2024
The Clinical Trajectory of NYHA Functional Class I Patients With Obstructive Hypertrophic Cardiomyopathy
Ahluwalia M, Liu J, Olivotto I, Parikh V, Ashley E, Michels M, Ingles J, Lampert R, Stendahl J, Colan S, Abrams D, Pereira A, Rossano J, Ryan T, Owens A, Ware J, Saberi S, Helms A, Day S, Claggett B, Ho C, Lakdawala N. The Clinical Trajectory of NYHA Functional Class I Patients With Obstructive Hypertrophic Cardiomyopathy. JACC Heart Failure 2024, 13: 332-343. PMID: 39520446, DOI: 10.1016/j.jchf.2024.09.008.Peer-Reviewed Original ResearchObstructive hypertrophic cardiomyopathyClass I patientsFunctional class II patientsNYHA functional class IClass II patientsFunctional class II patientsII patientsHypertrophic cardiomyopathyFollow-upLeft ventricular outflow tract obstructionVentricular outflow tract obstructionNYHA functional class IILeft ventricular ejection fractionRates of clinical outcomesClass INatural historyRestricted cubic spline modelsSeptal reduction therapyOutflow tract obstructionFunctional class IIVentricular ejection fractionComposite event rateCubic spline modelsCox proportional hazardsLong-Term Outcomes After Septal Reduction Therapies in Obstructive Hypertrophic Cardiomyopathy: Insights From the SHARE Registry
Maurizi N, Antiochos P, Owens A, Lakdwala N, Saberi S, Russell M, Fumagalli C, Skalidis I, Lin K, Nathan A, De Feria Alsina A, Reza N, Stendahl J, Abrams D, Semsarian C, Clagget B, Lampert R, Wheeler M, Parikh V, Ashley E, Michels M, Rossano J, Ryan T, Ingles J, Ware J, Ho C, Helms A, Day S, Olivotto I. Long-Term Outcomes After Septal Reduction Therapies in Obstructive Hypertrophic Cardiomyopathy: Insights From the SHARE Registry. Circulation 2024, 150: 1377-1390. PMID: 39355918, PMCID: PMC11493522, DOI: 10.1161/circulationaha.124.069378.Peer-Reviewed Original ResearchSeptal reduction therapyHCM-related deathOutflow tract obstructionObstructive hypertrophic cardiomyopathyHypertrophic cardiomyopathyHeart failureComposite outcomeMaximal left ventricular outflow tract gradientRecurrent left ventricular outflow tract obstructionRelief of outflow tract obstructionLeft ventricular outflow tract gradientLeft ventricular outflow tract obstructionReduction therapyVentricular outflow tract obstructionDe novo atrial fibrillationPredictor of adverse outcomesOccurrence of heart failureAlcohol septal ablationOutflow tract gradientEvent-free survivalLong-term disease courseHypertrophic cardiomyopathy centersLong-term reliefCox proportional hazards modelsLong-term outcomesEffect 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 thicknessBaseline 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
Right ventricular outflow tract obstruction associated with neointimal tissue accumulation and distortion of the Harmony TPV25 stent frame: Potential mechanisms and treatment
Steinberg Z, Cabalka A, Balzer D, Asnes J, Morray B, Gillespie M, McElhinney D. Right ventricular outflow tract obstruction associated with neointimal tissue accumulation and distortion of the Harmony TPV25 stent frame: Potential mechanisms and treatment. Catheterization And Cardiovascular Interventions 2023, 102: 1078-1087. PMID: 37925624, DOI: 10.1002/ccd.30901.Peer-Reviewed Original ResearchTissue accumulationStent frameRight ventricular outflow tract repairRight ventricular outflow tract obstructionVentricular outflow tract obstructionBalloon-expandable transcatheter valveOutflow tract obstructionTranscatheter pulmonary valveCircumferential narrowingTract obstructionPulmonary regurgitationTranscatheter valveEarly outcomesPulmonary valvePeak gradientValve implantsPericardial valveValve durabilityPatientsDysfunctionMultiple patientsValve framePotential mechanismsRegurgitationPotential causesIntra-Aortic Balloon Pump Exacerbates Left Ventricular Outflow Tract Obstruction in a Patient With Takotsubo and Hypertrophic Cardiomyopathy
Griffin M, Odanovic N, McNamara R, Altin S, Balan S, Thompson J, Young L. Intra-Aortic Balloon Pump Exacerbates Left Ventricular Outflow Tract Obstruction in a Patient With Takotsubo and Hypertrophic Cardiomyopathy. Case 2023, 7: 502-507. PMID: 38197115, PMCID: PMC10772928, DOI: 10.1016/j.case.2023.07.003.Peer-Reviewed Original Research
