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1. Proposition d'explication de la formation d'hydrogène sulfuré dans les stockages souterrains de gaz naturel par réduction des sulfures minéraux de la roche magasin Proposed Explanation of Hydrogen-Sulfide Formation in Underground Natural-Gas Storage Structures by Reduction of Mineral Sulfides in the Reservoir Rock.

Author

Bourgeois J. P., Aupaix N., Bloise R., and Millet J. L.

Subjects
Chemical technology, TP1-1185, Energy industries. Energy policy. Fuel trade, HD9502-9502.5
Abstract

La formation d'hydrogène sulfuré dans les structures de stockage peu expliquer autrement que par l'action de bactéries sulfato-réductrices. La contenue dans la roche magasin constitue une source de sulfures capable d'alimenter en H2S le gaz naturel. La réduction de la pyrite en sulfures du type Fe 1-x S et l'équilibre de dissolution précipitation, lié principalement à la pression de CO2, dans les structures stockages, constituent un processus de formation d'H2S capable d'expliquer tativement et quantitativement les phénomènes observés sur le terrain. Un modèle simplifié de stockage reprend ce schéma et teste la sensibililté de la teneur en H2S à la valeur des paramètres physiques et chimiques définissant le stockage. Cette étude permet de proposer un certain nombre d'actions susceptibles de limiter la formation d'H2S et d'orienter les choix futurs du couple gaz naturel - structures de stockage. The formation of hydrogen sulfide in storage structures can be explained otherwise thon by the action of sulfate-reducing bacteria. The pyrite contained in the reservoir rock makes up a source of sulfides capable of supplying the natural gas with H2S.Reduction of pyrite ta sulfides of the Fe,-,S type and the dissolution precipitation equilibrium, linked mainly ta C02 pressure in storage structures, make up an H2S for-mation process capable of qualitatively and quantitatively explained phenomena observed in the field.A simplified storage model reflects this scheme and can be used ta test the sensi-tivity of the H2S content ta the value of the physical and chemical parameters defining the storage structure.This investigation can be used to propose various means of action (sable ta "mit H2S formation and ta guide future choices of natural gas/storage-structure pairs.

Published
2006
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8. Natural History and Risk Stratification in Andersen-Tawil Syndrome Type 1

Author

Mazzanti, A, Guz, D, Trancuccio, A, Pagan, E, Kukavica, D, Chargeishvili, T, Olivetti, N, Biernacka, E, Sacilotto, L, Sarquella-Brugada, G, Campuzano, O, Nof, E, Anastasakis, A, Sansone, V, Jimenez-Jaimez, J, Cruz, F, Sanchez-Quinones, J, Hernandez-Afonso, J, Fuentes, M, Sredniawa, B, Garoufi, A, Andrsova, I, Izquierdo, M, Marinov, R, Danon, A, Exposito-Garcia, V, Garcia-Fernandez, A, Munoz-Esparza, C, Ortiz, M, Zienciuk-Krajka, A, Tavazzani, E, Monteforte, N, Bloise, R, Marino, M, Memmi, M, Napolitano, C, Zorio, E, Monserrat, L, Bagnardi, V, Priori, S, Mazzanti A., Guz D., Trancuccio A., Pagan E., Kukavica D., Chargeishvili T., Olivetti N., Biernacka E. K., Sacilotto L., Sarquella-Brugada G., Campuzano O., Nof E., Anastasakis A., Sansone V. A., Jimenez-Jaimez J., Cruz F., Sanchez-Quinones J., Hernandez-Afonso J., Fuentes M. E., Sredniawa B., Garoufi A., Andrsova I., Izquierdo M., Marinov R., Danon A., Exposito-Garcia V., Garcia-Fernandez A., Munoz-Esparza C., Ortiz M., Zienciuk-Krajka A., Tavazzani E., Monteforte N., Bloise R., Marino M., Memmi M., Napolitano C., Zorio E., Monserrat L., Bagnardi V., Priori S. G., Mazzanti, A, Guz, D, Trancuccio, A, Pagan, E, Kukavica, D, Chargeishvili, T, Olivetti, N, Biernacka, E, Sacilotto, L, Sarquella-Brugada, G, Campuzano, O, Nof, E, Anastasakis, A, Sansone, V, Jimenez-Jaimez, J, Cruz, F, Sanchez-Quinones, J, Hernandez-Afonso, J, Fuentes, M, Sredniawa, B, Garoufi, A, Andrsova, I, Izquierdo, M, Marinov, R, Danon, A, Exposito-Garcia, V, Garcia-Fernandez, A, Munoz-Esparza, C, Ortiz, M, Zienciuk-Krajka, A, Tavazzani, E, Monteforte, N, Bloise, R, Marino, M, Memmi, M, Napolitano, C, Zorio, E, Monserrat, L, Bagnardi, V, Priori, S, Mazzanti A., Guz D., Trancuccio A., Pagan E., Kukavica D., Chargeishvili T., Olivetti N., Biernacka E. K., Sacilotto L., Sarquella-Brugada G., Campuzano O., Nof E., Anastasakis A., Sansone V. A., Jimenez-Jaimez J., Cruz F., Sanchez-Quinones J., Hernandez-Afonso J., Fuentes M. E., Sredniawa B., Garoufi A., Andrsova I., Izquierdo M., Marinov R., Danon A., Exposito-Garcia V., Garcia-Fernandez A., Munoz-Esparza C., Ortiz M., Zienciuk-Krajka A., Tavazzani E., Monteforte N., Bloise R., Marino M., Memmi M., Napolitano C., Zorio E., Monserrat L., Bagnardi V., and Priori S. G.

Abstract

Background: Andersen-Tawil Syndrome type 1 (ATS1) is a rare arrhythmogenic disorder, caused by loss-of-function mutations in the KCNJ2 gene. We present here the largest cohort of patients with ATS1 with outcome data reported. Objectives: This study sought to define the risk of life-threatening arrhythmic events (LAE), identify predictors of such events, and define the efficacy of antiarrhythmic therapy in patients with ATS1. Methods: Clinical and genetic data from consecutive patients with ATS1 from 23 centers were entered in a database implemented at ICS Maugeri in Pavia, Italy, and pooled for analysis. Results: We enrolled 118 patients with ATS1 from 57 families (age 23 ± 17 years at enrollment). Over a median follow-up of 6.2 years (interquartile range: 2.7 to 16.5 years), 17 patients experienced a first LAE, with a cumulative probability of 7.9% at 5 years. An increased risk of LAE was associated with a history of syncope (hazard ratio [HR]: 4.54; p = 0.02), with the documentation of sustained ventricular tachycardia (HR 9.34; p = 0.001) and with the administration of amiodarone (HR: 268; p < 0.001). The rate of LAE without therapy (1.24 per 100 person-years [py]) was not reduced by beta-blockers alone (1.37 per 100 py; p = 1.00), or in combination with Class Ic antiarrhythmic drugs (1.46 per 100 py, p = 1.00). Conclusions: Our data demonstrate that the clinical course of patients with ATS1 is characterized by a high rate of LAE. A history of unexplained syncope or of documented sustained ventricular tachycardia is associated with a higher risk of LAE. Amiodarone is proarrhythmic and should be avoided in patients with ATS1.

