The Experts below are selected from a list of 270 Experts worldwide ranked by ideXlab platform
Matteo E. Mangoni - One of the best experts on this subject based on the ideXlab platform.
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Paradoxical Effect of Increased Diastolic Ca 2+ Release and Decreased Sinoatrial Node Activity in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia
Circulation, 2012Co-Authors: Patricia Neco, Angelo Torrente, Pietro Mesirca, Esther Zorio, Nian Liu, Silvia Priori, Carlo Napolitano, Sylvain Richard, Jean-pierre Benitah, Matteo E. MangoniAbstract:Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with Catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored.
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paradoxical effect of increased diastolic ca2 release and decreased sinoatrial node activity in a mouse model of Catecholaminergic polymorphic ventricular tachycardia
Circulation, 2012Co-Authors: Patricia Neco, Pietro Mesirca, Esther Zorio, Nian Liu, Silvia Priori, Carlo Napolitano, Sylvain Richard, Jean-pierre Benitah, Angelo G Torrente, Matteo E. MangoniAbstract:Background—Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with Catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored. Methods and Results—We investigated SAN [Ca2+]i handling in mice carrying the Catecholaminergic polymorphic ventricular tachycardia–linked mutation of ryanodine receptor (RyR2R4496C) and their wild-type (WT) littermates. In vivo telemetric recordings showed impaired SAN automaticity in RyR2R4496C mice after isoproterenol injection, analogous to what was observed in Catecholaminergic polymorphic ventricular tachycardia patients after exercise. Pacemaker activity was explored by measuring spontaneous [Ca2+]i transients in SAN cells within the intact SAN by confocal microscopy. RyR2R4496C SAN presented significantly slower pacemaker activity and impaired chronotropic response under β-adrenergic stimulation, accompanied by the appe...
Andrew D Krahn - One of the best experts on this subject based on the ideXlab platform.
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Treatment of asymptomatic Catecholaminergic polymorphic ventricular tachycardia.
Future cardiology, 2012Co-Authors: Manoj N. Obeyesekere, Peter Leong-sit, Lorne J. Gula, Raymond Yee, Allan C. Skanes, George Klein, Andrew D KrahnAbstract:Catecholaminergic polymorphic ventricular tachycardia is a rare genetic disorder caused by mutations in genes involved in the intracellular calcium homeostasis of cardiac cells. Affected patients typically present with life-threatening ventricular arrhythmias precipitated by emotional/physical stress. The diagnosis is based on the demonstration of polymorphic or bidirectional ventricular tachycardia associated with adrenergic stress. Genetic testing can be confirmatory in some patients. Treatment for Catecholaminergic polymorphic ventricular tachycardia includes medical and surgical efforts to suppress the effects of epinephrine at the myocardial level and/or modulation of calcium homeostasis. Mortality is high when untreated and sudden cardiac death may be the first manifestation of the disease. First-degree relatives of a proband should be offered genetic testing if the causal mutation is known. If the family mutation is not known, relatives should be clinically evaluated with provocative testing. In the absence of rigorous trials, prophylactic treatment of the asymptomatic Catecholaminergic polymorphic ventricular tachycardia patient appears to reduce morbidity and mortality.
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sudden cardiac death despite an implantable cardioverter defibrillator in a young female with Catecholaminergic ventricular tachycardia
Heart Rhythm, 2006Co-Authors: Uwais Mohamed, Michael H Gollob, Robert M Gow, Andrew D KrahnAbstract:d c r s a i d a r h v o ntroduction ardiac arrest due to primary electrical disease, such as long T syndrome, Brugada syndrome, or Catecholaminergic olymorphic ventricular tachycardia, typically is managed ith an implantable cardioverter-defibrillator (ICD). Afer ICD placement, patients and physicians consider the ikelihood of sudden death to be remote. We report a fatal utcome in a young female with an ICD, clinical catcholaminergic polymorphic ventricular tachycardia, and a isease-causing cardiac ryanodine receptor (RyR2) gene utation.
Silvia Priori - One of the best experts on this subject based on the ideXlab platform.
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Paradoxical Effect of Increased Diastolic Ca 2+ Release and Decreased Sinoatrial Node Activity in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia
Circulation, 2012Co-Authors: Patricia Neco, Angelo Torrente, Pietro Mesirca, Esther Zorio, Nian Liu, Silvia Priori, Carlo Napolitano, Sylvain Richard, Jean-pierre Benitah, Matteo E. MangoniAbstract:Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with Catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored.
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paradoxical effect of increased diastolic ca2 release and decreased sinoatrial node activity in a mouse model of Catecholaminergic polymorphic ventricular tachycardia
Circulation, 2012Co-Authors: Patricia Neco, Pietro Mesirca, Esther Zorio, Nian Liu, Silvia Priori, Carlo Napolitano, Sylvain Richard, Jean-pierre Benitah, Angelo G Torrente, Matteo E. MangoniAbstract:Background—Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with Catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored. Methods and Results—We investigated SAN [Ca2+]i handling in mice carrying the Catecholaminergic polymorphic ventricular tachycardia–linked mutation of ryanodine receptor (RyR2R4496C) and their wild-type (WT) littermates. In vivo telemetric recordings showed impaired SAN automaticity in RyR2R4496C mice after isoproterenol injection, analogous to what was observed in Catecholaminergic polymorphic ventricular tachycardia patients after exercise. Pacemaker activity was explored by measuring spontaneous [Ca2+]i transients in SAN cells within the intact SAN by confocal microscopy. RyR2R4496C SAN presented significantly slower pacemaker activity and impaired chronotropic response under β-adrenergic stimulation, accompanied by the appe...