2022
Impact 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 class
2021
Effect 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 capacitySuccessful Conservative Treatment of Cardiac Rupture Associated with Takotsubo Syndrome
Mano Y, Baba A, Sukegawa H, Sawano M, Nishiyama T, Ohki T. Successful Conservative Treatment of Cardiac Rupture Associated with Takotsubo Syndrome. Internal Medicine 2021, 60: 2097-2102. PMID: 33551402, PMCID: PMC8313900, DOI: 10.2169/internalmedicine.5831-20.Peer-Reviewed Case Reports and Technical NotesConceptsTakotsubo syndromeCardiac ruptureLeft ventricular outflow tract obstructionVentricular outflow tract obstructionProphylactic anticoagulation therapyOutflow tract obstructionSuccessful conservative treatmentAnticoagulation therapyTract obstructionApical thrombusConservative treatmentCardiac surgeryHemoglobin levelsCareful observationPatientsSyndromeTreatmentRuptureHemopericardiumSurgeryAdmissionThrombusObstructionTherapyWeeksCharacteristics and Outcomes of Elderly Patients With Hypertrophic Cardiomyopathy
Alashi A, Smedira NG, Popovic ZB, Fava A, Thamilarasan M, Kapadia SR, Wierup P, Lever HM, Desai MY. Characteristics and Outcomes of Elderly Patients With Hypertrophic Cardiomyopathy. Journal Of The American Heart Association 2021, 10: e018527. PMID: 33506683, PMCID: PMC7955450, DOI: 10.1161/jaha.120.018527.Peer-Reviewed Original ResearchConceptsLeft ventricular outflow tract gradientVentricular outflow tract gradientOutflow tract gradientElderly patientsHypertrophic cardiomyopathyTract gradientObstructive subgroupRisk factorsDynamic left ventricular outflow tract obstructionSudden cardiac death risk factorsLeft ventricular outflow tract obstructionAppropriate internal defibrillator dischargeVentricular outflow tract obstructionBasal septal thicknessConclusion Elderly patientsDeath risk factorsSeptal reduction therapyThoracic Surgeons scoreConsecutive elderly patientsOutflow tract obstructionVentricular mass indexBasal septal hypertrophyLong-term outcomesHospital deathTract obstruction
2020
Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial
Olivotto I, Oreziak A, Barriales-Villa R, Abraham TP, Masri A, Garcia-Pavia P, Saberi S, Lakdawala NK, Wheeler MT, Owens A, Kubanek M, Wojakowski W, Jensen MK, Gimeno-Blanes J, Afshar K, Myers J, Hegde SM, Solomon SD, Sehnert AJ, Zhang D, Li W, Bhattacharya M, Edelberg JM, Waldman CB, Lester SJ, Wang A, Ho CY, Jacoby D, investigators E, Bartunek J, Bondue A, Van Craenenbroeck E, Kubanek M, Zemanek D, Jensen M, Mogensen J, Thune J, Charron P, Hagege A, Lairez O, Trochu J, Axthelm C, Duengen H, Frey N, Mitrovic V, Preusch M, Schulz-Menger J, Seidler T, Arad M, Halabi M, Katz A, Monakier D, Paz O, Viskin S, Zwas D, Olivotto I, Rocca H, Michels M, Dudek D, Oko-Sarnowska Z, Oreziak A, Wojakowski W, Cardim N, Pereira H, Barriales-Villa R, Pavia P, Blanes J, Urbano R, Diaz L, Elliott P, Yousef Z, Abraham T, Afshar K, Alvarez P, Bach R, Becker R, Choudhury L, Fermin D, Jacoby D, Jefferies J, Kramer C, Lakdawala N, Lester S, Marian A, Masri A, Maurer M, Nagueh S, Owens A, Owens D, Rader F, Saberi S, Sherrid M, Shirani J, Symanski J, Turer A, Wang A, Wever-Pinzon O, Wheeler M, Wong T, Yamani M. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial. The Lancet 2020, 396: 759-769. PMID: 32871100, DOI: 10.1016/s0140-6736(20)31792-x.Peer-Reviewed Original ResearchConceptsObstructive hypertrophic cardiomyopathyKansas City Cardiomyopathy Questionnaire clinical summary scoreSymptomatic obstructive hypertrophic cardiomyopathyHypertrophic cardiomyopathyLVOT gradientNYHA classPrimary endpointDynamic left ventricular outflow tract obstructionNew York Heart Association class IILeft ventricular outflow tract obstructionTreatment-emergent adverse eventsVentricular outflow tract obstructionAvailable pharmacological optionsKey pathophysiological abnormalityNYHA class reductionNYHA functional classOutflow tract obstructionPlacebo-controlled trialPhase 3 trialML/Disease-specific treatmentPeak oxygen consumptionGreater increaseLVOT obstructionPlacebo groupStudy Design and Rationale of EXPLORER-HCM
