2024
Cardiac phenotype in adolescents and young adults with long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency
Elizondo G, Saini A, Gonzalez de Alba C, Gregor A, Harding C, Gillingham M, Vinocur J. Cardiac phenotype in adolescents and young adults with long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency. Genetics In Medicine 2024, 26: 101123. PMID: 38501492, DOI: 10.1016/j.gim.2024.101123.Peer-Reviewed Original ResearchLong-chain 3-hydroxyacyl-CoA dehydrogenase deficiencyLong-chain 3-hydroxyacyl-CoA dehydrogenaseCardiac involvementCardiac arrestMetabolic decompensationCardiac phenotypeDilated cardiomyopathyLong-chain 3-hydroxyacyl-CoA dehydrogenase deficient patientsEpisodes of metabolic decompensationEnd-stage dilated cardiomyopathyOut-of-hospital cardiac arrestAcute cardiac decompensationIn-hospital cardiac arrestFatty acid oxidation disordersLife-threatening arrhythmiasInfantile dilated cardiomyopathySudden cardiac arrestPotential risk factorsCardiac manifestationsCardiac decompensationRestrictive cardiomyopathyRecurrent episodesRetrospective cohortRisk stratificationPeripheral neuropathyReduced kinase function in two ultra‐rare TNNI3K variants in families with congenital junctional ectopic tachycardia
Pham C, Koopmann T, Vinocur J, Blom N, Silbiger V, Mittal K, Bootsma M, Palm K, Clur S, Barge‐Schaapveld D, Hamilton R, Lodder E. Reduced kinase function in two ultra‐rare TNNI3K variants in families with congenital junctional ectopic tachycardia. Clinical Genetics 2024, 106: 37-46. PMID: 38424693, DOI: 10.1111/cge.14504.Peer-Reviewed Original ResearchCongenital junctional ectopic tachycardiaJunctional ectopic tachycardiaK variantEctopic tachycardiaDilated cardiomyopathySupraventricular tachycardiaReduction of kinase activityAssociated with dilated cardiomyopathyFour-generation familyAtrioventricular (AV) nodeInteracting kinasesMissense variantsKinase functionTroponin I-interacting kinaseKinase activityTNNI3KGenetic penetranceTNNI3Variant carriersMultigenerational familiesGenetic testingTroponin ICardiac arrhythmiasTachycardiaVariantsDoubly unusual in double discordance: Appendage-based accessory pathway in congenitally corrected transposition
Beach C, Vinocur J. Doubly unusual in double discordance: Appendage-based accessory pathway in congenitally corrected transposition. HeartRhythm Case Reports 2024, 10: 180-181. PMID: 38496739, PMCID: PMC10943534, DOI: 10.1016/j.hrcr.2023.12.018.Peer-Reviewed Original Research
2023
Lyme Carditis in the Pediatric Population
Beach C, Vinocur J. Lyme Carditis in the Pediatric Population. 2023, 111-119. DOI: 10.1007/978-3-031-41169-4_11.Peer-Reviewed Original ResearchHigh-grade AV blockTemporary transvenous pacingPediatricPediatric patientsLyme carditisHemodynamic instabilityAntibiotic therapyPediatric populationPR prolongationTransvenous pacingAV blockCommon manifestationConduction abnormalitiesTimely administrationCarditisFull recoveryPatientsDiseaseGreat majorityAccompanying increaseTherapyPacingAbnormalitiesSuspicionAdministrationProlongationClinical presentation of calmodulin mutations: the International Calmodulinopathy Registry
