2023
Clinical 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 Research
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 individualsArrhythmiasClinical 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
Long-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 Notes
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
2017
Brugada phenocopy associated with diabetic ketoacidosis in two pediatric patients
Alanzalon RE, Burris JR, Vinocur JM. Brugada phenocopy associated with diabetic ketoacidosis in two pediatric patients. Journal Of Electrocardiology 2017, 51: 323-326. PMID: 29174707, DOI: 10.1016/j.jelectrocard.2017.10.017.Peer-Reviewed Case Reports and Technical NotesDevelopment of quality metrics for ambulatory pediatric cardiology: Infection prevention
Johnson JN, Barrett CS, Franklin WH, Graham EM, Halnon NJ, Hattendorf BA, Krawczeski CD, McGovern JJ, O'Connor MJ, Schultz AH, Vinocur JM, Chowdhury D, Anderson JB. Development of quality metrics for ambulatory pediatric cardiology: Infection prevention. Congenital Heart Disease 2017, 12: 756-761. PMID: 28741863, DOI: 10.1111/chd.12519.Peer-Reviewed Original ResearchConceptsInfection preventionPediatric cardiologyAntibiotic prophylaxisRheumatic feverHand hygieneAdult CongenitalAmbulatory practiceRespiratory syncytial virus prophylaxisAsplenia/hyposplenismInfluenza vaccination complianceInfluenza vaccination recommendationsTetralogy of FallotQuality improvement initiativesAmbulatory pediatric cardiologyChest painInfluenza vaccinationKawasaki diseaseSecondary preventionVaccination recommendationsArterial switchCardiopulmonary bypassGreat arteriesSplenic functionVaccination compliancePreoperative methodDevelopment of Quality Metrics in Ambulatory Pediatric Cardiology
Chowdhury D, Gurvitz M, Marelli A, Anderson J, Baker-Smith C, Diab K, Edwards T, Hougen T, Jedeikin R, Johnson J, Karpawich P, Lai W, Lu J, Mitchell S, Newburger J, Penny D, Portman M, Satou G, Teitel D, Villafane J, Williams R, Jenkins K, Group A, Williams R, Jenkins K, Gurvitz M, Marelli A, Campbell R, Chowdhury D, Jedeikin R, Behera S, Hokanson J, Lu J, Kakavand B, Boris J, Cardis B, Bansal M, Anderson J, Schultz A, O'Connor M, Vinocur J, Halnon N, Johnson J, Barrett C, Graham E, Krawczeski C, Franklin W, McGovern J, Hattendorf B, Teitel D, Cotts T, Davidson A, Harahsheh A, Johnson W, Jone P, Sutton N, Tani L, Dahdah N, Portman M, Mensch D, Newburger J, Hougen T, Cross R, Diab K, Karpawich P, Lai W, Peuster M, Schiff R, Saarel E, Satou G, Serwer G, Villafane J, Edwards T, Penny D, Carlson K, Jayakumar K, Park M, Tede N, Uzark K, Smith C, Fleishman C, Connuck D, Ettedgui J, Likes M, Tsuda T. Development of Quality Metrics in Ambulatory Pediatric Cardiology. Journal Of The American College Of Cardiology 2017, 69: 541-555. PMID: 28153110, DOI: 10.1016/j.jacc.2016.11.043.Peer-Reviewed Original ResearchThe clinical and genetic spectrum of catecholaminergic polymorphic ventricular tachycardia: findings from an international multicentre registry
Roston TM, Yuchi Z, Kannankeril PJ, Hathaway J, Vinocur JM, Etheridge SP, Potts JE, Maginot KR, Salerno JC, Cohen MI, Hamilton RM, Pflaumer A, Mohammed S, Kimlicka L, Kanter RJ, LaPage MJ, Collins KK, Gebauer RA, Temple JD, Batra AS, Erickson C, Miszczak-Knecht M, Kubuš P, Bar-Cohen Y, Kantoch M, Thomas VC, Hessling G, Anderson C, Young ML, Choi SHJ, Cabrera Ortega M, Lau YR, Johnsrude CL, Fournier A, Van Petegem F, Sanatani S. The clinical and genetic spectrum of catecholaminergic polymorphic ventricular tachycardia: findings from an international multicentre registry. EP Europace 2017, 20: 541-547. PMID: 28158428, PMCID: PMC6059141, DOI: 10.1093/europace/euw389.