2025
Current management of hypertrophic cardiomyopathy
Sikand N, Stendahl J, Sen S, Lampert R, Day S. Current management of hypertrophic cardiomyopathy. The BMJ 2025, 389: e077274. PMID: 40425241, DOI: 10.1136/bmj-2023-077274.Peer-Reviewed Original ResearchConceptsOutflow tract gradientObstructive hypertrophic cardiomyopathyHypertrophic cardiomyopathyAtrial fibrillationLeft ventricular outflow tract gradientNon-obstructive hypertrophic cardiomyopathyManagement of hypertrophic cardiomyopathyRisk of sudden deathCardiac sarcomere genesImplantable cardioverter-defibrillator implantationSeptal reduction therapyNon-obstructive diseaseSymptomatic atrial fibrillationCardiac myosin inhibitorLeft ventricular hypertrophySurgical treatment optionsCardioverter-defibrillator implantationAdvanced heart failureStructural heart conditionsArrhythmic riskCardiac transplantationImprove patient outcomesRhythm controlMedical therapyVentricular hypertrophyConcomitant Aficamten and Disopyramide in Symptomatic Obstructive Hypertrophic Cardiomyopathy
Masri A, Maron M, Abraham T, Nassif M, Barriales-Villa R, Bilen O, Coats C, Elliott P, Garcia-Pavia P, Massera D, Olivotto I, Oreziak A, Owens A, Saberi S, Solomon S, Tower-Rader A, Heitner S, Jacoby D, Melloni C, Wei J, Sherrid M, Olivotto I, Michels M, Barriales-Villa R, Garcia-Pavia P, Abraham T, Choudhury L, Kramer C, Lakdawala N, Maron M, Masri A, Nagueh S, Owens A, Rader F, Saberi S, Sherrid M, Symanski J, Tower-Rader A, Turer A, Wang A, Wong T, Zhang Y, Yang H, Shao C, Yuan Z, Zeng Q, Li X, Ma C, Wang Y, Shu Y, Chen M, Tao L, Li X, Wang J, Yu Z, Cheng X, Hong K, Zemanek D, Bundgaard H, Thune J, Jensen M, Mogensen J, Hagège A, Habib G, Charron P, Lhermusier T, Trochu J, Reant P, Logeart D, Mitrovic V, Bekfani T, Edelman F, Seidler T, Meder B, Schulze P, Stoerk S, Rassaf T, Merkely B, Zfat-Zwas D, Arad M, Halabi M, Paz O, Piltz X, Targetti M, Metra M, Canepa M, Musumeci B, Emdin M, Michels M, Amin A, Knackstedt C, Oreziak A, Wojakowski W, Dudek D, Toste A, Bastos J, Barriales-Villa R, Pavia P, Gimeno Blanes J, Hidalgo Urbano R, Alvarez A, Rincón Diaz L, Ripoll Vera T, Elliott P, Coats C, Cooper R, Mahmod M, Bradlow W, Pantazis A, Tome Esteban M, Masri A, Nassif M, Marian A, Owens D, Wheeler M, McGrew F, Bach R, Wever-Pinzon O, Collado E, Turer A, Hannawi B, Geske J, Owens A, Symanski J, Kramer C, Sarswat N, Ahmad F, Abraham T, Markowitz J, Lakdawala N, Choudhury L, Jani S, Brinkley D, Bilen O, Asher C, Emani S, Sharma A, Fermin D, Lyle M, Raymer D, Darlington A, Maron M, Nielsen C, Wang A, Nagueh S, Martinez M, Desai M, Tower-Rader A, Kelly J, Rader F, Sen S, Bering P, Maurer M, Mitter S, Sherrid M, Wong T, Hussain Z, Saberi S, Naidu S, Enciso J, Zemanek D, Bundgaard H, Jensen M, Mogensen J, Thune J, Donal E, Habib G, Hagege A, Logeart D, Reant P, Trochu J, Brandenburg S, Edelman F, Meder B, Rassaf T, Schulze P, Stoerk S, Merkely B, Arad M, Halabi M, Piltz X, Zfat-Zwas D, Emdin M, Musumeci B, Knackstedt C, Michels M, Oreziak A, Wojakowski W, Bastos J, Toste A, Bradlow W, Coats C, Cooper R, Elliott P, Pantazis A, Raman B, Tome Esteban M, Abraham