Crotti L, Spazzolini C, Nyegaard M, Overgaard M, Kotta M, Dagradi F, Sala L, Aiba T, Ayers M, Baban A, Barc J, Beach C, Behr E, Bos J, Cerrone M, Covi P, Cuneo B, Denjoy I, Donner B, Elbert A, Eliasson H, Etheridge S, Fukuyama M, Girolami F, Hamilton R, Horie M, Iascone M, Jiménez-Jaimez J, Jensen H, Kannankeril P, Kaski J, Makita N, Muñoz-Esparza C, Odland H, Ohno S, Papagiannis J, Porretta A, Prandstetter C, Probst V, Robyns T, Rosenthal E, Rosés-Noguer F, Sekarski N, Singh A, Spentzou G, Stute F, Tfelt-Hansen J, Till J, Tobert K, Vinocur J, Webster G, Wilde A, Wolf C, Ackerman M, Schwartz P. Clinical presentation of calmodulin mutations: the International Calmodulinopathy Registry. European Heart Journal 2023, 44: 3357-3370. PMID: 37528649, PMCID: PMC10499544, DOI: 10.1093/eurheartj/ehad418.Peer-Reviewed Original ResearchConceptsClinical presentationPrimary neurological manifestationsCardiac structural abnormalitiesLife-threatening arrhythmia syndromesArrhythmic event rateAbsence of symptomsLife-threatening arrhythmiasSodium channel blockersCongenital heart defectsAntiadrenergic interventionsCardiac eventsHeart failureNeurological manifestationsUnderlying molecular mechanismsDefinitive recommendationsClinical severityChannel blockersObservational studySudden deathIndex caseArrhythmia syndromesHeart defectsPrevalent phenotypeStructural abnormalitiesCurrent managementTranslation of Tools and Techniques from the Adult Electrophysiology World to Pediatric Cardiac Implantable Electronic Devices
Howard T, Vinocur J. Translation of Tools and Techniques from the Adult Electrophysiology World to Pediatric Cardiac Implantable Electronic Devices. Cardiac Electrophysiology Clinics 2023, 15: 515-525. PMID: 37865524, DOI: 10.1016/j.ccep.2023.06.004.Peer-Reviewed Original ResearchWolff-Parkinson-White (WPW) Syndrome
Vinocur J. Wolff-Parkinson-White (WPW) Syndrome. In Clinical Practice 2023, 103-127. DOI: 10.1007/978-3-031-41479-4_6.Peer-Reviewed Original ResearchAsymptomatic patientsRisk stratificationSudden deathCalcium channel blockersEarliest atrial activationAntidromic AVRTAsymptomatic WPWOrthodromic AVRTPharmacologic therapyUncommon manifestationCatheter ablationVariety of manifestationsAtrial fibrillationPathway conductionWolff-ParkinsonAccessory pathwaySerious manifestationsVentricular pacingBidirectional accessory pathwaysCatheter stabilityWhite syndromeAtrial activationFirst lesionPatientsWPW
2022
Life-threatening cardiac arrhythmia and sudden death during electronic gaming: An international case series and systematic review
Lawley C, Tester M, Sanatani S, Prendiville T, Beach C, Vinocur J, Horie M, Uhm J, Khongphatthanayothin A, Ayers M, Starling L, Yoshida Y, Shah M, Skinner J, Turner C. Life-threatening cardiac arrhythmia and sudden death during electronic gaming: An international case series and systematic review. Heart Rhythm 2022, 19: 1826-1833. PMID: 37850595, DOI: 10.1016/j.hrthm.2022.08.003.Peer-Reviewed Original ResearchConceptsInternational case seriesLife-threatening cardiac arrhythmiasCardiac arrhythmiasCase seriesSystematic reviewCatecholaminergic polymorphic ventricular tachycardiaPolymorphic ventricular tachycardiaLong QT syndromeLethal cardiac arrhythmiasCardiac eventsCoronary ischemiaCardiac surgeryVentricular arrhythmiasCardiac arrestSusceptible childrenVentricular tachycardiaVentricular fibrillationSudden deathCardiac conditionsArrhythmic triggersCardiac diagnosisPatientsQT syndromeSusceptible individualsArrhythmiasAblation of ventricular ectopy in complex congenital heart disease: The best of both worlds
Vinocur JM. Ablation of ventricular ectopy in complex congenital heart disease: The best of both worlds. HeartRhythm Case Reports 2022, 8: 548. PMID: 35996706, PMCID: PMC9391403, DOI: 10.1016/j.hrcr.2022.05.021.Commentaries, Editorials and LettersAn unusual cause of repolarization abnormality after congenital heart surgery: A case report