Published
2020

9. Unexpected Risk Profile of a Large Pediatric Population With Brugada Syndrome

Author

Mazzanti, A, Ovics, P, Shauer, A, Mameli, S, Marino, M, Bloise, R, Monteforte, N, Raimondo, C, Maltret, A, Napolitano, C, Bagnardi, V, Priori, S, Mazzanti A., Ovics P., Shauer A., Mameli S., Marino M., Bloise R., Monteforte N., Raimondo C., Maltret A., Napolitano C., Bagnardi V., Priori S. G., Mazzanti, A, Ovics, P, Shauer, A, Mameli, S, Marino, M, Bloise, R, Monteforte, N, Raimondo, C, Maltret, A, Napolitano, C, Bagnardi, V, Priori, S, Mazzanti A., Ovics P., Shauer A., Mameli S., Marino M., Bloise R., Monteforte N., Raimondo C., Maltret A., Napolitano C., Bagnardi V., and Priori S. G.

Published
2019

11. Natural History and Risk Stratification in Andersen-Tawil Syndrome Type 1

Author

Mazzanti, A. Guz, D. Trancuccio, A. Pagan, E. Kukavica, D. Chargeishvili, T. Olivetti, N. Biernacka, E.K. Sacilotto, L. Sarquella-Brugada, G. Campuzano, O. Nof, E. Anastasakis, A. Sansone, V.A. Jimenez-Jaimez, J. Cruz, F. Sánchez-Quiñones, J. Hernandez-Afonso, J. Fuentes, M.E. Średniawa, B. Garoufi, A. Andršová, I. Izquierdo, M. Marinov, R. Danon, A. Expósito-García, V. Garcia-Fernandez, A. Muñoz-Esparza, C. Ortíz, M. Zienciuk-Krajka, A. Tavazzani, E. Monteforte, N. Bloise, R. Marino, M. Memmi, M. Napolitano, C. Zorio, E. Monserrat, L. Bagnardi, V. Priori, S.G.

Abstract

Background: Andersen-Tawil Syndrome type 1 (ATS1) is a rare arrhythmogenic disorder, caused by loss-of-function mutations in the KCNJ2 gene. We present here the largest cohort of patients with ATS1 with outcome data reported. Objectives: This study sought to define the risk of life-threatening arrhythmic events (LAE), identify predictors of such events, and define the efficacy of antiarrhythmic therapy in patients with ATS1. Methods: Clinical and genetic data from consecutive patients with ATS1 from 23 centers were entered in a database implemented at ICS Maugeri in Pavia, Italy, and pooled for analysis. Results: We enrolled 118 patients with ATS1 from 57 families (age 23 ± 17 years at enrollment). Over a median follow-up of 6.2 years (interquartile range: 2.7 to 16.5 years), 17 patients experienced a first LAE, with a cumulative probability of 7.9% at 5 years. An increased risk of LAE was associated with a history of syncope (hazard ratio [HR]: 4.54; p = 0.02), with the documentation of sustained ventricular tachycardia (HR 9.34; p = 0.001) and with the administration of amiodarone (HR: 268; p < 0.001). The rate of LAE without therapy (1.24 per 100 person-years [py]) was not reduced by beta-blockers alone (1.37 per 100 py; p = 1.00), or in combination with Class Ic antiarrhythmic drugs (1.46 per 100 py, p = 1.00). Conclusions: Our data demonstrate that the clinical course of patients with ATS1 is characterized by a high rate of LAE. A history of unexplained syncope or of documented sustained ventricular tachycardia is associated with a higher risk of LAE. Amiodarone is proarrhythmic and should be avoided in patients with ATS1. © 2020

Published
2020

18. Efficacy and Limitations of Quinidine in Patients with Brugada Syndrome

Author

Mazzanti, A, Tenuta, E, Marino, M, Pagan, E, Morini, M, Memmi, M, Colombi, B, Tibollo, V, Frassoni, S, Curcio, A, Raimondo, C, Maltret, A, Monteforte, N, Bloise, R, Napolitano, C, Bellazzi, R, Bagnardi, V, Priori, S, Mazzanti, Andrea, Tenuta, Elisavietta, Marino, Maira, Pagan, Eleonora, Morini, Massimo, Memmi, Mirella, Colombi, Barbara, Tibollo, Valentina, Frassoni, Samuele, Curcio, Antonio, Raimondo, Cristina, Maltret, Alice, Monteforte, Nicola, Bloise, Raffaella, Napolitano, Carlo, Bellazzi, Riccardo, Bagnardi, Vincenzo, Priori, Silvia G., Mazzanti, A, Tenuta, E, Marino, M, Pagan, E, Morini, M, Memmi, M, Colombi, B, Tibollo, V, Frassoni, S, Curcio, A, Raimondo, C, Maltret, A, Monteforte, N, Bloise, R, Napolitano, C, Bellazzi, R, Bagnardi, V, Priori, S, Mazzanti, Andrea, Tenuta, Elisavietta, Marino, Maira, Pagan, Eleonora, Morini, Massimo, Memmi, Mirella, Colombi, Barbara, Tibollo, Valentina, Frassoni, Samuele, Curcio, Antonio, Raimondo, Cristina, Maltret, Alice, Monteforte, Nicola, Bloise, Raffaella, Napolitano, Carlo, Bellazzi, Riccardo, Bagnardi, Vincenzo, and Priori, Silvia G.

Abstract

Background Quinidine at high dose is suggested as antiarrhythmic treatment in patients with Brugada Syndrome (BrS), but its efficacy to prevent life-threatening arrhythmic events (LAE) in this population is unproven and its use limited by frequent side effects. We assessed whether low-dose quinidine in patients with BrS reduces the following: (1) the occurrence of LAEs at follow-up, as compared to no therapy, in a population of both high-risk survivors of LAE and lower risk patients asymptomatic for LAEs and (2) the arrhythmic burden in the high-risk group of cardiac arrest survivors. Methods We compared the clinical course of 53 patients with BrS treated with quinidine to that of 441 untreated controls, matched by sex, age, symptoms, and the duration of observation. Furthermore, we calculated the annual incidence of LAE off-quinidine and on-quinidine in 123 patients with BrS who were cardiac arrest survivors. Results Fifty-three patients with BrS (89% males, median age 39.8 years) received quinidine (439±115 mg/d) for 5.0±3.7 years. Therapy was stopped in 3 cases (6%) for side effects. Quinidine reduced by 26% the risk of experiencing an LAE in cases versus controls (hazard ratio, 0.74; 95% CI, 0.22-2.48; P=0.62). Furthermore, in 27 of 123 patients with BrS symptomatic for LAEs who were treated for 7.0±3.5 years, the annual rate of LAEs decreased from 14.7% while off-quinidine to 3.9% while on-quinidine (P=0.03). Of note, recurrent LAEs were recorded in 4 (15%) cardiac arrest survivors while on-quinidine. Conclusions We demonstrated for the first time in the long-term that low-dose quinidine reduces recurrent LAEs in patients with BrS who had already survived an LAE, with few side effects. Remarkably, 15% of patients symptomatic for LAEs experience recurrent life-threatening arrhythmias while on-treatment, suggesting that quinidine cannot replace implantable defibrillators in high-risk subjects. Visual Overview A visual overview is available for this article.