T, Afshar K, Alsidawi S, Bering P, Bilen O, Brinkley D, Choudhury L, Darlington A, Desai M, Drakos S, Hannawi B, Hussain Z, Kelly J, Kramer C, Lakdawala N, Martinez M, Masri A, Mitter S, Moss N, Nagueh S, Naidu S, Nassif M, Owens A, Rader F, Ravi S, Rowin E, Saberi S, Sen S, Sherrid M, Enciso J, Rader A, Turer A, Wang A, Weiner S, Wong T, Zenker M. Concomitant Aficamten and Disopyramide in Symptomatic Obstructive Hypertrophic Cardiomyopathy. JACC Heart Failure 2025, 102441. PMID: 40285763, DOI: 10.1016/j.jchf.2025.03.008.Peer-Reviewed Original ResearchSymptomatic obstructive hypertrophic cardiomyopathyObstructive hypertrophic cardiomyopathyLeft ventricular outflow tractLVOT obstructionOpen-labelHypertrophic cardiomyopathyObstructive hypertrophic cardiomyopathy patientsPlacebo add-on therapyEfficacy of concomitant useEpisodes of atrial fibrillationVentricular outflow tractAdd-on therapyCardiac myosin inhibitorCohort of patientsNegative inotropic propertiesWorsening of symptomsDisopyramide doseBackground therapyPlacebo-ControlledCombination therapyNT-proBNPOutflow tractAficamtenConcomitant useClinical efficacyAficamten vs Metoprolol for Obstructive Hypertrophic Cardiomyopathy MAPLE-HCM Rationale, Study Design, and Baseline Characteristics
Garcia-Pavia P, Bilen O, Burroughs M, Costabel J, de Barros Correia E, Dybro A, Elliott P, Lakdawala N, Mann A, Nair A, Nassif M, Poulsen S, Reant P, Schulze P, Wang A, Berhane I, Heitner S, Jacoby D, Kupfer S, Malik F, Meng L, Sohn R, Wohltman A, Fifer M, Investigators M. Aficamten vs Metoprolol for Obstructive Hypertrophic Cardiomyopathy MAPLE-HCM Rationale, Study Design, and Baseline Characteristics. JACC Heart Failure 2025, 13: 346-357. PMID: 39909646, DOI: 10.1016/j.jchf.2024.11.011.Peer-Reviewed Original ResearchConceptsObstructive hypertrophic cardiomyopathyStandard-of-careCardiac myosin inhibitorStandard-of-care medicationsBaseline characteristicsSymptomatic obstructive hypertrophic cardiomyopathyNondihydropyridine calcium channel blockersBaseline characteristics of patientsStandard-of-care drugsSecond-line therapyFirst-line therapyCalcium channel blockersCharacteristics of patientsImprove exercise capacityMyosin inhibitorFirst-lineBeta-blockersHypertrophic cardiomyopathyExercise capacityAlleviate symptomsPatientsAficamtenMedicationTherapyStudy design
2024
Aficamten and Cardiopulmonary Exercise Test Performance
Lee M, Masri A, Nassif M, Barriales-Villa R, Abraham T, Claggett B, Coats C, Gimeno J, Kulac I, Landsteiner I, Ma C, Maron M, Olivotto I, Owens A, Solomon S, Veselka J, Jacoby D, Heitner S, Kupfer S, Malik F, Meng L, Wohltman A, Lewis G, Wang A, Sherrid M, Kelly J, Marian A, Owens A, Wever-Pinzon O, Owens D, Wheeler M, Nagueh S, Rader F, McGrew F, Wong T, O'Neill T, Bach R, Martinez M, Lakdawala N, Collado E, Turer A, Desai Y M, Hussain Z, Tower-Rader A, Hannawi B, Geske J, Saberi S, Phelan D, Kramer C, Sarswat N, Ahmad F, Choudhury L, Markowitz J, Sen S, Bering P, Maron M, Jani S, Brinkley D, Naidu S, Maurer M, Moss N, Bilen O, Silva Enciso J, Fraser R, Akinboboye O, Asher C, Emani S, Sharma A, Fermin D, Lyle M, Raymer D, Darlington