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 ResearchMeSH KeywordsCalmodulinChildDeath, Sudden, CardiacHumansLong QT SyndromeMutationRegistriesTachycardia, VentricularConceptsClinical 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 management
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 rootRevascularisation
2017
The 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
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
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
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