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentCalsequestrinChildDeath, Sudden, CardiacDNA Mutational AnalysisFemaleGenetic MarkersGenetic Predisposition to DiseaseHeredityHumansMaleModels, MolecularMutationPedigreePhenotypePrognosisProtein ConformationRegistriesRetrospective StudiesRisk FactorsRyanodine Receptor Calcium Release ChannelStructure-Activity RelationshipTachycardia, VentricularConceptsCatecholaminergic polymorphic ventricular tachycardiaPolymorphic ventricular tachycardiaCardiac eventsCPVT patientsVentricular tachycardiaLife-threatening cardiac eventsInternational multicentre registrySevere CPVT phenotypeRetrospective cohort studyFirst-degree relativesYears of ageRyanodine receptor 2CPVT phenotypeMulticentre registryCohort studySymptomatic patientsMulticentre studyVentricular arrhythmiasCardiac arrestPrognostic markerReceptor 2Ion channelopathiesClinical phenotypeGenetic spectrumGenotypic spectrum
2016
Factors Affecting Length of Postoperative Hospitalization for Pediatric Cardiac Operations in a Large North American Registry (1982–2007)
al-Haddad BJ, Menk JS, Kochilas L, Vinocur JM. Factors Affecting Length of Postoperative Hospitalization for Pediatric Cardiac Operations in a Large North American Registry (1982–2007). Pediatric Cardiology 2016, 37: 884-891. PMID: 26965705, PMCID: PMC5724563, DOI: 10.1007/s00246-016-1364-0.Peer-Reviewed Original ResearchConceptsPostoperative hospitalizationSurgical volumeAnnual surgical volumeHealthcare resource useLow-volume centersPediatric cardiac operationsFactors Affecting LengthCongenital heart diseaseNorth American RegistrySurgical treatmentEarly dischargeCardiac operationsPediatric hospitalizationsVolume centersHeart diseaseClinical significanceMerit further investigationSex-specific differencesHospitalizationEfficient careMajor causeRisk adjustmentOlder childrenTime interactionRandom effects
2015
Catecholaminergic Polymorphic Ventricular Tachycardia in Children
Roston TM, Vinocur JM, Maginot KR, Mohammed S, Salerno JC, Etheridge SP, Cohen M, Hamilton RM, Pflaumer A, Kanter RJ, Potts JE, LaPage MJ, Collins KK, Gebauer RA, Temple JD, Batra AS, Erickson C, Miszczak-Knecht M, Kubuš P, Bar-Cohen Y, Kantoch M, Thomas VC, Hessling G, Anderson C, Young ML, Cabrera Ortega M, Lau YR, Johnsrude CL, Fournier A, Kannankeril PJ, Sanatani S. Catecholaminergic Polymorphic Ventricular Tachycardia in Children. Circulation Arrhythmia And Electrophysiology 2015, 8: 633-642. PMID: 25713214, PMCID: PMC4472494, DOI: 10.1161/circep.114.002217.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAge FactorsAnti-Arrhythmia AgentsChildDeath, Sudden, CardiacDefibrillators, ImplantableElectric CountershockFemaleHumansMalePatient SelectionPhenotypeRegistriesRetrospective StudiesRisk FactorsSeverity of Illness IndexSympathectomyTachycardia, VentricularTime FactorsTreatment OutcomeConceptsCatecholaminergic polymorphic ventricular tachycardiaPolymorphic ventricular tachycardiaCardiac sympathetic denervationImplantable cardioverter defibrillatorVentricular tachycardiaSympathetic denervationTreatment failureCardioverter defibrillatorΒ-blockersCatecholaminergic polymorphic ventricular tachycardia patientsQuarter of patientsRetrospective cohort studyDevice-related complicationsTreatment failure eventsYears of ageVentricular tachycardia patientsCohort studyStandard therapySymptom onsetSymptomatic presentationCardiac arrestSubtherapeutic dosingTreatment outcomesElectrical stormTachycardia patients
2014
Age‐Dependent Sex Effects on Outcomes After Pediatric Cardiac Surgery
Kochilas LK, Vinocur JM, Menk JS. Age‐Dependent Sex Effects on Outcomes After Pediatric Cardiac Surgery. Journal Of The American Heart Association 2014, 3: e000608. PMID: 24496232, PMCID: PMC3959673, DOI: 10.1161/jaha.113.000608.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAge FactorsCardiac Surgical ProceduresChildChild, PreschoolChi-Square DistributionFemaleHealth Status DisparitiesHeart Defects, CongenitalHospital MortalityHumansInfantInfant, NewbornLogistic ModelsMaleMultivariate AnalysisNorth AmericaOdds RatioRegistriesRetrospective StudiesRisk FactorsSex FactorsTime FactorsTreatment OutcomeConceptsPediatric cardiac operationsPediatric cardiac surgeryCardiac surgeryCardiac operationsPostsurgical mortalityHigh-risk cardiac operationsPediatric Cardiac Care ConsortiumRisk of deathMonths of lifePotential sex differencesAge-dependent relationshipSurgical yearHospital mortalityUnadjusted mortalityPatient sexWhole cohortHigh-risk operationsCardiovascular diseaseSex effectsCardiovascular stressCare ConsortiumEarly infancyZ-scoreLogistic regressionMortality