A, Resnic F, Nielsen C, Metra M, Musumeci B, Emdin M, Targetti M, Canepa M, Michels M, Knackstedt C, Amin A, Barriales Villa R, Garcia Pavia P, Gimeno Blanes J, Hidalgo Urbano R, Rincon Diaz L, Ripoll Vera T, Garcia Alvarez A, Zemanek D, Jensen M, Mogensen J, Thune J, Bundgaard H, Charron P, Trochu J, Habib G, Lhermusier T, Reant P, Hagege A, Logeart D, Mitrovic V, Edelmann F, Seidler T, Meder B, Schulze P, Stoerk S, Bekfani T, Rassaf T, Merkely B, Arad M, Halabi M, Zwas D, Piltz X, Paz O, Habib M, Dudek D, Oreziak A, Wojakowski W, Toste Batista A, Mesquita Bastos J, Elliott P, Mahmod M, Coats C, Cooper R, Bradlow W, Pantazis A, Tome Esteban M, McGinnis S, Campain J, Cocca-Spofford D, Giverts I, Griskowitz C, Newlands C, Moreno F. Aficamten and Cardiopulmonary Exercise Test Performance. JAMA Cardiology 2024, 9: 990-1000. PMID: 39230885, PMCID: PMC11375526, DOI: 10.1001/jamacardio.2024.2781.Peer-Reviewed Original ResearchObstructive hypertrophic cardiomyopathySymptomatic obstructive hypertrophic cardiomyopathyBaseline to weekHypertrophic cardiomyopathyRandomized clinical trialsExercise performanceVentilatory efficiencyClinical trialsPrimary outcomeExercise testPlacebo-corrected improvementCardiac myosin inhibitorPhase 3 trialClinical measuresImpaired exercise capacityCardiopulmonary exercise testingExercise performance measuresHeart rate reserveSubmaximal exercise performancePeak heart rateClinically meaningful thresholdsPlacebo groupDouble-blindPlacebo-ControlledAficamtenImpact of Aficamten on Disease and Symptom Burden in Obstructive Hypertrophic Cardiomyopathy Results From SEQUOIA-HCM
Maron M, Masri A, Nassif M, Barriales-Villa R, Abraham T, Arad M, Cardim N, Choudhury L, Claggett B, Coats C, Düngen H, Garcia-Pavia P, Hagège A, Januzzi J, Kulac I, Lee M, Lewis G, Ma C, Michels M, Oreziak A, Owens A, Spertus J, Solomon S, Tfelt-Hansen J, van Sinttruije M, Veselka J, Watkins H, Jacoby D, Heitner S, Kupfer S, Malik F, Meng L, Wohltman A, Olivotto I, Investigators S. Impact of Aficamten on Disease and Symptom Burden in Obstructive Hypertrophic Cardiomyopathy Results From SEQUOIA-HCM. Journal Of The American College Of Cardiology 2024, 84: 1821-1831. PMID: 39352339, DOI: 10.1016/j.jacc.2024.09.003.Peer-Reviewed Original ResearchObstructive hypertrophic cardiomyopathyN-terminal pro-B-type natriuretic peptidePro-B-type natriuretic peptideSeptal reduction therapyHypertrophic cardiomyopathyNatriuretic peptideReduction therapySymptomatic obstructive hypertrophic cardiomyopathyExercise capacityCardiac myosin inhibitorProportion of patientsTreatment of patientsHemodynamic responseAssociated with substantial improvementsOutflow gradientPlacebo groupAficamtenEnhanced exercise capacityClinical efficacyEfficacy measuresClinical impactPlaceboResponder analysisPatientsCardiomyopathyEffect of Aficamten on Cardiac Structure and Function in Obstructive Hypertrophic Cardiomyopathy SEQUOIA-HCM CMR Substudy
