2024
Efficacy and Safety of aficamten in Symptomatic Non-Obstructive Hypertrophic Cardiomyopathy: Results From the REDWOOD-HCM Trial, Cohort 4
Masri A, Sherrid M, Abraham T, Choudhury L, Garcia-Pavia P, Kramer C, Barriales-Villa R, Owens A, Rader F, Nagueh S, Olivotto I, Saberi S, Tower-Rader A, Wong T, Coats C, Watkins H, Fifer M, Solomon S, Heitner S, Jacoby D, Kupfer S, Malik F, Meng L, Sohn R, Wohltman A, Maron M, Investigators R. Efficacy and Safety of aficamten in Symptomatic Non-Obstructive Hypertrophic Cardiomyopathy: Results From the REDWOOD-HCM Trial, Cohort 4. Journal Of Cardiac Failure 2024 PMID: 38493832, DOI: 10.1016/j.cardfail.2024.02.020.Peer-Reviewed Original ResearchNonobstructive hypertrophic cardiomyopathyHypertrophic cardiomyopathyKansas City Cardiomyopathy Questionnaire Clinical Summary ScoreOpen-label phase 2 trialNew York Heart Association classHistory of aborted sudden cardiac deathNon-obstructive hypertrophic cardiomyopathyHigh-sensitivity cardiac troponin IAborted sudden cardiac deathBlood levels of biomarkersNT-proBNP levelsPhase 2 trialPlacebo-controlled studyHeart failure symptomsCardiac myosin inhibitorClinical summary scoreSudden cardiac deathClinically relevant improvementWeeks of washoutLevels of biomarkersCardiac troponin IHeart muscle cellsAsymptomatic reductionNT-proBNPAssociated with improvementsCardiovascular Hospitalizationspost Septal Myectomy For Obstructive Hypertrophic Cardiomyopathy: A 3-year Analysis Of 5,101 Patients
Altibi A, Elman M, Zhao Y, Chehab J, Butzner M, Heitner S, Jacoby D, Sohn R, Masri A. Cardiovascular Hospitalizationspost Septal Myectomy For Obstructive Hypertrophic Cardiomyopathy: A 3-year Analysis Of 5,101 Patients. Journal Of Cardiac Failure 2024, 30: 199-200. DOI: 10.1016/j.cardfail.2023.10.201.Peer-Reviewed Original ResearchObstructive hypertrophic cardiomyopathyIncidence of cardiovascular hospitalizationsSeptal myectomyCardiovascular hospitalizationChronic kidney diseaseChronic obstructive pulmonary diseaseHypertrophic cardiomyopathyPost-SMVentricular arrhythmiasHeart failureCumulative incidence of cardiovascular hospitalizationsFollow-upYears median follow-upMedian follow-upAdvanced HF therapiesHealth claims databaseAdvanced heart failureEmergency department visitsPacemaker/implantable cardioverter defibrillatorObstructive pulmonary diseaseHF therapyAtrial fibrillation/flutterCumulative incidenceCardiac arrestMultivariate regression modelCharacteristics And Procedural Complications Of Septal Myectomy For Obstructive Hypertrophic Cardiomyopathy In The United States
Altibi A, Elman M, Zhao Y, Chehab J, Butzner M, Heitner S, Jacoby D, Sohn R, Masri A. Characteristics And Procedural Complications Of Septal Myectomy For Obstructive Hypertrophic Cardiomyopathy In The United States. Journal Of Cardiac Failure 2024, 30: 177-178. DOI: 10.1016/j.cardfail.2023.10.149.Peer-Reviewed Original ResearchObstructive hypertrophic cardiomyopathySeptal myectomyBaseline characteristicsHypertrophic cardiomyopathyInpatient complicationsConcomitant mitral valve repairVentricular septal defectMitral valve repairMitral valve replacementPredictor of inpatient complicationsCohort of patientsOutcomes of SMValve repairSeptal defectValve replacementAtrial fibrillation/flutterAnoxic brain damagePacemaker implantationAdult patientsHeart blockCardiac arrestEmergency intubationProcedural outcomesContinuous enrollmentMyectomyLong-term Safety And Tolerability Of Acoramidis (AG10) In Symptomatic Transthyretin Amyloid Cardiomyopathy: 4-year Update From An Ongoing, Phase 2, Open-label Extension Study
