Full Text Journal Articles by
Author Vassilios J Bezzerides

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Gene Therapy for Inherited Arrhythmias.

Vassilios J Bezzerides, Maksymilian Prondzynski, Lucie Carrier, William T Pu,

Inherited arrhythmias are disorders caused by one or more genetic mutations that increase the risk of arrhythmia, which result in life-long risk of sudden death. These mutations either primarily perturb electrophysiological homeostasis (e.g., long QT syndrome and catecholaminergic polymorphic ventricular tachycardia), cause structural disease that is closely associated with severe ... Read more >>

Cardiovasc. Res. (Cardiovascular research)
[2020, :]

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Clinical and Genetic Findings in Children Presenting With Ventricular Fibrillation as the First Manifestation of Cardiovascular Disease.

Robyn J Hylind, Stephanie F Chandler, Virginie Beausejour Ladouceur, Shelina M Jamal, Mark E Alexander, Douglas Y Mah, Emily A Phaneuf, Elizabeth S DeWitt, Vassilios J Bezzerides, Dominic J Abrams,

J Am Heart Assoc (Journal of the American Heart Association)
[2020, 9(10):e016322]

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CITED4 Protects Against Adverse Remodeling in Response to Physiological and Pathological Stress.

Carolin Lerchenmüller, Charles P Rabolli, Ashish Suresh Yeri, Robert Kitchen, Ane M Salvador, Laura X Liu, Olivia Ziegler, Kirsty M Danielson, Colin Platt, Ravi Shah, Federico Damilano, Piyusha Kundu, Eva Riechert, Hugo A Katus, Jeffrey E Saffitz, Hasmik Keshishian, Steve Carr, Vassilios J Bezzerides, Saumya Das, Anthony Rosenzweig,

Rationale: Cardiac CITED4 is induced by exercise and is sufficient to cause physiological hypertrophy and mitigate adverse ventricular remodeling after ischemic injury. However, the role of endogenous CITED4 in response to physiological or pathological stress is unknown. Objective: To investigate the role of CITED4 in murine models of exercise and ... Read more >>

Circ. Res. (Circulation research)
[2020, :]

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Value of provocative electrophysiology testing in the management of pediatric patients after congenital heart surgery.

Audrey Dionne, John N Kheir, Kimberlee Gauvreau, John K Triedman, Dominic J Abrams, Mark E Alexander, Elizabeth S DeWitt, Douglas Y Mah, Edward P Walsh, Vassilios J Bezzerides,

OBJECTIVE:To determine the impact of provocative electrophysiology testing in post-operative congenital heart disease (CHD) patients on the management of supraventricular tachycardia (SVT) and clinical outcomes. METHODS:This is a retrospective study including patients < 18 years of age with surgery for CHD who had post-operative SVT between 2006-2017. Post-operative outcomes were ... Read more >>

Pacing Clin Electrophysiol (Pacing and clinical electrophysiology : PACE)
[2020, :]

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AAV Gene Therapy Prevents and Reverses Heart Failure in a Murine Knockout Model of Barth Syndrome.

Suya Wang, Yifei Li, Yang Xu, Qing Ma, Zhiqiang Lin, Michael Schlame, Vassilios J Bezzerides, Douglas Strathdee, William T Pu,

RATIONALE:Barth syndrome is an X-linked cardiac and skeletal myopathy caused by mutation of the gene Tafazzin (TAZ). Currently, there is no targeted treatment for Barth syndrome. Lack of a proper genetic animal model that recapitulates the features of Barth syndrome has hindered understanding of disease pathogenesis and therapeutic development. OBJECTIVE:We ... Read more >>

Circ. Res. (Circulation research)
[2020, 126(8):1024-1039]

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Low mortality in fetal supraventricular tachycardia: Outcomes in a 30-year single-institution experience.

Edward T O'Leary, Mark E Alexander, Vassilios J Bezzerides, Monika Drogosz, Katherine E Economy, Kevin G Friedman, Sarah S Pickard, Wayne Tworetzky, Douglas Y Mah,

OBJECTIVES:To describe a single institutional experience managing fetuses with supraventricular tachycardia (SVT) and to identify associations between patient characteristics and fetal and postnatal outcomes. BACKGROUND:Sustained fetal SVT is associated with significant morbidity and mortality if untreated, yet the optimal management strategy remains unclear. METHODS:Retrospective cohort study including fetuses diagnosed with ... Read more >>

J. Cardiovasc. Electrophysiol. (Journal of cardiovascular electrophysiology)
[2020, 31(5):1105-1113]

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Adverse event rate during inpatient sotalol initiation for the management of supraventricular and ventricular tachycardia in the pediatric and young adult population.