Published
2019

22. Arrhythmogenic Right Ventricular Cardiomyopathy: Clinical Course and Predictors of Arrhythmic Risk

Author

Mazzanti, A, Ng, K, Faragli, A, Maragna, R, Chiodaroli, E, Orphanou, N, Monteforte, N, Memmi, M, Gambelli, P, Novelli, V, Bloise, R, Catalano, O, Moro, G, Tibollo, V, Morini, M, Bellazzi, R, Napolitano, C, Priori, S., BAGNARDI, VINCENZO, Mazzanti, A, Ng, K, Faragli, A, Maragna, R, Chiodaroli, E, Orphanou, N, Monteforte, N, Memmi, M, Gambelli, P, Novelli, V, Bloise, R, Catalano, O, Moro, G, Tibollo, V, Morini, M, Bellazzi, R, Napolitano, C, Bagnardi, V, and Priori, S

Subjects
implantable cardioverter-defibrillator, athlete, ventricular tachycardia, genetic, sudden cardiac death
Abstract

Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of sudden cardiac death, but its progression over time and predictors of arrhythmias are still being defined. Objectives This study sought to describe the clinical course of ARVC and occurrence of life-threatening arrhythmic events (LAE) and cardiovascular mortality; identify risk factors associated with increased LAE risk; and define the response to therapy. Methods We determined the clinical course of 301 consecutive patients with ARVC using the Kaplan-Meier method adjusted to avoid the bias of delayed entry. Predictors of LAE over 5.8 years of follow-up were determined with Cox multivariable analysis. Treatment efficacy was assessed comparing LAE rates during matched time intervals. Results A first LAE occurred in 1.5 per 100 person-years between birth and age 20 years, in 4.0 per 100 person-years between ages 21 and 40 years, and in 2.4 per 100 person-years between ages 41 and 60 years. Cumulative probability of a first LAE at follow-up was 14% at 5 years, 23% at 10 years, and 30% at 15 years. Higher risk of LAE was predicted by atrial fibrillation (hazard ratio [HR]: 4.38; p = 0.002), syncope (HR: 3.36; p < 0.001), participation in strenuous exercise after the diagnosis (HR: 2.98; p = 0.028), hemodynamically tolerated sustained monomorphic ventricular tachycardia (HR: 2.19; p = 0.023), and male sex (HR: 2.49; p = 0.012). No difference was observed in the occurrence of LAE before and after treatment with amiodarone, beta-blockers, sotalol, or ablation. A total of 81 patients received an implantable cardioverter-defibrillator, 34 were successfully defibrillated. Conclusions The high risk of life-threatening arrhythmias in patients with ARVC spans from adolescence to advanced age, reaching its peak between ages 21 and 40 years. Atrial fibrillation, syncope, participation in strenuous exercise after the diagnosis of ARVC, hemodynamically tolerated sustained monomorphic ventricular tachycardia, and male sex predicted lethal arrhythmias at follow-up. The lack of efficacy of antiarrhythmic therapy and the life-saving role of the implantable cardioverter-defibrillator highlight the importance of risk stratification for patient management.

Published
2016

23. Interplay Between Genetic Substrate, QTc Duration, and Arrhythmia Risk in Patients With Long QT Syndrome

Author

Mazzanti, A, Maragna, R, Vacanti, G, Monteforte, N, Bloise, R, Marino, M, Braghieri, L, Gambelli, P, Memmi, M, Pagan, E, Morini, M, Malovini, A, Ortiz, M, Sacilotto, L, Bellazzi, R, Monserrat, L, Napolitano, C, Bagnardi, V, Priori, S, Priori, SG, Mazzanti, A, Maragna, R, Vacanti, G, Monteforte, N, Bloise, R, Marino, M, Braghieri, L, Gambelli, P, Memmi, M, Pagan, E, Morini, M, Malovini, A, Ortiz, M, Sacilotto, L, Bellazzi, R, Monserrat, L, Napolitano, C, Bagnardi, V, Priori, S, and Priori, SG

Abstract

Background: Long QT syndrome (LQTS) is a common inheritable arrhythmogenic disorder, often secondary to mutations in the KCNQ1, KCNH2, and SCN5A genes. The disease is characterized by a prolonged ventricular repolarization (QTc interval) that confers susceptibility to life-threatening arrhythmic events (LAEs). Objectives: This study sought to create an evidence-based risk stratification scheme to personalize the quantification of the arrhythmic risk in patients with LQTS. Methods: Data from 1,710 patients with LQTS followed up for a median of 7.1 years (interquartile range [IQR]: 2.7 to 13.4 years) were analyzed to estimate the 5-year risk of LAEs based on QTc duration and genotype and to assess the antiarrhythmic efficacy of beta-blockers. Results: The relationship between QTc duration and risk of events was investigated by comparison of linear and cubic spline models, and the linear model provided the best fit. The 5-year risk of LAEs while patients were off therapy was then calculated in a multivariable Cox model with QTc and genotype considered as independent factors. The estimated risk of LAEs increased by 15% for every 10-ms increment of QTc duration for all genotypes. Intergenotype comparison showed that the risk for patients with LQT2 and LTQ3 increased by 130% and 157% at any QTc duration versus patients with LQT1. Analysis of response to beta-blockers showed that only nadolol reduced the arrhythmic risk in all genotypes significantly compared with no therapy (hazard ratio: 0.38; 95% confidence interval: 0.15 to 0.93; p = 0.03). Conclusions: The study provides an estimator of risk of LAEs in LQTS that allows a granular estimate of 5-year arrhythmic risk and demonstrate the superiority of nadolol in reducing the risk of LAEs in LQTS

Published
2018

24. Efficacy of a biological therapy for recessive Catecholaminergic Polymorphic Ventricular Tachycardia in human induced pluripotent stem cells-derived cardiomyocytes

Author

Lodola, F, Di Pasquale, E, Bongianino, R, Denegri, M, Rutigliano, L, Buonocore, M, Bloise, R, Condorelli, G, Napolitano, C, Priori, S, Lodola F, Di Pasquale E, Bongianino R, Denegri M, Rutigliano L, Buonocore M, Bloise R, Condorelli G, Napolitano C, Priori SG, Lodola, F, Di Pasquale, E, Bongianino, R, Denegri, M, Rutigliano, L, Buonocore, M, Bloise, R, Condorelli, G, Napolitano, C, Priori, S, Lodola F, Di Pasquale E, Bongianino R, Denegri M, Rutigliano L, Buonocore M, Bloise R, Condorelli G, Napolitano C, and Priori SG

Published
2015

25. Efficacy of a biological therapy for recessive Catecholaminergic Polymorphic Ventricular Tachycardia in human induced pluripotent stem cells-derived cardiomyocytes

Author

Lodola F, Di Pasquale E, Bongianino R, Denegri M, Rutigliano L, Buonocore M, Bloise R, Condorelli G, Napolitano C, Priori SG, Lodola, F, Di Pasquale, E, Bongianino, R, Denegri, M, Rutigliano, L, Buonocore, M, Bloise, R, Condorelli, G, Napolitano, C, and Priori, S

Subjects
Cardiac arrhythmias, gene therapy, physiology, Cardiac arrhythmias, gene therapy, CPVT, hiPS-CMs
Published
2015

27. Hydroquinidine Prevents Life-Threatening Arrhythmic Events in Patients With Short QT Syndrome

Author

Mazzanti, A, Maragna, R, Vacanti, G, Kostopoulou, A, Marino, M, Monteforte, N, Bloise, R, Underwood, K, Tibollo, V, Pagan, E, Napolitano, C, Bellazzi, R, Bagnardi, V, Priori, S, Priori, SG, Mazzanti, A, Maragna, R, Vacanti, G, Kostopoulou, A, Marino, M, Monteforte, N, Bloise, R, Underwood, K, Tibollo, V, Pagan, E, Napolitano, C, Bellazzi, R, Bagnardi, V, Priori, S, and Priori, SG

Abstract

Background Short 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. Objectives 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. Methods 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. Results 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). Conclusions 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.