Masri A, Aras M, Falk R, Grogan M, Jacoby D, Maurer M, Shah S, Witteles R, Wong P, Ji A, Du J, Siddhanti S, Sinha U, Fox J, Judge D. Long-term Safety And Tolerability Of Acoramidis (AG10) In Symptomatic Transthyretin Amyloid Cardiomyopathy: 4-year Update From An Ongoing, Phase 2, Open-label Extension Study. Journal Of Cardiac Failure 2024, 30: 221. DOI: 10.1016/j.cardfail.2023.10.251.Peer-Reviewed Original ResearchOpen-label extensionATTR-CMAmyloid cardiomyopathyPhase 3 randomized clinical trialMedian NT-proBNP levelOpen-label extension studyNYHA class IIMedian follow-upNT-proBNP levelsPhase 2 studyPhase 2 trialTransthyretin amyloid cardiomyopathyNT-pro-BNPOpen-label studyLong-term treatmentLong-term safetyEx vivo assaysDouble-blindPlacebo-controlledNT-proAdverse eventsFollow-upClinical trialsConcurrent illnessDay 1Baseline Characteristics of Patients in SEQUOIA-HCM: A Phase 3 Trial of Aficamten in Obstructive Hypertrophic Cardiomyopathy
Maron M, Abraham T, Coats C, Olivotto I, Lee M, Arad M, Cardim N, Ma C, Choudhury L, Düngen H, Garcia-Pavia P, Hagege A, Lewis G, Michels M, Oreziak A, Owens A, Tfelt-Hansen J, Veselka J, Watkins H, Heitner S, Jacoby D, Kupfer S, Malik F, Meng L, Wohltman A, Masri A. Baseline Characteristics of Patients in SEQUOIA-HCM: A Phase 3 Trial of Aficamten in Obstructive Hypertrophic Cardiomyopathy. Journal Of Cardiac Failure 2024, 30: s3. DOI: 10.1016/j.cardfail.2023.11.006.Peer-Reviewed Original ResearchLeft ventricular outflow tract obstructionObstructive hypertrophic cardiomyopathySymptomatic obstructive hypertrophic cardiomyopathyBaseline characteristics of patientsCardiac myosin inhibitorPhase 3 trialCharacteristics of patientsBaseline characteristicsHypertrophic cardiomyopathyNew York Heart Association functional classVentricular outflow tract obstructionN-terminal pro-B-type natriuretic peptidePro-B-type natriuretic peptideTreated with beta-blockersHigh-sensitivity cardiac troponin IBaseline New York Heart Association functional classOutflow tract obstructionPlacebo-controlled trialBaseline to weekHeart failure symptomsIndividual daily doseReduced exercise capacityCardiac troponin IMyocardial hypercontractilityOral placebo
2023
Long term outcome after septal reduction therapies in patients with hypertrophic cardiomyopathy: insights from the SHaRe registry
Maurizi N, Day S, Ashley E, Michels M, Pereira A, Helms A, Saberi S, Jacoby D, Ware J, Colan S, Semsarian C, Ingles J, Ho C, Olivotto I. Long term outcome after septal reduction therapies in patients with hypertrophic cardiomyopathy: insights from the SHaRe registry. European Heart Journal 2023, 44: ehad655.1852. DOI: 10.1093/eurheartj/ehad655.1852.Peer-Reviewed Original ResearchLong-term outcomesPeri-procedural mortalityAlcohol septal ablationTerm outcomesHF outcomesComposite outcomeLV maximal wall thicknessDilated left atriumSeptal reduction therapyPredictor of prognosisMaximal wall thicknessPost-operative resultsProportional hazards modelMost available studiesLA diameterSeptal ablationVA outcomesHeart failureOverall mortalityLV EFVentricular arrhythmiasHF eventsHCM patientsSarcomeric mutationsSpecialty centers
2022
Correlates of cognition among people with chronic heart failure and insomnia