Stephanie F Chandler, Esther Chu, Robert D Whitehill, Laura M Bevilacqua, Vassilios J Bezzerides, Elizabeth S DeWitt, Mark E Alexander, Dominic J Abrams, John K Triedman, Edward P Walsh, Douglas Y Mah,

BACKGROUND:Sotalol is an important antiarrhythmic drug in the pediatric population. Given the risk of proarrhythmia, sotalol is initiated in inpatient settings, with adult studies as recent as 2015 supporting this practice. OBJECTIVE:The purpose of this study was to determine the frequency of adverse events (AEs) during sotalol initiation for the ... Read more >>

Heart Rhythm (Heart rhythm)
[2020, 17(6):984-990]

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Inhibition of mTOR Signaling Enhances Maturation of Cardiomyocytes Derived From Human-Induced Pluripotent Stem Cells via p53-Induced Quiescence.

Jessica C Garbern, Aharon Helman, Rebecca Sereda, Mohsen Sarikhani, Aishah Ahmed, Gabriela O Escalante, Roza Ogurlu, Sean L Kim, John F Zimmerman, Alexander Cho, Luke MacQueen, Vassilios J Bezzerides, Kevin Kit Parker, Douglas A Melton, Richard T Lee,

BACKGROUND:Current differentiation protocols to produce cardiomyocytes from human induced pluripotent stem cells (iPSCs) are capable of generating highly pure cardiomyocyte populations as determined by expression of cardiac troponin T. However, these cardiomyocytes remain immature, more closely resembling the fetal state, with a lower maximum contractile force, slower upstroke velocity, and ... Read more >>

Circulation (Circulation)
[2020, 141(4):285-300]

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Insights Into the Pathogenesis of Catecholaminergic Polymorphic Ventricular Tachycardia From Engineered Human Heart Tissue.

Sung-Jin Park, Donghui Zhang, Yan Qi, Yifei Li, Keel Yong Lee, Vassilios J Bezzerides, Pengcheng Yang, Shutao Xia, Sean L Kim, Xujie Liu, Fujian Lu, Francesco S Pasqualini, Patrick H Campbell, Judith Geva, Amy E Roberts, Andre G Kleber, Dominic J Abrams, William T Pu, Kevin Kit Parker,

BACKGROUND:Modeling of human arrhythmias with induced pluripotent stem cell-derived cardiomyocytes has focused on single-cell phenotypes. However, arrhythmias are the emergent properties of cells assembled into tissues, and the impact of inherited arrhythmia mutations on tissue-level properties of human heart tissue has not been reported. METHODS:Here, we report an optogenetically based, ... Read more >>

Circulation (Circulation)
[2019, 140(5):390-404]

Cited: 3 times

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Gene Therapy for Catecholaminergic Polymorphic Ventricular Tachycardia by Inhibition of Ca2+/Calmodulin-Dependent Kinase II.

Vassilios J Bezzerides, Ana Caballero, Suya Wang, Yulan Ai, Robyn J Hylind, Fujian Lu, Danielle A Heims-Waldron, Kristina D Chambers, Donghui Zhang, Dominic J Abrams, William T Pu,

BACKGROUND:Catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited cardiac arrhythmia characterized by adrenergically triggered arrhythmias, is inadequately treated by current standard of care. Ca2+/calmodulin-dependent protein kinase II (CaMKII), an adrenergically activated kinase that contributes to arrhythmogenesis in heart disease models, is a candidate therapeutic target in CPVT. However, translation of CaMKII ... Read more >>

Circulation (Circulation)
[2019, 140(5):405-419]

Cited: 2 times

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Differentiation of fasciculoventricular fibers from anteroseptal accessory pathways using the surface electrocardiogram.

Edward T O'Leary, Elizabeth S Dewitt, Douglas Y Mah, Kimberlee Gauvreau, Edward P Walsh, Vassilios J Bezzerides,

BACKGROUND:Fasciculoventricular fibers (FVFs) are responsible for 1%-5% of cases of asymptomatic preexcitation on the surface electrocardiogram (ECG). Unlike ventricular preexcitation seen in Wolff-Parkinson-White (WPW) syndrome, FVFs are not associated with sudden cardiac death from preexcited atrial fibrillation. OBJECTIVE:The purpose of this study was to identify surface ECG variables that differentiate ... Read more >>

Heart Rhythm (Heart rhythm)
[2019, 16(7):1072-1079]

Cited: 1 time

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The Real-World Utility of the LINQ Implantable Loop Recorder in Pediatric and Adult Congenital Heart Patients.