Published
2017

28. CaMKII inhibition rectifies arrhythmic phenotype in a patient-specific model of Catecholaminergic Polymorphic Ventricular Tachycardia

Author

Di Pasquale, E, Lodola, F, Miragoli, M, Denegri, M, Avelino-Cruz, J, Buonocore, M, Nakahama, H, Portararo, P, Bloise, R, Napolitano, C, Condorelli, G, Priori, S, Di Pasquale E, Lodola F, Miragoli M, Denegri M, Avelino-Cruz JE, Buonocore M, Nakahama H, Portararo P, Bloise R, Napolitano C, Condorelli G, Priori SG, Di Pasquale, E, Lodola, F, Miragoli, M, Denegri, M, Avelino-Cruz, J, Buonocore, M, Nakahama, H, Portararo, P, Bloise, R, Napolitano, C, Condorelli, G, Priori, S, Di Pasquale E, Lodola F, Miragoli M, Denegri M, Avelino-Cruz JE, Buonocore M, Nakahama H, Portararo P, Bloise R, Napolitano C, Condorelli G, and Priori SG

Abstract

Induced pluripotent stem cells (iPSC) offer a unique opportunity for developmental studies, disease modeling and regenerative medicine approaches in humans. The aim of our study was to create an in vitro 'patient-specific cell-based system' that could facilitate the screening of new therapeutic molecules for the treatment of catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited form of fatal arrhythmia. Here, we report the development of a cardiac model of CPVT through the generation of iPSC from a CPVT patient carrying a heterozygous mutation in the cardiac ryanodine receptor gene (RyR2) and their subsequent differentiation into cardiomyocytes (CMs). Whole-cell patch-clamp and intracellular electrical recordings of spontaneously beating cells revealed the presence of delayed afterdepolarizations (DADs) in CPVT-CMs, both in resting conditions and after beta-adrenergic stimulation, resembling the cardiac phenotype of the patients. Furthermore, treatment with KN-93 (2-[N-(2-hydroxyethyl)]-N-(4methoxybenzenesulfonyl)]amino-N-(4-chlorocinnamyl)-N-methylbenzylamine), an antiarrhythmic drug that inhibits Ca2+/calmodulin-dependent serine-threonine protein kinase II (CaMKII), drastically reduced the presence of DADs in CVPT-CMs, rescuing the arrhythmic phenotype induced by catecholaminergic stress. In addition, intracellular calcium transient measurements on 3D beating clusters by fast resolution optical mapping showed that CPVT clusters developed multiple calcium transients, whereas in the wild-type clusters, only single initiations were detected. Such instability is aggravated in the presence of isoproterenol and is attenuated by KN-93. As seen in our RyR2 knock-in CPVT mice, the antiarrhythmic effect of KN-93 is confirmed in these human iPSC-derived cardiac cells, supporting the role of this in vitro system for drug screening and optimization of clinical treatment strategies.

Published
2013

29. Human Induced Pluripotent Stem Cells as an innovative tool to optimize treatment strategies for Catecholaminergic Polimorphic Ventricular Tachicardia

Author

Lodola, F, Di Pasquale, E, Curcio, A, Miragoli, M, Avelino-Cruz, J, Denegri, M, Novelli, V, Bongianino, R, Buonocore, M, Portararo, P, Bloise, R, Napolitano, C, Condorelli, G, Priori, S, Lodola F, Di Pasquale E, Curcio A, Miragoli M, Avelino-Cruz JE, Denegri M, Novelli V, Bongianino R, Buonocore M, Portararo P, Bloise R, Napolitano C, Condorelli G, Priori SG, Lodola, F, Di Pasquale, E, Curcio, A, Miragoli, M, Avelino-Cruz, J, Denegri, M, Novelli, V, Bongianino, R, Buonocore, M, Portararo, P, Bloise, R, Napolitano, C, Condorelli, G, Priori, S, Lodola F, Di Pasquale E, Curcio A, Miragoli M, Avelino-Cruz JE, Denegri M, Novelli V, Bongianino R, Buonocore M, Portararo P, Bloise R, Napolitano C, Condorelli G, and Priori SG

Published
2013

31. Human induced pluripotent stem cells as an innovative tool to optimize treatment strategies for Catecholaminergic Polymorphic Ventricular Tachycardia

Author

Lodola, F, Di Pasquale, E, Avelino-Cruz, J, Denegri, M, Leccioli, V, Bongianino, R, Buonocore, M, Bloise, R, Condorelli, G, Napolitano, C, Priori, S, Lodola F, Di Pasquale E, Avelino-Cruz JE, Denegri M, Leccioli V, Bongianino R, Buonocore M, Bloise R, Condorelli G, Napolitano C, Priori SG, Lodola, F, Di Pasquale, E, Avelino-Cruz, J, Denegri, M, Leccioli, V, Bongianino, R, Buonocore, M, Bloise, R, Condorelli, G, Napolitano, C, Priori, S, Lodola F, Di Pasquale E, Avelino-Cruz JE, Denegri M, Leccioli V, Bongianino R, Buonocore M, Bloise R, Condorelli G, Napolitano C, and Priori SG

Published
2012

32. CaMKII inhibition in human induced pluripotent stem cells derived from Catecholaminergic polymorphic ventricular tachycardia patients

Author

Lodola, F, Di Pasquale, E, Novelli, V, Denegri, M, Avelino-Cruz, J, Curcio, A, Bongianino, R, Leccioli, V, Buonocore, M, Bloise, R, Condorelli, G, Napolitano, C, Priori, S, Lodola F, Di Pasquale E, Novelli V, Denegri M, Avelino-Cruz JE, Curcio A, Bongianino R, Leccioli V, Buonocore M, Bloise R, Condorelli G, Napolitano C, Priori SG, Lodola, F, Di Pasquale, E, Novelli, V, Denegri, M, Avelino-Cruz, J, Curcio, A, Bongianino, R, Leccioli, V, Buonocore, M, Bloise, R, Condorelli, G, Napolitano, C, Priori, S, Lodola F, Di Pasquale E, Novelli V, Denegri M, Avelino-Cruz JE, Curcio A, Bongianino R, Leccioli V, Buonocore M, Bloise R, Condorelli G, Napolitano C, and Priori SG