Geer JH, Jeon S, O’Connell M, Linsky S, Conley S, Hollenbeak CS, Jacoby D, Yaggi HK, Redeker NS. Correlates of cognition among people with chronic heart failure and insomnia. Sleep And Breathing 2022, 27: 1287-1296. PMID: 36214945, PMCID: PMC10084469, DOI: 10.1007/s11325-022-02716-w.Peer-Reviewed Original ResearchConceptsChronic heart failureHigher body mass indexHeart failureBody mass indexQuality of lifeMass indexDaytime sleepinessNew York Heart Association class ICognitive impairmentSignificant risk factorsNon-white raceSelf-reported cognitive impairmentInsomnia Severity IndexSleep disturbance questionnaireCross-sectional analysisCognitive behavioral therapyFrequency of lapsesMedical comorbiditiesCommon comorbiditiesClinical factorsEjection fractionPulmonary diseaseMultivariable analysisRisk factorsSleep characteristicsThe Yale Roadmap for Health Psychology and Integrated Cardiovascular Care
Gaffey AE, Harris KM, Mena-Hurtado C, Sinha R, Jacoby DL, Smolderen KG. The Yale Roadmap for Health Psychology and Integrated Cardiovascular Care. Health Psychology 2022, 41: 779-791. PMID: 35201804, DOI: 10.1037/hea0001152.Peer-Reviewed Original ResearchConceptsCardiovascular careCardiovascular diseaseHealth care systemRisk factorsBehavioural risk factor managementTraditional atherosclerotic risk factorsWagner's Chronic Care ModelCare systemAtherosclerotic risk factorsRisk factor managementChronic Care ModelCause of morbidityMental health burdenBehavioral risk factorsChronic cardiovascular diseaseHigh-intensity careAdverse health outcomesMental health disordersHealth psychologyEvidence-based approachComplex multimorbidityRisk patientsCardiovascular populationFactor managementChronic diseasesPragmatic randomized trial assessing the impact of digital health technology on quality of life in patients with heart failure: Design, rationale and implementation
Victoria‐Castro A, Martin M, Yamamoto Y, Ahmad T, Arora T, Calderon F, Desai N, Gerber B, Lee KA, Jacoby D, Melchinger H, Nguyen A, Shaw M, Simonov M, Williams A, Weinstein J, Wilson FP. Pragmatic randomized trial assessing the impact of digital health technology on quality of life in patients with heart failure: Design, rationale and implementation. Clinical Cardiology 2022, 45: 839-849. PMID: 35822275, PMCID: PMC9346973, DOI: 10.1002/clc.23848.Peer-Reviewed Original ResearchConceptsQuality of lifeHF patientsYale New Haven Health SystemKansas City Cardiomyopathy QuestionnaireDigital health technologiesHeart failure careHealth technologiesDigital health interventionsDisease exacerbationHF clinicUsual carePrimary outcomeClinical outcomesHeart failurePatient engagementPatientsHealth interventionsDigital health toolsPatient empowermentHealth systemPatient usabilityHealth toolsEarly detectionFirst enrollmentHealthcare systemImpact of SARS‐Cov‐2 infection in patients with hypertrophic cardiomyopathy: results of an international multicentre registry
Gimeno JR, Olivotto I, Rodríguez AI, Ho CY, Fernández A, Quiroga A, Espinosa MA, Gómez‐González C, Robledo M, Tojal‐Sierra L, Day SM, Owens A, Barriales‐Villa R, Larrañaga JM, Rodríguez‐Palomares J, González‐del‐Hoyo M, Piqueras‐Flores J, Reza N, Chumakova O, Ashley EA, Parikh V, Wheeler M, Jacoby D, Pereira AC, Saberi S, Helms AS, Villacorta E, Gallego‐Delgado M, de Castro D, Domínguez F, Ripoll‐Vera T, Zorio‐Grima E, Sánchez‐Martínez J, García‐Álvarez A, Arbelo E, Mogollón MV, Fuentes‐Cañamero M, Grande E, Peña C, Monserrat L, Lakdawala NK, Muñoz‐Esparza C, García‐Pinilla