Masri A, Cardoso R, Abraham T, Claggett B, Coats C, Hegde S, Kulac I, Lee M, Maron M, Merkely B, Michels M, Olivotto I, Oreziak A, Jacoby D, Heitner S, Kupfer S, Malik F, Meng L, Solomon S, Wohltman A, Kwong R, Kramer C, Investigators S. Effect of Aficamten on Cardiac Structure and Function in Obstructive Hypertrophic Cardiomyopathy SEQUOIA-HCM CMR Substudy. Journal Of The American College Of Cardiology 2024, 84: 1806-1817. PMID: 39217563, DOI: 10.1016/j.jacc.2024.08.015.Peer-Reviewed Original ResearchCardiovascular magnetic resonanceObstructive hypertrophic cardiomyopathyOutflow tract obstructionReduce cardiovascular eventsCardiac remodelingAtrial fibrillationHeart failureCardiovascular eventsAssociated with progressive heart failureLeft ventricular (LV) hypertrophyPhase 3 double-blindSymptomatic obstructive hypertrophic cardiomyopathyLV outflow tract obstructionMaximal LV wall thicknessLeft atrial volume indexAtrial volume indexLV mass indexLV) hypertrophyPlacebo-controlled trialProgressive heart failureFavorable cardiac remodelingLeft atrial dilatationCardiac myosin inhibitorLeft atrial sizeLV wall thicknessEffect of Aficamten on Health Status Outcomes in Obstructive Hypertrophic Cardiomyopathy Results From SEQUOIA-HCM
Sherrod C, Saberi S, Nassif M, Claggett B, Coats C, Garcia-Pavia P, Januzzi J, Lewis G, Ma C, Maron M, Miao Z, Olivotto I, Veselka J, Butzner M, Jacoby D, Heitner S, Kupfer S, Malik F, Meng L, Wohltman A, Spertus J. Effect of Aficamten on Health Status Outcomes in Obstructive Hypertrophic Cardiomyopathy Results From SEQUOIA-HCM. Journal Of The American College Of Cardiology 2024, 84: 1773-1785. PMID: 39217569, PMCID: PMC11975529, DOI: 10.1016/j.jacc.2024.08.014.Peer-Reviewed Original ResearchKCCQ overall summary scoresKansas City Cardiomyopathy QuestionnaireObstructive hypertrophic cardiomyopathyHealth statusQuality of lifePatient-reported health statusParticipants' health statusHealth status outcomesHypertrophic cardiomyopathyHealth status benefitsHealth status improvementPatients' health statusClinically important changeProportion of participantsSymptoms of fatigueSymptomatic obstructive hypertrophic cardiomyopathyStatus outcomesSummary scoreImprove healthCardiac myosin inhibitorProportion of patientsBaseline scoresImportant changesShortness of breathStatus improvementDosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM
Coats C, Masri A, Nassif M, Barriales‐Villa R, Arad M, Cardim N, Choudhury L, Claggett B, Düngen H, Garcia‐Pavia P, Hagège A, Januzzi J, Lee M, Lewis G, Ma C, Maron M, Miao Z, Michels M, Olivotto I, Oreziak A, Owens A, Spertus J, Solomon S, Tfelt‐Hansen J, van Sinttruije M, Veselka J, Watkins H, Jacoby D, German P, Heitner S, Kupfer S, Lutz J, Malik F, Meng L, Wohltman A, Abraham T, Zhang Y, Yang H, Shao C, Yuan Z, Zeng Q, Li X, Wang Y, Shu Y, Chen M, Tao L, Li X, Wang J, Yu Z, Cheng X, Hong K, Zemanek D, Bundgaard H, Thune J, Jensen M, Mogensen J, Habib G, Charron P, Lhermusier T, Trochu J, Reant P, Logeart D, Mitrovic V, Bekfani T, Edelmann F, Seidler T, Meder B, Schulze P, Stoerk S, Rassaf T, Merkely B, Zfat‐Zwas D, Halabi M, Paz O, Piltz X, Metra M, Canepa M, Musumeci B, Emdin M, Amin A, Knackstedt C, Wojakowski W, Dudek D, Toste A, Bastos J, Blanes J, Urbano R, Alvarez A, Diaz L, Vera T, Elliott P, Glasgow N, Cooper