Published
2012

33. CaMKII inhibition in human induced pluripotent stem cells derived from Catecholaminergic polymorphic ventricular tachycardia patients

Author

Lodola F, Di Pasquale E, Novelli V, Denegri M, Avelino-Cruz JE, Curcio A, Bongianino R, Leccioli V, Buonocore M, Bloise R, Condorelli G, Napolitano C, Priori SG, Lodola, F, Di Pasquale, E, Novelli, V, Denegri, M, Avelino-Cruz, J, Curcio, A, Bongianino, R, Leccioli, V, Buonocore, M, Bloise, R, Condorelli, G, Napolitano, C, and Priori, S

Subjects
CaMKII, human induced pluripotent stem cells cardiomyocytes, Catecholaminergic polymorphic ventricular tachycardia
Published
2012

34. Gene-specific therapy with mexiletine reduces arrhythmic events in patients with long QT syndrome type 3

Author

Mazzanti, A, Maragna, R, Faragli, A, Monteforte, N, Bloise, R, Memmi, M, Novelli, V, Baiardi, P, Bagnardi, V, Etheridge, S, Napolitano, C, Priori, S, Priori, S., BAGNARDI, VINCENZO, Mazzanti, A, Maragna, R, Faragli, A, Monteforte, N, Bloise, R, Memmi, M, Novelli, V, Baiardi, P, Bagnardi, V, Etheridge, S, Napolitano, C, Priori, S, Priori, S., and BAGNARDI, VINCENZO

Abstract

Background Long QT syndrome type 3 (LQT3) is a lethal disease caused by gain-of-function mutations in the SCN5A gene, coding for the alpha-subunit of the sodium channel NaV1.5. Mexiletine is used to block late sodium current and to shorten QT interval in LQT3 patients. Objectives The aim of this study was to determine whether mexiletine prevents arrhythmic events (arrhythmic syncope, aborted cardiac arrest, or sudden cardiac death) in LQT3 patients. 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 the annual rate of arrhythmic events during observation periods of equal duration before and after the beginning of therapy with mexiletine. Results The study population comprised 34 LQT3 patients, 19 (56%) of whom were male. The median age at beginning of treatment with mexiletine was 22 years, and median QTc interval before therapy 509 ms. The median duration of oral mexiletine therapy was 36 months, at an average daily dose of 8 ± 0.5 mg/kg. Mexiletine significantly shortened QTc (by 63 ± 6 ms; p < 0.0001) and reduced the percentage of patients with arrhythmic events (from 22% to 3%; p = 0.031), the mean number of arrhythmic events per patient (from 0.43 ± 0.17 to 0.03 ± 0.03; p = 0.027), and the annual rate of arrhythmic events (from 10.3% to 0.7%; p = 0.0097). Conclusions Besides shortening QTc interval, mexiletine caused a major reduction of life-threatening arrhythmic events in LQT3 patients, thus representing an efficacious therapeutic strategy.

Published
2016

36. Clinical and genetic heterogeneity of right bundle branch block and ST-segment elevation syndrome: A prospective evaluation of 52 families

Author

Priori, S, Napolitano, C, Gasparini, M, Pappone, C, Della Bella, P, Brignole, M, Giordano, U, Giovannini, T, Menozzi, C, Bloise, R, Crotti, L, Terreni, L, Schwartz, P, Priori SG, Napolitano C, Gasparini M, Pappone C, Della Bella P, Brignole M, Giordano U, Giovannini T, Menozzi C, Bloise R, Crotti L, Terreni L, Schwartz PJ, Priori, S, Napolitano, C, Gasparini, M, Pappone, C, Della Bella, P, Brignole, M, Giordano, U, Giovannini, T, Menozzi, C, Bloise, R, Crotti, L, Terreni, L, Schwartz, P, Priori SG, Napolitano C, Gasparini M, Pappone C, Della Bella P, Brignole M, Giordano U, Giovannini T, Menozzi C, Bloise R, Crotti L, Terreni L, and Schwartz PJ

Abstract

Background - The ECG pattern of right bundle branch block and ST-segment elevation in leads V1 to V3 (Brugada syndrome) is associated with high risk of sudden death in patients with a normal heart. Current management and prognosis are based on a single study suggesting a high mortality risk within 3 years for symptomatic and asymptomatic patients alike. As a consequence, aggressive management (implantable cardioverter defibrillator) is recommended for both groups. Methods and Results - Sixty patients (45 males aged 40±15 years) with the typical ECG pattern were clinically evaluated. Events at follow-up were analyzed for patients with at least one episode of aborted sudden death or syncope of unknown origin before recognition of the syndrome (30 symptomatic patients) and for patients without previous history of events (30 asymptomatic patients). Prevalence of mutations of the cardiac sodium channel was 15%, demonstrating genetic heterogeneity. During a mean follow-up of 33±38 months, ventricular fibrillation occurred in 5 (16%) of 30 symptomatic patients and in none of the 30 asymptomatic patients. Programmed electrical stimulation was of limited value in identifying patients at risk (positive predictive value 50%, negative predictive value 46%). Pharmacological challenge with sodium channel blockers was unable to unmask most silent gene carriers (positive predictive value 35%). Conclusions - At variance with current views, asymptomatic patients are at lower risk for sudden death. Programmed electrical stimulation identifies only a fraction of individuals at risk, and sodium channel blockade fails to unmask most silent gene carriers. This novel evidence mandates a reappraisal of therapeutic management

Published
2000

40. ProvinciaSense

Author

Arzilli, T., primary, Ficarola, F., additional, Massri, K., additional, Vitaletti, A., additional, Loriga, Francesco, additional, De Marinis, I., additional, Ferraresi, A., additional, Bloise, R., additional, and Goretti, M., additional

Published
2013
Full Text
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42. Cathecolaminergic polymorphic ventricular tachycardia: successful emergency treatment with intravenous propranolol

Author

De Rosa, Gabriella, Delogu, Angelica Bibiana, Piastra, Marco, Chiaretti, Antonio, Bloise, R., Priori, S. G., De Rosa, Gabriella (ORCID:0000-0002-8780-5105), Delogu, Angelica Bibiana (ORCID:0000-0002-2283-3180), Piastra, Marco (ORCID:0000-0002-3144-8970), Chiaretti, A (ORCID:0000-0002-9971-1640), De Rosa, Gabriella, Delogu, Angelica Bibiana, Piastra, Marco, Chiaretti, Antonio, Bloise, R., Priori, S. G., De Rosa, Gabriella (ORCID:0000-0002-8780-5105), Delogu, Angelica Bibiana (ORCID:0000-0002-2283-3180), Piastra, Marco (ORCID:0000-0002-3144-8970), and Chiaretti, A (ORCID:0000-0002-9971-1640)

Abstract

Catecholaminergic polymorphic ventricular tachycardia (VT) is a rare arrhythmogenic disorder, which may cause sudden death and whose relationships with mutations in cardiac ryanodine receptor gene have been recently established. The present article reports a catecholaminergic polymorphic VT case of a 9-year-old girl, without any previous history of syncope, who has been found unconscious while playing and referred comatose to pediatric intensive care unit. The electrocardiogram pattern showed runs of bidirectional and polymorphic VT degenerating into ventricular fibrillation, without QT interval abnormalities. Various attempts of cardioversion, lidocaine, and magnesium sulfate intravenous infusions were only partially effective. Owing to catecholaminergic polymorphic VT highly suggesting electrocardiogram pattern, intravenous propranolol was administered, achieving immediate VT interruption. Long-term nadolol therapy effectively prevented further arrhythmias, with no relapses up to 10 months later; a good neurologic recovery was also obtained. Genetic evaluation revealed in this patient-but not in relatives-a mutation in ryanodine receptor gene on chromosome 1.

