker therapy reduces this risk in females; efficacy in males could not be determined conclusively because of the low number of events.

Published
2016

45. Asthma and the risk of cardiac events in the Long QT syndrome. Long QT Syndrome Investigative Group.

Author

Rosero, Spencer Z., Zareba, Wojciech, Rosero, S Z, Zareba, W, Moss, A J, Robinson, J L, Hajj Ali, R H, Locati, E H, Benhorin, J, and Andrews, M L

Subjects
*CARDIAC arrest, *ASTHMA
Abstract

While acquiring data for the International Long QT Syndrome Registry, we noticed that a number of patients referred for long QT syndrome (LQTS) were affected by asthma. The effect of asthma comorbidity on clinical course of LQTS has not been studied. This study aimed to evaluate the prevalence of asthma in patients with LQTS, determine the influence of asthma comorbidity on outcome of LQTS patients, and to investigate the confounding effects of beta mimetics and beta blockers on the occurrence of cardiac events in asthmatic patients. The influence of asthma on risk of cardiac events (syncope, aborted cardiac arrest, or LQTS death) was evaluated after accounting for age, gender, QTc, and RR interval duration, beta-blocker and beta-mimetic use. Asthma was identified in 226 (5.2%) of 4,310 studied LQTS family members. Longer QTc duration was associated with higher incidence of asthma (p <0.001). Asthma was independently associated with significantly increased risk of cardiac events in affected LQTS patients (hazard ratio 1.32; p = 0.048) and in borderline-affected family members (hazard ratio 2.08; p = 0.004) after adjustment for QTc, RR interval, and gender. An increased risk of cardiac events in asthmatic patients observed before beta-blocker therapy was reduced after initiation of treatment with beta blockers. In conclusion, the occurrence of asthma in LQTS patients increases with QTc duration. Asthma comorbidity in LQTS patients is associated with an increased risk of cardiac events. The asthma-associated increase in the risk of LQTS-related cardiac events is diminished after initiation of beta-blocker therapy, suggesting a possible role of beta-receptor modulation underlying asthma-LQTS association. [ABSTRACT FROM AUTHOR]

Published
1999
Full Text
View/download PDF

46. Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome.

Author

Moss, Arthur J., Robinson, Jennifer L., Moss, A J, Robinson, J L, Gessman, L, Gillespie, R, Zareba, W, Schwartz, P J, Vincent, G M, Benhorin, J, Heilbron, E L, Towbin, J A, Priori, S G, Napolitano, C, Zhang, L, Medina, A, Andrews, M L, and Timothy, K

Subjects
*HEART disease risk factors, *CORONARY heart disease risk factors
Abstract

Acute auditory stimuli and swimming activities are frequently associated with syncope, aborted cardiac arrest, and death in the long QT syndrome (LQTS). We investigated the clinical and genetic findings associated with cardiac events precipitated by these arousal factors. The study population involved 195 patients with an index cardiac event associated with a loud noise (n = 77) or swimming activity (n = 118). Patients with events associated with loud auditory stimuli were older at their index event and were more likely to be women than patients who experienced events during swimming-related activities. Patients with an index event associated with loud noise were likely to have subsequent events related to auditory stimuli; patients with an index event associated with swimming were likely to have recurrent events related to swimming or physical activities. Family patterning of auditory and swimming and/or physical activity-related events was evident. Genotype analyses in 25 patients revealed a significant difference in the distribution of index cardiac events by genotype (p <0.001), with all 19 patients with swimming-related episodes associated with LQT1 genotype and 5 of 6 patients with auditory-related events associated with LQT2 genotype. The clinical profile and genotype findings of patients with LQTS who experience cardiac events related to acute auditory stimuli are quite different from those who experience events accompanying swimming activities. [ABSTRACT FROM AUTHOR]

Published
1999
Full Text
View/download PDF

47. Clinical Implications for Patients With Long QT Syndrome Who Experience a Cardiac Event During Infancy

Author

Thomas Fugate, Gregory M. Ouellet, Carla Spazzolini, Peter J. Schwartz, Arthur J. Moss, Lia Crotti, Jennifer L. Robinson, Jamie Mullally, Wojciech Zareba, Scott McNitt, Silvia G. Priori, Christian Jons, Jesaia Benhorin, Jeffrey A. Towbin, Ming Qi, Ilan Goldenberg, G. Michael Vincent, Emanuela H. Locati, Elizabeth S. Kaufman, Michael J. Ackerman, Carlo Napolitano, Spazzolini, C, Mullally, J, Schwartz, P, Moss, A, Mcnitt, S, Ouellet, G, Fugate, T, Goldenberg, I, Jons, C, Zareba, W, Robinson, J, Ackerman, M, Benhorin, J, Crotti, L, Kaufman, E, Locati, E, Ming, Q, Napolitano, C, Priori, S, Towbin, J, and Vincent, G