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diagnosis and treatment of Catecholaminergic polymorphic ventricular tachycardia
Heart Rhythm, 2007Co-Authors: Carlo Napolitano, Silvia PrioriAbstract:e v t Catecholaminergic polymorphic ventricular tachycardia VT) was first described by Reid et al in 1975 and by oumel et al in 1978. The condition was described as a amilial cardiac arrhythmia that occurs in patients with tructurally normal heart and causes exercise-/emotion-trigered syncope and sudden death with a distinctive pattern of entricular and supraventricular arrhythmias. Since the first yanodine receptor mutations were identified in 2001, it ppeared evident that Catecholaminergic polymorphic VT as caused by uncontrolled Ca release from the sarcolasmic reticulum. Subsequent experimental studies demnstrated that such abnormal calcium handling caused arhythmias mediated by delayed afterdepolarizations and riggered activity. This article reviews the current knowldge of the diagnosis and therapy for Catecholaminergic olymorphic VT and outlines the open issues to be adressed in order to reduce the burden of life-threatening ardiac events in patients with this lethal disorder.
Carlo Napolitano - One of the best experts on this subject based on the ideXlab platform.
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Paradoxical Effect of Increased Diastolic Ca 2+ Release and Decreased Sinoatrial Node Activity in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia
Circulation, 2012Co-Authors: Patricia Neco, Angelo Torrente, Pietro Mesirca, Esther Zorio, Nian Liu, Silvia Priori, Carlo Napolitano, Sylvain Richard, Jean-pierre Benitah, Matteo E. MangoniAbstract:Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with Catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored.
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paradoxical effect of increased diastolic ca2 release and decreased sinoatrial node activity in a mouse model of Catecholaminergic polymorphic ventricular tachycardia
Circulation, 2012Co-Authors: Patricia Neco, Pietro Mesirca, Esther Zorio, Nian Liu, Silvia Priori, Carlo Napolitano, Sylvain Richard, Jean-pierre Benitah, Angelo G Torrente, Matteo E. MangoniAbstract:Background—Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with Catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored. Methods and Results—We investigated SAN [Ca2+]i handling in mice carrying the Catecholaminergic polymorphic ventricular tachycardia–linked mutation of ryanodine receptor (RyR2R4496C) and their wild-type (WT) littermates. In vivo telemetric recordings showed impaired SAN automaticity in RyR2R4496C mice after isoproterenol injection, analogous to what was observed in Catecholaminergic polymorphic ventricular tachycardia patients after exercise. Pacemaker activity was explored by measuring spontaneous [Ca2+]i transients in SAN cells within the intact SAN by confocal microscopy. RyR2R4496C SAN presented significantly slower pacemaker activity and impaired chronotropic response under β-adrenergic stimulation, accompanied by the appe...
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diagnosis and treatment of Catecholaminergic polymorphic ventricular tachycardia
Heart Rhythm, 2007Co-Authors: Carlo Napolitano, Silvia PrioriAbstract:e v t Catecholaminergic polymorphic ventricular tachycardia VT) was first described by Reid et al in 1975 and by oumel et al in 1978. The condition was described as a amilial cardiac arrhythmia that occurs in patients with tructurally normal heart and causes exercise-/emotion-trigered syncope and sudden death with a distinctive pattern of entricular and supraventricular arrhythmias. Since the first yanodine receptor mutations were identified in 2001, it ppeared evident that Catecholaminergic polymorphic VT as caused by uncontrolled Ca release from the sarcolasmic reticulum. Subsequent experimental studies demnstrated that such abnormal calcium handling caused arhythmias mediated by delayed afterdepolarizations and riggered activity. This article reviews the current knowldge of the diagnosis and therapy for Catecholaminergic olymorphic VT and outlines the open issues to be adressed in order to reduce the burden of life-threatening ardiac events in patients with this lethal disorder.
Patricia Neco - One of the best experts on this subject based on the ideXlab platform.
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Paradoxical Effect of Increased Diastolic Ca 2+ Release and Decreased Sinoatrial Node Activity in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia
Circulation, 2012Co-Authors: Patricia Neco, Angelo Torrente, Pietro Mesirca, Esther Zorio, Nian Liu, Silvia Priori, Carlo Napolitano, Sylvain Richard, Jean-pierre Benitah, Matteo E. MangoniAbstract:Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with Catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored.
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paradoxical effect of increased diastolic ca2 release and decreased sinoatrial node activity in a mouse model of Catecholaminergic polymorphic ventricular tachycardia
Circulation, 2012Co-Authors: Patricia Neco, Pietro Mesirca, Esther Zorio, Nian Liu, Silvia Priori, Carlo Napolitano, Sylvain Richard, Jean-pierre Benitah, Angelo G Torrente, Matteo E. MangoniAbstract:Background—Catecholaminergic polymorphic ventricular tachycardia is characterized by stress-triggered syncope and sudden death. Patients with Catecholaminergic polymorphic ventricular tachycardia manifest sinoatrial node (SAN) dysfunction, the mechanisms of which remain unexplored. Methods and Results—We investigated SAN [Ca2+]i handling in mice carrying the Catecholaminergic polymorphic ventricular tachycardia–linked mutation of ryanodine receptor (RyR2R4496C) and their wild-type (WT) littermates. In vivo telemetric recordings showed impaired SAN automaticity in RyR2R4496C mice after isoproterenol injection, analogous to what was observed in Catecholaminergic polymorphic ventricular tachycardia patients after exercise. Pacemaker activity was explored by measuring spontaneous [Ca2+]i transients in SAN cells within the intact SAN by confocal microscopy. RyR2R4496C SAN presented significantly slower pacemaker activity and impaired chronotropic response under β-adrenergic stimulation, accompanied by the appe...