Barresi NV, Marcus BS, Beach CM, Vinocur JM. An unusual cause of repolarization abnormality after congenital heart surgery: A case report. Journal Of Electrocardiology 2022, 72: 91-94. PMID: 35395484, DOI: 10.1016/j.jelectrocard.2022.03.007.Peer-Reviewed Case Reports and Technical NotesClinical and Functional Characterization of Ryanodine Receptor 2 Variants Implicated in Calcium-Release Deficiency Syndrome
Roston TM, Wei J, Guo W, Li Y, Zhong X, Wang R, Estillore JP, Peltenburg PJ, Noguer FRI, Till J, Eckhardt LL, Orland KM, Hamilton R, LaPage MJ, Krahn AD, Tadros R, Vinocur JM, Kallas D, Franciosi S, Roberts JD, Wilde AAM, Jensen HK, Sanatani S, Chen SRW. Clinical and Functional Characterization of Ryanodine Receptor 2 Variants Implicated in Calcium-Release Deficiency Syndrome. JAMA Cardiology 2022, 7: 84-92. PMID: 34730774, PMCID: PMC8567190, DOI: 10.1001/jamacardio.2021.4458.Peer-Reviewed Original ResearchConceptsCatecholaminergic polymorphic ventricular tachycardiaExercise stress testingDeficiency syndromeRYR2 variantsVentricular tachyarrhythmiasArrhythmic eventsVentricular fibrillationLife-threatening arrhythmic eventsMulticenter observational cohort studyFunction variantsCardiac ryanodine receptor 2Complex ventricular tachyarrhythmiasPrevious arrhythmic eventsΒ-blocker therapyObservational cohort studyPolymorphic ventricular tachycardiaLife-threatening eventsSpectrum of diseaseVentricular fibrillation episodesFirst clinical seriesRyanodine receptor 2Better diagnostic toolsCohort studyVentricular arrhythmiasClinical series
2021
Fortuitous Left Bundle Branch Area Pacing in a Small Child
Vinocur JM. Fortuitous Left Bundle Branch Area Pacing in a Small Child. JACC Case Reports 2021, 3: 1730-1735. PMID: 34825198, PMCID: PMC8603135, DOI: 10.1016/j.jaccas.2021.07.032.Peer-Reviewed Case Reports and Technical NotesLong-term Outcomes of Children Operated on for Anomalous Left Coronary Artery From the Pulmonary Artery
Thomas AS, Chan A, Alsoufi B, Vinocur JM, Kochilas L. Long-term Outcomes of Children Operated on for Anomalous Left Coronary Artery From the Pulmonary Artery. The Annals Of Thoracic Surgery 2021, 113: 1223-1230. PMID: 34419434, PMCID: PMC9012605, DOI: 10.1016/j.athoracsur.2021.07.053.Peer-Reviewed Original ResearchConceptsSevere mitral regurgitationLong-term outcomesAnomalous left coronary arteryLeft coronary arteryMitral regurgitationHospital deathCoronary reimplantationLV dysfunctionPulmonary arteryCoronary arteryMR severitySevere left ventricular dysfunctionBetter long-term survivalFavorable long-term outcomePediatric Cardiac Care ConsortiumCoronary artery reimplantationPreoperative mitral regurgitationTransplant-free survivalLeft ventricular dysfunctionSevere LV dysfunctionMitral valve operationsNational Death IndexRisk of deathOutcome of childrenLong-term survivalAn interesting response to adenosine during right ventricular pacing. What is the mechanism?
Gupta A, Vinocur JM. An interesting response to adenosine during right ventricular pacing. What is the mechanism? Journal Of Electrocardiology 2021, 68: 41-43. PMID: 34311213, DOI: 10.1016/j.jelectrocard.2021.07.010.Peer-Reviewed Case Reports and Technical NotesIdentification and successful management of near-lethal ventricular tachycardia in 2q24 deletion-associated developmental and epileptic encephalopathy
Toth J, Waickman A, Jost J, Seltzer L, Vinocur JM, Auerbach DS. Identification and successful management of near-lethal ventricular tachycardia in 2q24 deletion-associated developmental and epileptic encephalopathy. Seizure 2021, 91: 146-149. PMID: 34161902, DOI: 10.1016/j.seizure.2021.06.003.Peer-Reviewed Case Reports and Technical NotesAuthor’s Reply—Phase-4 RBBB in Lyme carditis?