Published
2004

43. Poster Session 4

Author

Tada, H., primary, Yamasaki, H., additional, Sekiguchi, Y., additional, Igarashi, M., additional, Kuroki, K., additional, Machino, T., additional, Yoshida, K., additional, Aonuma, K., additional, Heinzel, F. R., additional, Forstner, H., additional, Lercher, P., additional, Bisping, E., additional, Rotman, B., additional, Fruhwald, F. M., additional, Pieske, B. M., additional, Dabrowski, R., additional, Kowalik, I., additional, Borowiec, A., additional, Smolis-Bak, E., additional, Trybuch, A., additional, Sosnowski, C., additional, Szwed, H., additional, Baturova, M. A., additional, Lindgren, A., additional, Shubik, Y. V., additional, Olsson, B., additional, Platonov, P. G., additional, Van Den Broek, K. C., additional, Denollet, J., additional, Widdershoven, J., additional, Kupper, N., additional, Allam, R., additional, Allam, R. A. G. A. B., additional, Galal, W. A. G. D. Y., additional, El-Damnhoury, H. A. Y. A. M., additional, Mortada, A. Y. M. A. N., additional, Jimenez-Candil, J., additional, Martin, A., additional, Hernandez, J., additional, Martin, F., additional, Gallego, M., additional, Martin-Luengo, C., additional, Quintanilla, J. G., additional, Moreno Planas, J., additional, Molina-Morua, R., additional, Archondo, T., additional, Garcia-Torrent, M. J., additional, Perez-Castellano, N., additional, Macaya, C., additional, Perez-Villacastin, J., additional, Saiz, J., additional, Tobon, C., additional, Rodriguez, J. F., additional, Hornero, F., additional, Ferrero, J. M., additional, Ito, K., additional, Date, T., additional, Kawai, M., additional, Hioki, M., additional, Narui, R., additional, Matsuo, S., additional, Yoshimura, M., additional, Yamane, T., additional, Tabatabaei, N., additional, Lin, G., additional, Powell, B. D., additional, Smairat, R., additional, Glockner, J. F., additional, Brady, P. A., additional, Fichtner, S., additional, Czudnochowsky, U., additional, Estner, H., additional, Reents, T., additional, Jilek, C., additional, Ammar, S., additional, Hessling, G., additional, Deisenhofer, I., additional, Shah, D. C., additional, Kautzner, J., additional, Saoudi, N., additional, Herrera, C., additional, Jais, P., additional, Hindricks, G., additional, Neuzil, P., additional, Kuck, K. H., additional, Wong, K. C. K., additional, Jones, M., additional, Qureshi, N., additional, Muthumala, A., additional, Betts, T. R., additional, Bashir, Y., additional, Rajappan, K., additional, Vogtmann, T., additional, Wagner, M., additional, Schurig, J., additional, Hein, P., additional, Hamm, B., additional, Baumann, G., additional, Lembcke, A., additional, Saad, B., additional, Slater, C., additional, Oliveira, L. A., additional, Elias, R., additional, Camiletti, A., additional, Moura, D., additional, Maldonado, P., additional, Camanho, L. E., additional, Bulava, A., additional, Hanis, J., additional, Sitek, D., additional, Novotny, A., additional, Chik, W. B., additional, Lim, T. W., additional, Choon, H. K., additional, See, V. A., additional, Mccall, R., additional, Thomas, L., additional, Ross, D. L., additional, Thomas, S. P., additional, Chen, J., additional, De Bortoli, A., additional, Rossvoll, O., additional, Hoff, P. I., additional, Solheim, E., additional, Sun, L. Z., additional, Schuster, P., additional, Ohm, O. J., additional, Ardashev, A. V., additional, Zhelyakov, E., additional, Rybachenko, M. S., additional, Konev, A. V., additional, Belenkov, Y. U. N., additional, Gunawardene, M., additional, Chun, K. R. J., additional, Schulte-Hahn, B., additional, Windhorst, V., additional, Kulikoglu, M., additional, Nowak, B., additional, Schmidt, B., additional, Albina, G. A., additional, Rivera, R. S., additional, Scazzuso, F., additional, Laino, R. L., additional, Giniger, G. A., additional, Arbelo, E., additional, Calvo, N., additional, Tamborero, D., additional, Andreu, D., additional, Borras, R., additional, Berruezo, A., additional, Brugada, J., additional, Mont, L., additional, Stefan, L., additional, Eisenberger, M., additional, Celentano, E., additional, Peytchev, P., additional, Bodea, O., additional, Geelen, P., additional, De Potter, T., additional, Oliveira, M. M., additional, Silva, N., additional, Cunha, P. S., additional, Feliciano, J., additional, Lousinha, A., additional, Toste, A., additional, Santos, S., additional, Ferreira, R. C., additional, Matsuda, H., additional, Harada, T., additional, Soejima, K., additional, Ishikawa, Y., additional, Mizukoshi, K., additional, Sasaki, T., additional, Mizuno, K., additional, Miyake, F., additional, Adragao, P. P., additional, Cavaco, D., additional, Miranda, R., additional, Santos, M., additional, Morgado, F., additional, Reis Santos, K., additional, Candeias, R., additional, Marcelino, S., additional, Zoppo, F., additional, Grandolino, G., additional, Zerbo, F., additional, Bertaglia, E., additional, Schlueter, S. M., additional, Grebe, O., additional, Vester, E. G., additional, Miracle Blanco, A. L., additional, Arenal Maiz, A., additional, Atienza Fernandez, F., additional, Datino Romaniega, T., additional, Gonzalez Torrecilla, E., additional, Eidelman, G., additional, Hernandez Hernandez, J., additional, Fernandez Aviles, F., additional, Fukumoto, K., additional, Takatsuki, S., additional, Kimura, T., additional, Nishiyama, N., additional, Aizawa, Y., additional, Sato, T., additional, Miyoshi, S., additional, Fukuda, K., additional, Richter, B., additional, Gwechenberger, M., additional, Socas, A., additional, Zorn, G., additional, Albinni, S., additional, Marx, M., additional, Wojta, J., additional, Goessinger, H., additional, Deneke, T., additional, Balta, O., additional, Paesler, M., additional, Buenz, K., additional, Anders, H., additional, Horlitz, M., additional, Muegge, A., additional, Shin, D.- I., additional, Natsuyama, K., additional, Yamaguchi, K. M., additional, Nishida, Y. N., additional, Kosiuk, J., additional, Bode, K., additional, Arya, A., additional, Piorkowski, C., additional, Gaspar, T., additional, Sommer, P., additional, Bollmann, A., additional, Wichterle, D., additional, Peichl, P., additional, Simek, J., additional, Havranek, S., additional, Bulkova, V., additional, Cihak, R., additional, Jurado Roman, A., additional, Salguero Bodes, R., additional, Lopez Gil, M., additional, Fontenla Cerezuela, A., additional, De Riva Silva, M., additional, Arribas Ynsaurriaga, F., additional, Fernandez Herranz, A. I., additional, De Dios Perez, S., additional, Revishvili, A. S., additional, Dishekov, M., additional, Tembotova, Z., additional, Barsamyan, S., additional, Vaccari, D., additional, Alvarenga, C., additional, Jesus, I., additional, Layher, J., additional, Takahashi, A., additional, Singh, N., additional, Siot, P., additional, Elkaim, J. P., additional, Savelieva, I., additional, Mcclelland, L., additional, Lovegrove, A., additional, Jones, S., additional, Camm, J., additional, Folino, A. F., additional, Breda, R., additional, Calzavara, P., additional, Comisso, J., additional, Borghetti, F., additional, Iliceto, S., additional, Buja, G., additional, Mlynarski, R., additional, Mlynarska, A., additional, Sosnowski, M., additional, Wilczek, J., additional, Mabo, P., additional, Carrault, G., additional, Bordachar, P., additional, Makdissi, A., additional, Duchemin, L., additional, Alonso, C., additional, Neri, G., additional, Masaro, G., additional, Vittadello, S., additional, Gardin, A., additional, Barbetta, A., additional, Di Gregorio, F., additional, Sciaraffia, E., additional, Ginks, M. R., additional, Gustafsson, J. S., additional, Hollmark, M. C., additional, Rinaldi, C. A., additional, Blomstrom Lundqvist, C., additional, Brusich, S., additional, Tomasic, D., additional, Ferek-Petric, B., additional, Mavric, Z., additional, Kutarski, A., additional, Malecka, B., additional, Kolodzinska, A., additional, Grabowski, M., additional, Dovellini, E. V., additional, Giurlani, L., additional, Cerisano, G., additional, Carrabba, N., additional, Valenti, R., additional, Antoniucci, D., additional, Opolski, G., additional, Tomassoni, G., additional, Baker, J., additional, Corbisiero, R., additional, Martin, D., additional, Niazi, I., additional, Sheppard, R., additional, Sperzel, J., additional, Gutleben, K., additional, Petru, J., additional, Sediva, L., additional, Skoda, J., additional, Mazzone, P., additional, Ciconte, G., additional, Vergara, P., additional, Marzi, A., additional, Paglino, G., additional, Sora, N., additional, Gulletta, S., additional, Della Bella, P., additional, Pietura, R., additional, Czajkowski, M., additional, Cabanelas, N., additional, Martins, V. P., additional, Alves, M., additional, Valente, F. X., additional, Marta, L., additional, Francisco, A., additional, Silva, R., additional, Ferreira Da Silva, G., additional, Huo, Y., additional, Holmqvist, F., additional, Carlson, J., additional, Wetzel, U., additional, Platonov, P., additional, Nof, E., additional, Abu Shama, R., additional, Kuperstein, R., additional, Feinberg, M. S., additional, Eldar, M., additional, Glikson, M., additional, Luria, D., additional, Kubus, P., additional, Materna, O., additional, Gebauer, R. A., additional, Matejka, T., additional, Gebauer, R., additional, Tlaskal, T., additional, Janousek, J., additional, Muessigbrodt, A., additional, Richter, S., additional, Stockburger, M., additional, Boveda, S., additional, Defaye, P., additional, Stancak Branislav, P., additional, Kaliska, G., additional, Rolando, M., additional, Moreno, J., additional, Ohlow, M.