R, Heart L, Mahmod M, Pantazis A, Esteban M, Health O, Marian A, Owens D, McGrew F, Bach R, Wever‐Pinzon O, Collado E, Turer A, Hannawi B, Geske J, Heart P, Symanski J, Heart S, Kramer C, Sarswat N, Ahmad F, Markowitz J, Lakdawala N, Jani S, Brinkley M, Bilen O, Asher C, Emani S, Sharma A, Fermin D, Lyle M, Raymer D, Darlington A, Nielsen C, Wang A, Nagueh S, Martinez M, Desai M, Tower‐Rader A, Kelly J, Heart A, Rader F, Sen S, Bering P, Maurer M, Mitter S, Sherrid M, Wong T, Hussain Z, Saberi S, Naidu S, Enciso J. Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA‐HCM. Journal Of The American Heart Association 2024, 13: e035993. PMID: 39056349, PMCID: PMC11964075, DOI: 10.1161/jaha.124.035993.Peer-Reviewed Original ResearchConceptsLeft ventricular ejection fractionObstructive hypertrophic cardiomyopathyTreatment-emergent adverse eventsHypertrophic cardiomyopathySafety profileAdverse eventsAtrial fibrillationLeft ventricular outflow tract gradientSymptomatic obstructive hypertrophic cardiomyopathyNew-onset atrial fibrillationImplantable cardioverter-defibrillator dischargeOutflow tract gradientCardioverter-defibrillator dischargeVentricular ejection fractionCardiac myosin inhibitorAdverse cardiac eventsLowest effective doseAdverse cardiovascular eventsDose reductionTreatment interruptionEjection fractionAficamtenDosing algorithmBaseline characteristicsCardiac eventsEfficacy and Safety of Aficamten in Symptomatic Nonobstructive 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 Nonobstructive Hypertrophic Cardiomyopathy: Results From the REDWOOD-HCM Trial, Cohort 4. Journal Of Cardiac Failure 2024, 30: 1439-1448. 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 improvementsAre Cardiac Myosin Inhibitors Useful in Patients With Hypertrophic Obstructive Cardiomyopathy and Comorbid Hypertension? ∗
Sikand N, Sen S. Are Cardiac Myosin Inhibitors Useful in Patients With Hypertrophic Obstructive Cardiomyopathy and Comorbid Hypertension? ∗. JACC Heart Failure 2024, 12: 580-582. PMID: 38448152, DOI: 10.1016/j.jchf.2023.12.021.Commentaries, Editorials and LettersBaseline 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
Baseline and Longitudinal Imaging of Hypertrophic Cardiomyopathy in the Era of Emerging Therapeutics
Altibi A, Alani A, Zhao Y, Masri A. Baseline and Longitudinal Imaging of Hypertrophic Cardiomyopathy in the Era of Emerging Therapeutics. Current Cardiology Reports 2023, 25: 583-595. PMID: 37103749, DOI: 10.1007/s11886-023-01883-w.Peer-Reviewed Original ResearchConceptsCardiac myosin inhibitorCardiac magnetic resonance imagingHypertrophic cardiomyopathyCare of patientsClinical trialsPathophysiology of hypertrophic cardiomyopathyCardiac magnetic resonanceNew drug therapiesHypertrophic cardiomyopathy diagnosisActin-myosin cross-bridgesLongitudinal imagingMagnetic resonance imagingMyosin inhibitorTherapeutic optionsAbstract PurposeDrug therapyOral moleculesLongitudinal imaging modalityCardiomyopathyPatientsMonitoring PatientsImaging modalitiesResonance imagingInvestigate new drug therapiesSarcomere level
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