2013
Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up
Foker J, Berry J, Vinocur J, Harvey B, Pyles L. Two-ventricle repairs in the unbalanced atrioventricular canal defect spectrum with midterm follow-up. Journal Of Thoracic And Cardiovascular Surgery 2013, 146: 854-860.e3. PMID: 23871139, DOI: 10.1016/j.jtcvs.2013.05.013.Peer-Reviewed Original ResearchAbnormalities, MultipleAdolescentAdultCardiac Surgical ProceduresChildChild, PreschoolEndocardial Cushion DefectsFemaleHeart Septal DefectsHeart Septal Defects, AtrialHeart Septal Defects, VentricularHeart VentriclesHumansInfantInfant, NewbornMaleTime FactorsTreatment OutcomeUltrasonographyYoung AdultFrequency of Superior Vena Cava Obstruction in Pediatric Heart Transplant Recipients and Its Relation to Previous Superior Cavopulmonary Anastomosis
Aldoss O, Arain NI, Vinocur JM, Menk J, Ameduri RK, Bryant R, Kochilas LK, Gruenstein DH. Frequency of Superior Vena Cava Obstruction in Pediatric Heart Transplant Recipients and Its Relation to Previous Superior Cavopulmonary Anastomosis. The American Journal Of Cardiology 2013, 112: 286-291. PMID: 23587279, PMCID: PMC6118126, DOI: 10.1016/j.amjcard.2013.03.029.Peer-Reviewed Original ResearchConceptsOrthotopic heart transplantationSuperior vena cava obstructionSVC obstructionVena cava obstructionCavopulmonary anastomosisSuperior cavopulmonary anastomosisCardiac surgeryCava obstructionRisk factorsPediatric heart transplant recipientsPediatric orthotopic heart transplantationSubsequent orthotopic heart transplantationPediatric OHT patientsHeart transplant recipientsPrevious cardiac surgerySignificant risk factorsOHT patientsPosttransplant developmentHeart transplantationTransplant recipientsRetrospective reviewPatientsObstructionSurgeryTransplantationSurgical Volume and Center Effects on Early Mortality After Pediatric Cardiac Surgery: 25-Year North American Experience From a Multi-institutional Registry
Vinocur JM, Menk JS, Connett J, Moller JH, Kochilas LK. Surgical Volume and Center Effects on Early Mortality After Pediatric Cardiac Surgery: 25-Year North American Experience From a Multi-institutional Registry. Pediatric Cardiology 2013, 34: 1226-1236. PMID: 23377381, PMCID: PMC4357309, DOI: 10.1007/s00246-013-0633-4.Peer-Reviewed Original ResearchConceptsPediatric cardiac surgerySurgical volumePostoperative mortalityCardiac surgeryOdds ratioCenter effectVolume categoriesHierarchical multivariate logistic regression analysisMultivariate logistic regression analysisPediatric Cardiac Care ConsortiumRisk categoriesStudy periodCenter-specific variationCentres' surgical volumeRisk Adjusted ClassificationRetrospective cohort studyEarly postoperative mortalityMulti-institutional registryOdds of deathCongenital heart surgeryNorth American centersLogistic regression analysisLow-risk operationCohort studyPatient characteristics
2012
Putting the Pediatric Cardiac Care Consortium in Context
Vinocur JM, Moller JH, Kochilas LK. Putting the Pediatric Cardiac Care Consortium in Context. Circulation Cardiovascular Quality And Outcomes 2012, 5: 577-579. PMID: 22811500, DOI: 10.1161/circoutcomes.111.964841.Peer-Reviewed Original ResearchAdolescentAge FactorsCardiac Surgical ProceduresCardiologyChildChild, PreschoolCooperative BehaviorHealth Services ResearchHeart DiseasesHumansInfantInfant, NewbornMulticenter Studies as TopicOutcome Assessment, Health CarePediatricsRegistriesRisk AssessmentRisk FactorsTreatment OutcomeUnited States