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 individualsArrhythmiasAn 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
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 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 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 analysisOutcomes after surgical coronary artery revascularisation in children with congenital heart disease
Thammineni K, Vinocur J, Harvey B, Menk J, Kelleman M, Korakiti A, Thomas A, Moller J, St Louis J, Kochilas L. Outcomes after surgical coronary artery revascularisation in children with congenital heart disease. Heart 2018, 104: 1417. PMID: 29472291, PMCID: PMC6092219, DOI: 10.1136/heartjnl-2017-312652.Peer-Reviewed Original ResearchConceptsSurgical coronary revascularisationCongenital heart diseaseTransplant-free survivalCoronary revascularisationHeart diseaseLong-term transplant-free survivalUS National Death IndexPediatric Cardiac Care ConsortiumRetrospective cohort studyCoronary artery revascularisationNational Death IndexLong-term outcomesGraft patency dataLong-term survivalCoronary anomaliesHospital mortalityMulticentre registryBypass GraftingHospital deathCohort studyHospital dischargePatency dataDeath IndexAortic rootRevascularisationHereditary arrhythmias and cardiomyopathies
Louis C, Calamaro E, Vinocur JM. Hereditary arrhythmias and cardiomyopathies. Current Opinion In Cardiology 2018, 33: 78-86. PMID: 29059074, DOI: 10.1097/hco.0000000000000477.Commentaries, Editorials and LettersMeSH KeywordsArrhythmias, CardiacArrhythmogenic Right Ventricular DysplasiaBrugada SyndromeCardiomyopathiesCardiomyopathy, DilatedCardiomyopathy, Hypertrophic, FamilialClinical Decision-MakingGenetic CounselingGenetic TestingHealth Services AccessibilityHumansLong QT SyndromePatient Care TeamPractice Guidelines as TopicTachycardia, VentricularConceptsHereditary arrhythmiasGenetic testingClass I recommendationManagement of patientsStandard of careAppropriateness of useI recommendationConsensus guidelinesCardiomyopathy diagnosisFamily screeningClinical geneticsReproductive planningClinical practiceMultidisciplinary teamOptimal managementArrhythmiasAppropriate useCardiomyopathyDiagnosisGenetic counselorsGuidelinesCase-based approachPatientsPrognosisTesting
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 NotesLong-Term Transplant-Free Survival After Repair of Total Anomalous Pulmonary Venous Connection
St. Louis J, McCracken C, Turk E, Hancock H, Menk J, Harvey B, Vinocur J, Oster M, Moller J, Spector L, Kochilas L. Long-Term Transplant-Free Survival After Repair of Total Anomalous Pulmonary Venous Connection. The Annals Of Thoracic Surgery 2017, 105: 186-192. PMID: 28847536, PMCID: PMC5729081, DOI: 10.1016/j.athoracsur.2017.05.052.Peer-Reviewed Original ResearchConceptsTotal anomalous pulmonary venous connectionTransplant-free survivalAnomalous pulmonary venous connectionPediatric Cardiac Care ConsortiumPulmonary venous connectionNational Death IndexLong-term outcomesTAPVC repairPostoperative lengthVenous connectionCardiac lesionsDeath IndexUnited NetworkOrgan SharingCare ConsortiumRepair of TAPVCLong-term transplant-free survivalWorse long-term outcomesBilateral lung transplantationDeath/transplantRisks of transplantationRisk of mortalityTime of procedureMajority of deathsCause of deathDevelopment 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 spectrumCongenital Diaphragmatic Hernia with Liver Herniation into the Pericardial Sac in a 30-Week Gestation Infant
Patel N, Sollinger C, D’Angio C, Vinocur JM, Ackerman KG, Katzman PJ. Congenital Diaphragmatic Hernia with Liver Herniation into the Pericardial Sac in a 30-Week Gestation Infant. Pediatric And Developmental Pathology 2017, 20: 421-425. PMID: 28812464, DOI: 10.1177/1093526616686446.Peer-Reviewed Case Reports and Technical NotesConceptsLiver herniationDiaphragmatic defectPericardial tumorPericardial sacAnterior diaphragmatic defectSubset of patientsCongenital diaphragmatic herniaAtrioventricular septal defectPentalogy of CantrellMultiple congenital anomaliesComplex of syndromesPericardial involvementGestation infantsDiaphragmatic herniaSeptal defectUnusual presentationAssociated anomaliesEarly recognitionPostmortem studiesCongenital anomaliesChallenging entityPrenatal ultrasoundHerniationPentalogyTumors
2016
In‐Hospital Vital Status and Heart Transplants After Intervention for Congenital Heart Disease in the Pediatric Cardiac Care Consortium: Completeness of Ascertainment Using the National Death Index and United Network for Organ Sharing Datasets
Spector L, Menk J, Vinocur J, Oster M, Harvey B, St. Louis J, Moller J, Kochilas L. In‐Hospital Vital Status and Heart Transplants After Intervention for Congenital Heart Disease in the Pediatric Cardiac Care Consortium: Completeness of Ascertainment Using the National Death Index and United Network for Organ Sharing Datasets. Journal Of The American Heart Association 2016, 5: e003783. PMID: 27506544, PMCID: PMC5015299, DOI: 10.1161/jaha.116.003783.Peer-Reviewed Original ResearchConceptsPediatric Cardiac Care ConsortiumNational Death IndexCongenital heart diseaseOrgan Sharing datasetHeart transplantUNOS registryDeath IndexUnited NetworkHeart diseaseCare ConsortiumLong-term deathLong-term outcomesCompleteness of ascertainmentHospital deathTransplant statusVital statusNational registryCase ascertainmentCardiovascular conditionsTransplant eventsTransplantYears postinterventionRegistryDeathPatientsFactors 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