- A. G., additional, Lauer, B., additional, Buchter, B., additional, Schreiber, M., additional, Geller, J. C., additional, Val-Mejias, J. E., additional, Ouali, S., additional, Azzez, S., additional, Kacem, S., additional, Ben Salem, H., additional, Hammas, S., additional, Neffeti, E., additional, Remedi, F., additional, Boughzela, E., additional, Miyazaki, H., additional, Miyanaga, S., additional, Shibayama, K., additional, Tokuda, M., additional, Kudo, T., additional, Coppola, B., additional, Shehada, R. E. N., additional, Costandi, P., additional, Healey, J., additional, Hohnloser, S. H., additional, Gold, M. R., additional, Capucci, A., additional, Van Gelder, I. C., additional, Carlson, M., additional, Lau, C. P., additional, Connolly, S. J., additional, Bogaard, M. D., additional, Leenders, G. E., additional, Maskara, B., additional, Tuinenburg, A. E., additional, Loh, P., additional, Hauer, R. N., additional, Doevendans, P. A., additional, Meine, M., additional, Thibault, B., additional, Dubuc, M., additional, Karst, E., additional, Ryu, K., additional, Paiement, P., additional, Farazi, T., additional, Puetz, V., additional, Berndt, C., additional, Buchholz, J., additional, Dorszewski, A., additional, Mornos, C., additional, Cozma, D., additional, Ionac, A., additional, Petrescu, L., additional, Mornos, A., additional, Pescariu, S., additional, Benser, M., additional, Roscoe, G., additional, De Jong, S., additional, Roberts, G., additional, Boileau, P., additional, Rec, A., additional, Folman, C., additional, Morttada, A., additional, Abd El Kader, M., additional, Samir, R., additional, Roushdy, R., additional, Khaled, S., additional, Abo El Maaty, M., additional, Van Gelder, B., additional, Houthuizen, P., additional, Bracke, F. A., additional, Osca Asensi, J., additional, Tejada, D., additional, Sanchez, J. M., additional, Munoz, B., additional, Cano, O., additional, Rodriguez, M., additional, Sancho-Tello, M. J., additional, Olague, J., additional, Hou, W., additional, Rosenberg, S., additional, Koh, S., additional, Poore, J., additional, Snell, J., additional, Yang, M., additional, Nirav, D., additional, Bornzin, G., additional, Deering, T., additional, Dan, D., additional, Wickliffe, A. C., additional, Cazeau, S., additional, Karimzadeh, K., additional, Mukerji, S., additional, Loghin, C., additional, Kantharia, B., additional, Jones, M. A., additional, Lamba, J., additional, Simpson, C. S., additional, Redfearn, D. P., additional, Michael, K. A., additional, Fitzpatrick, M., additional, Baranchuk, A., additional, Heinke, M., additional, Ismer, B., additional, Kuehnert, H., additional, Surber, R., additional, Haltenberger, A. M., additional, Prochnau, D., additional, Figulla, H. R., additional, Delarche, N., additional, Bizeau, O., additional, Couderc, P., additional, Chapelet, A., additional, Amara, W., additional, Lazarus, A., additional, Krupickova, S., additional, Van Deursen, C. J. M., additional, Strik, M., additional, Vernooy, K., additional, Van Hunnik, A., additional, Kuiper, M., additional, Crijns, H. J. G. M., additional, Prinzen, F. W., additional, Islam, N., additional, Gras, D., additional, Abraham, W., additional, Calo, L., additional, Birgersdotter-Green, U., additional, Clyne, C., additional, Herre, J., additional, Klein, N., additional, Kowalski, O., additional, Lenarczyk, R., additional, Pruszkowska, P., additional, Sokal, A., additional, Kukulski, T., additional, Zielinska, T., additional, Pluta, S., additional, Kalarus, Z., additional, Schwab, J. O., additional, Gasparini, M., additional, Anselme, F., additional, Clementy, J., additional, Santini, M., additional, Martinez Ferrer, J., additional, Burrone, V., additional, Santi, E., additional, Nevzorov, R., additional, Porter, A., additional, Kusniec, J., additional, Golovchiner, G., additional, Ben-Gal, T., additional, Strasberg, B., additional, Haim, M., additional, Rordorf, R., additional, Savastano, S., additional, Sanzo, A., additional, Vicentini, A., additional, Petracci, B., additional, De Amici, M., additional, Striuli, L., additional, Landolina, M., additional, Tolosana, J. M., additional, Martin, A. M., additional, Hernandez-Madrid, A., additional, Macias, A., additional, Fernandez-Lozano, I., additional, Osca, J., additional, Quesada, A., additional, Tada, H., additional, Noguchi, Y., additional, Shahrzad, S., additional, Karim Soleiman, N., additional, Tavoosi, A., additional, Taban, S., additional, Emkanjoo, Z., additional, Fukunaga, M., additional, Goya, M., additional, Hiroshima, K., additional, Ohe, M., additional, Hayashi, K., additional, Iwabuchi, M., additional, Nosaka, H., additional, Nobuyoshi, M., additional, Doiny, D., additional, Perez-Silva, A., additional, Castrejon Castrejon, S., additional, Estrada, A., additional, Ortega, M., additional, Lopez-Sendon, J. L., additional, Merino, J. L., additional, Garcia Fernandez, F. J., additional, Gallardo, R., additional, Pachon, M., additional, Almendral, J., additional, Martin, J., additional, Yahya, D., additional, Al-Mogheer, B., additional, Gouda, S., additional, Eweis, E., additional, El Ramly, M., additional, Abdelwahab, A., additional, Kassenberg, W., additional, Wittkampf, F. H. M., additional, Hof, I. E., additional, Heijden, J. H., additional, Neven, K. G. E. J., additional, Hauer, R. N. W., additional, Baratto, F., additional, Bignami, E., additional, Pappalardo, F., additional, Maccabelli, G., additional, Nicolotti, D., additional, Zangrillo, A., additional, Nagashima, M., additional, An, Y., additional, Okreglicki, A., additional, Russouw, C., additional, Tilz, R., additional, Yoshiga, Y., additional, Mathew, S., additional, Fuernkranz, A., additional, Rillig, A., additional, Wissner, E., additional, Ouyang, F., additional, De Sisti, A., additional, Tonet, J., additional, Gueffaf, F., additional, Touil, F., additional, Aouate, P., additional, Hidden-Lucet, F., additional, Makimoto, H., additional, Satomi, K., additional, Yamada, Y., additional, Okamura, H., additional, Noda, T., additional, Shimizu, W., additional, Aihara, N., additional, Kamakura, S., additional, Perez Silva, A., additional, Castrejon, S., additional, Gonzalez Vasserot, M., additional, Senges, J., additional, Brachmann, J., additional, Andresen, D., additional, Hoffmann, E., additional, Schumacher, B., additional, Willems, S., additional, Springer, B., additional, Kolb, C., additional, Akca, F., additional, Bauernfeind, T., additional, De Groot, N. M. S., additional, Schwagten, B., additional, Witsenburg, M., additional, Jordaens, L., additional, Szili-Torok, T., additional, Hata, Y., additional, Nakagami, R., additional, Watanabe, T., additional, Sato, A., additional, Watanabe, H., additional, Kabutoya, T., additional, Mituhashi, T., additional, Theuns, D. A. M. J., additional, Smith, T., additional, Pedersen, S. S., additional, Dabiri-Abkenari, L., additional, Prull, M. W., additional, Unverricht, S., additional, Bittlinsky, A., additional, Wirdemann, H., additional, Sasko, B., additional, Wirdeier, S., additional, Trappe, H. J., additional, Zorio Grima, E., additional, Rueda, J., additional, Medina, P., additional, Jaijo, T., additional, Sevilla, T., additional, Arnau, M. A., additional, Salvador, A., additional, Starrenburg, A. H., additional, Kraaier, K., additional, Scholten, M. F., additional, Van Der Palen, J., additional, De Haan, S., additional, Commandeur, J., additional, De Boer, K., additional, Beek, A. M., additional, Van Rossum, A. C., additional, Allaart, C. P., additional, Berne, P., additional, Porres, J. M., additional, Arnaiz, J. A., additional, Brugada, R., additional, Man, S., additional, Maan, A. C., additional, Thijssen, J., additional, Van Der Wall, E. E., additional, Schalij, M. J., additional, Burattini, L., additional, Burattini, R., additional, Swenne, C. A., additional, Bonny, A., additional, Ditah, I., additional, Larrazet, F., additional, Frank, R., additional, Fontaine, G., additional, Van Der Voort, P. H., additional, Alings, M., additional, Shimane, A., additional, Okajima, K., additional, Kanda, G., additional, Yokoi, K., additional, Yamada, S., additional, Taniguchi, Y., additional, Hayashi, T., additional, Kajiya, T., additional, Santos, M. C., additional, Wright, J., additional, Betts, J., additional, Denman, R., additional, Dominguez-Perez, L., additional, Arias Palomares, M. 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Published
2011
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47. The Long QT Syndrome