Subjects
Adult, Male, congenital, hereditary, and neonatal diseases and abnormalities, medicine.medical_specialty, Adolescent, Heart disease, Heart block, Long QT syndrome, risk stratification, Risk Assessment, Asymptomatic, QT interval, Article, Sudden cardiac death, Electrocardiography, Risk Factors, Internal medicine, medicine, Humans, genetics, cardiovascular diseases, Child, medicine.diagnostic_test, infants, business.industry, Hazard ratio, Infant, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, Heart Arrest, long qt syndrome, major cardiac event, arrhythmia, infancy, clinical severity, Long QT Syndrome, Death, Sudden, Cardiac, Child, Preschool, cardiovascular system, Cardiology, Female, medicine.symptom, Cardiology and Cardiovascular Medicine, business
Abstract

Objectives: This study was designed to evaluate the clinical and prognostic aspects of long QT syndrome (LQTS)-related cardiac events that occur in the first year of life (infancy). Background: The clinical implications for patients with long QT syndrome who experience cardiac events in infancy have not been studied previously. Methods: The study population of 3,323 patients with QT interval corrected for heart rate (QTc) ≥450 ms enrolled in the International LQTS Registry involved 20 patients with sudden cardiac death (SCD), 16 patients with aborted cardiac arrest (ACA), 34 patients with syncope, and 3,253 patients who were asymptomatic during the first year of life. Results: The risk factors for a cardiac event among 212 patients who had an electrocardiogram recorded in the first year of life included QTc ≥500 ms, heart rate ≤100 beats/min, and female sex. An ACA before age 1 year was associated with a hazard ratio of 23.4 (p < 0.01) for ACA or SCD during ages 1 to 10 years. During the 10-year follow-up after infancy, beta-blocker therapy was associated with a significant reduction in ACA/SCD only in those with a syncopal episode within 2 years before ACA/SCD but not for those who survived ACA in infancy. Conclusions: Patients with LQTS who experience ACA during the first year of life are at very high risk for subsequent ACA or death during their next 10 years of life, and beta-blockers might not be effective in preventing fatal or near-fatal cardiac events in this small but high-risk subset

Published
2009

48. Mutation-Specific Risk in Two Genetic Forms of Type 3 Long QT Syndrome

Author

Michael J. Ackerman, Lia Crotti, Ilan Goldenberg, Wojciech Zareba, Jesaia Benhorin, Peter J. Schwartz, Ming Qi, Jennifer L. Robinson, Christian Jons, Scott McNitt, Arthur J. Moss, Judy F. Liu, Carla Spazzolini, Liu, J, Moss, A, Jons, C, Benhorin, J, Schwartz, P, Spazzolini, C, Crotti, L, Ackerman, M, Mcnitt, S, Robinson, J, Qi, M, Goldenberg, I, and Zareba, W

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, Long QT syndrome, Mutation, Missense, Muscle Proteins, QT interval, Sudden death, Article, Sodium Channels, NAV1.5 Voltage-Gated Sodium Channel, Cohort Studies, Electrocardiography, Young Adult, Internal medicine, medicine, Missense mutation, Humans, Genetic Predisposition to Disease, Registries, Risk factor, Child, Survival analysis, Retrospective Studies, Sequence Deletion, business.industry, Proportional hazards model, long qt syndrome, long qt syndrome type 3, arrhythmic risk, genetics, mutation, Infant, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, Survival Analysis, Long QT Syndrome, Child, Preschool, Mutation (genetic algorithm), Cardiology, Female, Cardiology and Cardiovascular Medicine, business
Abstract

The clinical course of patients with 2 relatively common long QT syndrome type 3 mutations has not been well described. In the present study, we investigated the mutational-specific risk in patients with deletional (DeltaKPQ) and missense (D1790G) mutations involving the SCN5A gene. The study population involved 50 patients with the DeltaKPQ mutation and 35 patients with the D1790G mutation. The cumulative probability of a first cardiac event (syncope, aborted cardiac arrest, or long QT syndrome-related sudden death) was evaluated using the Kaplan-Meier method. The Cox proportional hazards survivorship model was used to determine the independent contribution of clinical and genetic factors to the first occurrence of cardiac events from birth through 40 years of age. The Andersen-Gill proportional intensity regression model was used to analyze the factors associated with recurrent syncope. Patients with a DeltaKPQ mutation had a significantly greater probability of a first cardiac event from birth through 40 years of age (34%) than those with the D1790G mutation (20%; p