Vassilios J Bezzerides, Amy Walsh, Maria Martuscello, Carolina A Escudero, Kimberlee Gauvreau, Geralyn Lam, Dominic J Abrams, John K Triedman, Mark E Alexander, Laura Bevilacqua, Douglas Y Mah,

OBJECTIVES:This study sought to determine the practical use of the recently introduced LINQ implantable loop recorder (LINQ-ILR) in a cohort of pediatric and adult congenital arrhythmia patients. BACKGROUND:Correlating symptoms to a causative arrhythmia is a key aspect of diagnosis and management in clinical electrophysiology. METHODS:Retrospective review of clinical data, implantation ... Read more >>

JACC Clin Electrophysiol (JACC. Clinical electrophysiology)
[2019, 5(2):245-251]

Cited: 0 times

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Channelopathy as a SUDEP Biomarker in Dravet Syndrome Patient-Derived Cardiac Myocytes.

Chad R Frasier, Helen Zhang, James Offord, Louis T Dang, David S Auerbach, Huilin Shi, Chunling Chen, Alica M Goldman, L Lee Eckhardt, Vassilios J Bezzerides, Jack M Parent, Lori L Isom,

Dravet syndrome (DS) is a severe developmental and epileptic encephalopathy with a high incidence of sudden unexpected death in epilepsy (SUDEP). Most DS patients carry de novo variants in SCN1A, resulting in Nav1.1 haploinsufficiency. Because SCN1A is expressed in heart and in brain, we proposed that cardiac arrhythmia contributes to ... Read more >>

Stem Cell Reports (Stem cell reports)
[2018, 11(3):626-634]

Cited: 3 times

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Dual-Site Ventricular Pacing in Patients With Fontan Physiology and Heart Block: Does it Mitigate the Detrimental Effects of Single-Site Ventricular Pacing?

Edward T O'Leary, Kimberlee Gauvreau, Mark E Alexander, Puja Banka, Vassilios J Bezzerides, Francis Fynn-Thompson, John K Triedman, Edward P Walsh, Douglas Y Mah,

OBJECTIVES:This study sought to determine the long-term effect of dual-site ventricular pacing (i.e., cardiac resynchronization therapy [CRT]) versus single-site pacing in patients with Fontan physiology and high-grade atrioventricular block (AVB). BACKGROUND:Chronic single-site ventricular pacing in Fontan patients is associated with significant morbidity and mortality. METHODS:The study conducted a retrospective review ... Read more >>

JACC Clin Electrophysiol (JACC. Clinical electrophysiology)
[2018, 4(10):1289-1297]

Cited: 0 times

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Low molecular weight heparin as an anticoagulation strategy for left-sided ablation procedures.

Karen Hinsley, Margaret Evans-Langhorst, Courtney Porter, Stephanie Chandler, Christina VanderPluym, John Triedman, Vassilios J Bezzerides,

This quality improvement study was implemented to demonstrate consistent and reliable post procedure anticoagulation for patients undergoing left-sided ablations. We evaluated the safety and efficacy of anticoagulation practice during a transition from anticoagulation with overnight infusion of unfractionated heparin to a single subcutaneous injection of low molecular weight heparin.Outcomes for ... Read more >>

Congenit Heart Dis (Congenital heart disease)
[2018, 13(2):222-225]

Cited: 0 times

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Genotype-phenotype-guided medical and surgical intervention in long QT syndrome.

Robyn J Hylind, Virginie Beausejour Ladouceur, Francis Fynn-Thompson, Shannon E Hourigan, Vassilios J Bezzerides, Dominic J Abrams,

HeartRhythm Case Rep (HeartRhythm case reports)
[2018, 4(1):14-17]

Cited: 0 times

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Inhibition of serum and glucocorticoid regulated kinase-1 as novel therapy for cardiac arrhythmia disorders.

Vassilios J Bezzerides, Aifeng Zhang, Ling Xiao, Bridget Simonson, Santosh A Khedkar, Shiro Baba, Filomena Ottaviano, Stacey Lynch, Katherine Hessler, Alan C Rigby, David Milan, Saumya Das, Anthony Rosenzweig,

Alterations in sodium flux (INa) play an important role in the pathogenesis of cardiac arrhythmias and may also contribute to the development of cardiomyopathies. We have recently demonstrated a critical role for the regulation of the voltage-gated sodium channel NaV1.5 in the heart by the serum and glucocorticoid regulated kinase-1 ... Read more >>

Sci Rep (Scientific reports)
[2017, 7(1):346]

Cited: 3 times

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Pausing With the Gauze: Inhibition of Temporary Pacemakers by Radiofrequency Scan During Cardiac Surgery.