Author

Priori, S, Bloise, R, Crotti, L, Priori, SG, Priori, S, Bloise, R, Crotti, L, and Priori, SG

Published
2001

48. The elusive link between LQT3 and Brugada syndrome: the role of flecainide challenge

Author

Priori, S, Napolitano, C, Schwartz, P, Bloise, R, Crotti, L, Ronchetti, E, Priori, SG, Schwartz, PJ, Priori, S, Napolitano, C, Schwartz, P, Bloise, R, Crotti, L, Ronchetti, E, Priori, SG, and Schwartz, PJ

Abstract

Background - 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 typical manifestations of long-QT syndrome (QT interval prolongation) and BS (ST segment elevation in leads V1 through V3) may coexist in the same patients, which raises questions about the actual differences between LQT3 and BS. Intravenous flecainide is the standard provocative test used to unmask BS in individuals with concealed forms of the disease, and oral flecainide has been proposed as a treatment option for LQT3 patients because it may shorten their QT interval. Methods and Results - We tested the possibility that in some LQT3 patients, flecainide might not only shorten the QT interval, but also produce an elevation of the ST segment. A total of 13 patients from 7 LQT3 families received intravenous flecainide using the protocol used for BS. As expected, QT, QTc, JT, and JTc interval shortening was observed in 12 of the 13 patients, and concomitant ST segment elevation in leads V1 through V3 (≥2 mm) was observed in 6 of the 13. Conclusions - The data demonstrate that flecainide may induce ST segment elevation in LQT3 patients, raising concerns about the safety of flecainide therapy and demonstrating the existence of an intriguing overlap between LQT3 and BS

Published
2000

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