J, Robles‐Mezcua A, Moreno‐Flores M, Peña M, Merlo M, Cubillo D, Climent‐Payá V, Dankovtseva E, Vilela A, García‐Pavía P, Casas G. Impact of SARS‐Cov‐2 infection in patients with hypertrophic cardiomyopathy: results of an international multicentre registry. ESC Heart Failure 2022, 9: 2189-2198. PMID: 36255281, PMCID: PMC9288745, DOI: 10.1002/ehf2.13964.Peer-Reviewed Original ResearchConceptsSARS-CoV-2 infectionVentricular outflow tract obstructionOutflow tract obstructionRisk of mortalityHypertrophic cardiomyopathySARS-CoV-2HCM patientsSystolic impairmentTract obstructionOdds ratioBaseline New York Heart Association classNew York Heart Association classLeft ventricular outflow tract obstructionControl groupPresence of HCMBaseline functional classInternational multicentre registryVentricular systolic impairmentSevere clinical courseMulticentre registryNew onsetRespiratory failureAdverse eventsICU careAssociation classAligning mission to digital health strategy in academic medical centers
Cohen AB, Stump L, Krumholz HM, Cartiera M, Jain S, Scott Sussman L, Hsiao A, Lindop W, Ying AK, Kaul RL, Balcezak TJ, Tereffe W, Comerford M, Jacoby D, Navai N. Aligning mission to digital health strategy in academic medical centers. Npj Digital Medicine 2022, 5: 67. PMID: 35654885, PMCID: PMC9163186, DOI: 10.1038/s41746-022-00608-7.Peer-Reviewed Original ResearchSarcomere protein modulation: The new frontier in cardiovascular medicine and beyond
Morelli C, Ingrasciotta G, Jacoby D, Masri A, Olivotto I. Sarcomere protein modulation: The new frontier in cardiovascular medicine and beyond. European Journal Of Internal Medicine 2022, 102: 1-7. PMID: 35534374, DOI: 10.1016/j.ejim.2022.04.020.Peer-Reviewed Original ResearchConceptsHeart failureCardiovascular medicineVentricular outflow obstructionHeart muscle diseaseDegenerative neuromuscular diseaseSpecific disease mechanismsCardiovascular deathOutflow obstructionSystolic functionRandomized trialsUnderlying pathophysiologyCardiac patientsOmecamtiv mecarbilHypertrophic cardiomyopathyFunctional capacityNeuromuscular diseaseMuscle diseaseAvailable evidenceContractile proteinsSkeletal muscleTranslational researchPromising targetDiseaseDisease mechanismsFailureSleep Characteristics, Mood, Somatic Symptoms, and Self-Care Among People With Heart Failure and Insomnia
Breazeale S, Jeon S, Hwang Y, O’Connell M, Nwanaji-Enwerem U, Linsky S, Yaggi HK, Jacoby DL, Conley S, Redeker NS. Sleep Characteristics, Mood, Somatic Symptoms, and Self-Care Among People With Heart Failure and Insomnia. Nursing Research 2022, 71: 189-199. PMID: 35149627, PMCID: PMC9038676, DOI: 10.1097/nnr.0000000000000585.Peer-Reviewed Original ResearchConceptsSelf-care maintenanceStable heart failureSelf-care confidenceVentricular ejection fractionHeart failureChronic insomniaSleep characteristicsSomatic symptomsClinical factorsEjection fractionLeft ventricular ejection fractionChronic heart failureSelf-care behaviorsPoor sleep characteristicsSymptoms of anxietyCross-sectional designSleep disturbancesSingle symptomWhite raceHigh riskCombined associationsInsomnia severityInsomniaSymptomsHealthcare needsSymptom Cluster Profiles Among Adults with Insomnia and Heart Failure