Matthew R Williams, Douglas B Atkinson, Vassilios J Bezzerides, Koichi Yuki, Kathryn Franklin, Alfonso Casta, Mark E Alexander,

Radiofrequency identification (RFID) detection systems are used to detect retained surgical sponges and may cause electromagnetic interference (EMI), altering intended function of cardiac pacing systems. Three pediatric patients requiring temporary pacing for postoperative atrioventricular block experienced transient inhibition of ventricular pacing during the use of RFID detection system. Bench testing ... Read more >>

Anesth. Analg. (Anesthesia and analgesia)
[2016, 123(5):1143-1148]

Cited: 1 time

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Acetylation of VGLL4 Regulates Hippo-YAP Signaling and Postnatal Cardiac Growth.

Zhiqiang Lin, Haidong Guo, Yuan Cao, Sylvia Zohrabian, Pingzhu Zhou, Qing Ma, Nathan VanDusen, Yuxuan Guo, Jin Zhang, Sean M Stevens, Feng Liang, Qimin Quan, Pim R van Gorp, Amy Li, Cristobal Dos Remedios, Aibin He, Vassilios J Bezzerides, William T Pu,

Binding of the transcriptional co-activator YAP with the transcription factor TEAD stimulates growth of the heart and other organs. YAP overexpression potently stimulates fetal cardiomyocyte (CM) proliferation, but YAP's mitogenic potency declines postnatally. While investigating factors that limit YAP's postnatal mitogenic activity, we found that the CM-enriched TEAD1 binding protein VGLL4 ... Read more >>

Dev. Cell (Developmental cell)
[2016, 39(4):466-479]

Cited: 25 times

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Modeling Inherited Arrhythmia Disorders Using Induced Pluripotent Stem Cell-Derived Cardiomyocytes.

Vassilios J Bezzerides, Donghui Zhang, William T Pu,

Inherited arrhythmia disorders (IADs) are a group of potentially lethal diseases that remain diagnostic and management challenges. Although the genetic basis for many of these disorders is well known, the pathogenicity of individual mutations and the resulting clinical outcomes are difficult to predict. Treatment options remain imperfect, and optimizing therapy ... Read more >>

Circ. J. (Circulation journal : official journal of the Japanese Circulation Society)
[2016, 81(1):12-21]

Cited: 3 times

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Phenotypic screen quantifying differential regulation of cardiac myocyte hypertrophy identifies CITED4 regulation of myocyte elongation.

Karen A Ryall, Vassilios J Bezzerides, Anthony Rosenzweig, Jeffrey J Saucerman,

Cardiac hypertrophy is controlled by a highly connected signaling network with many effectors of cardiac myocyte size. Quantification of the contribution of individual pathways to specific changes in shape and transcript abundance is needed to better understand hypertrophy signaling and to improve heart failure therapies. We stimulated cardiac myocytes with ... Read more >>

J. Mol. Cell. Cardiol. (Journal of molecular and cellular cardiology)
[2014, 72:74-84]

Cited: 17 times

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Rapid vesicular translocation and insertion of TRP channels.

Vassilios J Bezzerides, I Scott Ramsey, Suhas Kotecha, Anna Greka, David E Clapham,

The broadly expressed transient receptor potential (TRP) family of ion channels are permeant to cations, most resulting in increased intracellular calcium. However, their regulation and gating is not well understood. Here, we report that growth factor stimulation initiates the rapid translocation of the transient receptor potential ion channel, TRPC5, from ... Read more >>

Nat. Cell Biol. (Nature cell biology)
[2004, 6(8):709-720]

Cited: 303 times

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Mechanism of persistent protein kinase D1 translocation and activation.

Elena Oancea, Vassilios J Bezzerides, Anna Greka, David E Clapham,

The specificity of many signal transduction pathways relies on the spatiotemporal features of each signaling step. G protein-coupled receptor-mediated activation of protein kinases leads to diverse cellular effects. Upon receptor activation, PKD1 and several C-type protein kinases (PKCs), translocate to the plasma membrane and become catalytically active. Here we show ... Read more >>

Dev. Cell (Developmental cell)
[2003, 4(4):561-574]

Cited: 33 times

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