Vinocur JM, Baranchuk A. Author’s Reply—Phase-4 RBBB in Lyme carditis? HeartRhythm Case Reports 2021, 7: 506. PMID: 34307042, PMCID: PMC8283531, DOI: 10.1016/j.hrcr.2021.04.010.Commentaries, Editorials and Letters
2020
Phase 4 block of the right bundle branch suggesting His-Purkinje system involvement in Lyme carditis
Maxwell N, Dryer MM, Baranchuk A, Vinocur JM. Phase 4 block of the right bundle branch suggesting His-Purkinje system involvement in Lyme carditis. HeartRhythm Case Reports 2020, 7: 112-116. PMID: 33665114, PMCID: PMC7897763, DOI: 10.1016/j.hrcr.2020.11.017.Peer-Reviewed Case Reports and Technical NotesPermanent conduction system pacing for congenitally corrected transposition of the great arteries: A Pediatric and Congenital Electrophysiology Society (PACES)/International Society for Adult Congenital Heart Disease (ISACHD) Collaborative Study
Moore JP, Gallotti R, Shannon KM, Pilcher T, Vinocur JM, Cano Ó, Kean A, Mondesert B, Nürnberg JH, Schaller RD, Sharma PS, Nishimura T, Tung R. Permanent conduction system pacing for congenitally corrected transposition of the great arteries: A Pediatric and Congenital Electrophysiology Society (PACES)/International Society for Adult Congenital Heart Disease (ISACHD) Collaborative Study. Heart Rhythm 2020, 17: 991-997. PMID: 32243875, DOI: 10.1016/j.hrthm.2020.01.033.Peer-Reviewed Original ResearchConventional cardiac resynchronization therapyConduction system pacingConduction systemGreat arteriesVentricular pacingNew York Heart Association functional classConventional CRTYork Heart Association functional classChronic ventricular pacingJunctional escape rhythmSpontaneous atrioventricular blockCardiac resynchronization therapyCongenital Electrophysiology SocietyPacing-induced cardiomyopathyProximal left bundle branchLeft bundle branchBundle branch pacingUnique anatomic characteristicsCcTGA patientsEscape rhythmHV intervalPrevious surgeryResynchronization therapyAtrioventricular blockPulmonary valve
2018
Mortality Following Pediatric Congenital Heart Surgery: An Analysis of the Causes of Death Derived From the National Death Index
McCracken C, Spector LG, Menk JS, Knight JH, Vinocur JM, Thomas AS, Oster ME, St Louis JD, Moller JH, Kochilas L. Mortality Following Pediatric Congenital Heart Surgery: An Analysis of the Causes of Death Derived From the National Death Index. Journal Of The American Heart Association 2018, 7: e010624. PMID: 30571499, PMCID: PMC6404427, DOI: 10.1161/jaha.118.010624.Peer-Reviewed Original ResearchConceptsCongenital heart surgeryNational Death IndexCongenital heart defectsStandardized mortality ratioCause of deathHeart surgeryDeath IndexCardiovascular disordersHeart failureSeverity of CHDCause-specific standardized mortality ratiosPediatric Cardiac Care ConsortiumRetrospective cohort studyAdjusted odds ratioLong-term outcomesRisk of deathLong-term riskConclusions SurvivorsMedian followNoncardiac conditionsCohort studyResidual morbidityEarly outcomesLate causesCardiac arrestTrends in Long-Term Mortality After Congenital Heart Surgery
Spector LG, Menk JS, Knight JH, McCracken C, Thomas AS, Vinocur JM, Oster ME, St Louis JD, Moller JH, Kochilas L. Trends in Long-Term Mortality After Congenital Heart Surgery. Journal Of The American College Of Cardiology 2018, 71: 2434-2446. PMID: 29793633, PMCID: PMC5978758, DOI: 10.1016/j.jacc.2018.03.491.Peer-Reviewed Original ResearchConceptsStandardized mortality ratioCongenital heart surgeryLong-term mortalityHeart surgeryLong-term survivalGeneral populationOverall standardized mortality ratioSurvival of patientsLarge U.S. cohortNational Death IndexPatent ductus arteriosusPediatric cardiac surgeryAtrial septal defectForms of CHDMulticenter registryCardiac surgeryDuctus arteriosusSingle ventricleDeath IndexGreat arteriesSeptal defectMortality ratioU.S. cohortSurgerySurvival analysis