Conley S, Jeon S, Breazeale S, O’Connell M, Hollenbeak CS, Jacoby D, Linsky S, Yaggi HK, Redeker NS. Symptom Cluster Profiles Among Adults with Insomnia and Heart Failure. Behavioral Sleep Medicine 2022, 21: 150-161. PMID: 35388730, PMCID: PMC9537348, DOI: 10.1080/15402002.2022.2060226.Peer-Reviewed Original ResearchConceptsHigh symptom burdenHeart failureSymptom burdenSleep characteristicsLower symptom burdenChronic heart failureStable heart failureBody mass indexTreatment of insomniaStudy of insomniaHigh symptom profileCognitive behavioral therapyBeta blockersClinical characteristicsDaytime symptomsMass indexSevere insomniaSingle symptomInsomnia severitySymptom profilesInsomniaSymptom clustersBehavioral therapyHigher symptomsSymptomsMuscle LIM Protein Force-Sensing Mediates Sarcomeric Biomechanical Signaling in Human Familial Hypertrophic Cardiomyopathy
Riaz M, Park J, Sewanan LR, Ren Y, Schwan J, Das SK, Pomianowski PT, Huang Y, Ellis MW, Luo J, Liu J, Song L, Chen IP, Qiu C, Yazawa M, Tellides G, Hwa J, Young LH, Yang L, Marboe CC, Jacoby DL, Campbell SG, Qyang Y. Muscle LIM Protein Force-Sensing Mediates Sarcomeric Biomechanical Signaling in Human Familial Hypertrophic Cardiomyopathy. Circulation 2022, 145: 1238-1253. PMID: 35384713, PMCID: PMC9109819, DOI: 10.1161/circulationaha.121.056265.Peer-Reviewed Original ResearchConceptsHypertrophic cardiomyopathySarcomeric mutationsFamilial hypertrophic cardiomyopathySudden cardiac deathCardiac myosin heavy chainMechanism-based treatmentsDevelopment of hypertrophyActivated T cellsCalcineurin-nuclear factorForce productionPhenotypic expressionPluripotent stem cell-derived cardiomyocytesStem cell-derived cardiomyocytesHeart failureCardiac deathVentricular hypertrophyCell-derived cardiomyocytesCardiac contractilityPharmacological interventionsT cellsCardiac diseaseCardiac hypertrophyPatient-specific induced pluripotent stem cellsPharmacological meansTwitch relaxationAutomated multilabel diagnosis on electrocardiographic images and signals
Sangha V, Mortazavi BJ, Haimovich AD, Ribeiro AH, Brandt CA, Jacoby DL, Schulz WL, Krumholz HM, Ribeiro ALP, Khera R. Automated multilabel diagnosis on electrocardiographic images and signals. Nature Communications 2022, 13: 1583. PMID: 35332137, PMCID: PMC8948243, DOI: 10.1038/s41467-022-29153-3.Peer-Reviewed Original ResearchConceptsConvolutional neural networkArtificial intelligenceApplication of AISignal-based dataSignal-based modelElectrocardiographic imagesECG imagesGrad-CAMImage-based modelsNeural networkDiagnosis modelECG signalsImagesClinical labelsValidation setLabelsExternal validation setMultilabelIntelligenceNetworkApplicationsModelBroad useSetBroader setting
2021
Risk and predictors of mortality after implantable cardioverter-defibrillator implantation in patients with sarcoid cardiomyopathy
Higgins AY, Annapureddy AR, Wang Y, Minges KE, Bellumkonda L, Lampert R, Rosenfeld LE, Jacoby DL, Curtis JP, Miller EJ, Freeman JV. Risk and predictors of mortality after implantable cardioverter-defibrillator implantation in patients with sarcoid cardiomyopathy. American Heart Journal 2021, 246: 21-31. PMID: 34968442, DOI: 10.1016/j.ahj.2021.12.011.Peer-Reviewed Original ResearchConceptsNon-ischemic cardiomyopathyAtrial fibrillation/flutterChronic lung diseaseCardiac sarcoidosisHeart failureCS patientsNICM patientsICD implantationLung diseaseNew York Heart Association class III heart failureNational Cardiovascular Data Registry ICD RegistryNYHA class IV heart failureClass III heart failureClass IV heart failureImplantable cardioverter defibrillator implantationKaplan-Meier survival curvesCox proportional hazards modelPredictors of mortalityCardioverter-defibrillator implantationVentricular ejection fractionMultivariable logistic regressionPrior ventricular arrhythmiasProportional hazards modelRate of deathTime of implantationThe integration of genetically-regulated transcriptomics and electronic health records highlights a pattern of medical outcomes related to increased hepatic transthyretin expression