Published
2010

49. Long-term flecainide therapy in type 3 long QT syndrome.

Author

Chorin E, Taub R, Medina A, Flint N, Viskin S, and Benhorin J

Subjects
Adolescent, Adult, Anti-Arrhythmia Agents adverse effects, Brugada Syndrome chemically induced, Cardiac Conduction System Disease diagnosis, Cardiac Conduction System Disease genetics, Cardiac Conduction System Disease physiopathology, Child, Clinical Decision-Making, Electrocardiography, Female, Flecainide adverse effects, Genetic Predisposition to Disease, Humans, Long QT Syndrome diagnosis, Long QT Syndrome genetics, Long QT Syndrome physiopathology, Male, Mutation, NAV1.5 Voltage-Gated Sodium Channel genetics, Patient Selection, Phenotype, Risk Factors, Time Factors, Treatment Outcome, Voltage-Gated Sodium Channel Blockers adverse effects, Young Adult, Anti-Arrhythmia Agents administration & dosage, Cardiac Conduction System Disease drug therapy, Flecainide administration & dosage, Long QT Syndrome drug therapy, Voltage-Gated Sodium Channel Blockers administration & dosage
Abstract

Aims: Type 3 long QT syndrome (LQT3) is caused by gain-of-function mutations in the cardiac sodium channel gene (SCN5A). Previous reports on the long-term use of sodium channel blockers in LQT3 are sparse. The objective of the current study was to evaluate the long-term safety and efficacy of flecainide therapy in patients with LQT3 who carry the D1790G SCN5A mutation., Methods and Results: The study population comprised 30 D1790G carriers who were treated with flecainide and followed for 1-215 months (mean 145 ± 54 months, median 140 months). The mean baseline (off-drug) QTc was 522 ± 45 ms, and shortened to 469 ± 36 ms with flecainide therapy, a mean decrease of 53 ms [10.1%] (P < 0.01). A QTc longer than 500 ms was evident in 53% of carriers at baseline, and only in 13% on flecainide. All carriers while being compliant with flecainide therapy had no cardiac events during an average follow up of 83 ± 73 months. Twenty carriers stopped flecainide after an average follow up of 40 ± 42 months without symptoms. Six of them (30%) had cardiac events 1-11 months after stopping flecainide. Flecainide induced the appearance of Brugada pattern in six carriers (20%, 5 males), was stopped in three and was not associated with arrhythmia. Sinus-node dysfunction was evident in six carriers (20%) and was fully corrected by flecainide in three., Conclusions: These data suggest that long-term flecainide therapy is relatively safe and effective among LQT3 patients who carry the D1790G SCN5A mutation., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.)

Published
2018
Full Text
View/download PDF

50. Ranolazine for Congenital Long-QT Syndrome Type III: Experimental and Long-Term Clinical Data.

Author

Chorin E, Hu D, Antzelevitch C, Hochstadt A, Belardinelli L, Zeltser D, Barajas-Martinez H, Rozovski U, Rosso R, Adler A, Benhorin J, and Viskin S

Subjects
Adolescent, Adult, Aged, Electrocardiography, Female, Humans, Israel, Male, Middle Aged, Phenotype, Treatment Outcome, Long QT Syndrome drug therapy, Long QT Syndrome genetics, NAV1.5 Voltage-Gated Sodium Channel genetics, Ranolazine therapeutic use, Sodium Channel Blockers therapeutic use
Abstract

Background: The basic defect in long-QT syndrome type III (LQT3) is an excessive inflow of sodium current during phase 3 of the action potential caused by mutations in the SCN5A gene. Most sodium channel blockers reduce the early (peak) and late components of the sodium current (I Na and I NaL ), but ranolazine preferentially reduces I NaL . We, therefore, evaluated the effects of ranolazine in LQT3 caused by the D1790G mutation in SCN5A., Methods and Results: We performed an experimental study of ranolazine in TSA201 cells expressing the D1790G mutation. We then performed a long-term clinical evaluation of ranolazine in LQT3 patients carrying the D1790G mutation. In the experimental study, I NaL was significantly higher in D1790G than in wild-type channels expressed in the TSA201 cells. Ranolazine exerted a concentration-dependent block of I NaL of the SCN5A-D1790G channel without reducing peak I Na significantly. In the clinical study, among 8 patients with LQT3 and confirmed D1790G mutation, ranolazine had no effects on the sinus rate or QRS width but shortened the QTc from 509±41 to 451±26 ms, a mean decrease of 56±52 ms (10.6%; P=0.012). The QT-shortening effect of ranolazine remained effective throughout the entire study period of 22.8±12.8 months. Ranolazine reduced the QTc at all heart rates but less so during extreme nocturnal bradycardia. A type I Brugada ECG was never noticed., Conclusions: Ranolazine blocks I NaL in experimental models of LQT3 harboring the SCN5A-D1790G mutation and shortened the QT interval of LQT3 patients., Clinical Trial Registration: URL: https://clinicaltrials.gov; Unique identifier: NCT01728025., (© 2016 American Heart Association, Inc.)

Published
2016
Full Text
View/download PDF

Catalog

Books, media, physical & digital resources
See catalog results