Pathak GA, De Lillo A, Wendt FR, De Angelis F, Koller D, Mendoza B, Jacoby D, Miller EJ, Buxbaum JN, Polimanti R. The integration of genetically-regulated transcriptomics and electronic health records highlights a pattern of medical outcomes related to increased hepatic transthyretin expression. Amyloid 2021, 29: 110-119. PMID: 34935565, PMCID: PMC9213571, DOI: 10.1080/13506129.2021.2018678.Peer-Reviewed Original ResearchConceptsGenotype-Tissue Expression (GTEx) projectMulti-tissue analysisElectronic health recordsTTR amyloid formationGenetic regulationExpression projectRNA interferenceTranscriptomic profilesGene expressionExpression informationHepatic expressionHealth outcomesPhenotypic informationAmyloid formationHealth recordsCarpal tunnel syndromeTranscriptionTransthyretin expressionSystemic amyloidosesHepatic transcriptionPathological processesExpressionTunnel syndromeGastrointestinal diseasesSurgical proceduresImpact of Obesity on Heart Transplantation Outcomes
Chouairi F, Milner A, Sen S, Guha A, Stewart J, Jastreboff AM, Mori M, Clark KA, Miller PE, Fuery MA, Rogers JG, Notarianni A, Jacoby D, Maulion C, Anwer M, Geirsson A, Desai NR, Ahmad T, Mullan CW. Impact of Obesity on Heart Transplantation Outcomes. Journal Of The American Heart Association 2021, 10: e021346. PMID: 34854316, PMCID: PMC9075353, DOI: 10.1161/jaha.121.021346.Peer-Reviewed Original ResearchConceptsBody mass indexHigher BMI categoriesImpact of obesityHeart transplantation outcomesBMI categoriesTransplantation outcomesHeart transplantationHigher body mass indexAllocation system changeAdvanced heart failureOrgan Sharing databaseObese BMI categoriesVentricular assist deviceNew Organ ProcurementPosttransplant deathPosttransplantation deathPosttransplant mortalityBackground PatientsBMI 18.5Adult patientsCohort studyHeart failureHeart transplantSharing databaseWaitlist deathsEffect of Mavacamten on Echocardiographic Features in Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy
Hegde SM, Lester SJ, Solomon SD, Michels M, Elliott PM, Nagueh SF, Choudhury L, Zemanek D, Zwas DR, Jacoby D, Wang A, Ho CY, Li W, Sehnert AJ, Olivotto I, Abraham TP. Effect of Mavacamten on Echocardiographic Features in Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy. Journal Of The American College Of Cardiology 2021, 78: 2518-2532. PMID: 34915982, DOI: 10.1016/j.jacc.2021.09.1381.Peer-Reviewed Original ResearchConceptsObstructive hypertrophic cardiomyopathySystolic anterior motionAnterior motionDiastolic functionHypertrophic cardiomyopathyN-terminal pro-B-type natriuretic peptideMitral valve systolic anterior motionPro-B-type natriuretic peptidePeak exercise oxygen consumptionLeft ventricular diastolic functionVentricular outflow tract obstructionKey echocardiographic parametersAtrial volume indexOutflow tract obstructionVentricular diastolic functionExercise oxygen consumptionEffect of mavacamtenMyocardial wall stressEXPLORER-HCMLVOT gradientEchocardiographic featuresEchocardiographic parametersLVOT obstructionTract obstructionExercise capacity