Ho CY, Olivotto I, Jacoby D, Lester SJ, Roe M, Wang A, Waldman CB, Zhang D, Sehnert AJ, Heitner SB. Study Design and Rationale of EXPLORER-HCM. Circulation Heart Failure 2020, 13: e006853. PMID: 32498620, DOI: 10.1161/circheartfailure.120.006853.Peer-Reviewed Original ResearchConceptsNew York Heart Association classLV outflow tract obstructionObstructive hypertrophic cardiomyopathyOutflow tract obstructionPeak oxygen consumptionEXPLORER-HCMTract obstructionAssociation classEnd pointExercise capacityNew York Heart Association functional class IIDynamic LV outflow tract obstructionKansas City Cardiomyopathy QuestionnaireLV outflow tract gradientSymptomatic obstructive hypertrophic cardiomyopathyUnexplained left ventricular hypertrophyOxygen consumptionExploratory end pointsFunctional class IIOutflow tract gradientPlacebo-controlled trialSecondary end pointsPhase 2 studyPhase 3 trialCurrent medical therapy
2018
Surgical management of left ventricular outflow tract obstruction in a specialized hypertrophic obstructive cardiomyopathy center
Hodges K, Rivas CG, Aguilera J, Borden R, Alashi A, Blackstone EH, Desai MY, Smedira NG. Surgical management of left ventricular outflow tract obstruction in a specialized hypertrophic obstructive cardiomyopathy center. Journal Of Thoracic And Cardiovascular Surgery 2018, 157: 2289-2299. PMID: 30782406, DOI: 10.1016/j.jtcvs.2018.11.148.Peer-Reviewed Original ResearchConceptsVentricular outflow tract obstructionOutflow tract obstructionMitral valve interventionLeft ventricular outflow tract obstructionTract obstructionSeptal myectomyValve interventionMitral interventionPostoperative ventricular septal defectContemporary surgical outcomesHypertrophic cardiomyopathy centersPapillary muscle resectionMitral valve replacementPermanent pacemaker insertionComplete heart blockMitral valve pathologyVentricular septal defectHospital outcomesOperative mortalityConcomitant proceduresValve replacementMuscle resectionSingle surgeonHeart blockRetrospective review
2017
Comparison of valvar and right ventricular function following transcatheter and surgical pulmonary valve replacement
Li WF, Pollard H, Karimi M, Asnes JD, Hellenbrand WE, Shabanova V, Weismann CG. Comparison of valvar and right ventricular function following transcatheter and surgical pulmonary valve replacement. Congenital Heart Disease 2017, 13: 140-146. PMID: 29148206, PMCID: PMC5796854, DOI: 10.1111/chd.12544.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAdultBioprosthesisCardiac CatheterizationChildChild, PreschoolEchocardiographyFemaleFollow-Up StudiesHeart Valve Prosthesis ImplantationHeart VentriclesHumansMaleMiddle AgedPulmonary ValvePulmonary Valve InsufficiencyRetrospective StudiesTime FactorsTreatment OutcomeVentricular Function, RightVentricular RemodelingYoung AdultConceptsRight ventricular outflow tract obstructionPulmonary valve replacementSurgical pulmonary valve replacementRight ventricular functionPulmonic insufficiencyVentricular functionTC groupRV functionSurgical groupValve replacementRV sizeVentricular outflow tract obstructionYale-New Haven HospitalOutflow tract obstructionTract obstructionPatient ageRetrospective reviewSurgical valvesPostoperative declinePulmonary valveHigh riskValve functionPatientsFirst choiceTime points
2015
Extra-Anatomic Right Ventricle to Pulmonary Artery Conduit: The Apical-Aortic Equivalent for Complex Right Ventricular Outflow Tract Reconstruction
Mori M, Siddharthan R, Kogon B. Extra-Anatomic Right Ventricle to Pulmonary Artery Conduit: The Apical-Aortic Equivalent for Complex Right Ventricular Outflow Tract Reconstruction. The Annals Of Thoracic Surgery 2015, 99: 1058-1060. PMID: 25742829, DOI: 10.1016/j.athoracsur.2014.06.011.Peer-Reviewed Original ResearchConceptsVentricular outflow tract obstructionOutflow tract obstructionPulmonary artery conduitArtery conduitTract obstructionRight ventricleComplex left ventricular outflow tract obstructionLeft ventricular outflow tract obstructionRight ventricular outflow tract obstructionRight ventricular outflow tract reconstructionConduit changeVentricular outflow tract reconstructionVentricular septal defect closurePulmonary artery conduit placementExtra-anatomic conduitRight-sided obstructionOutflow tract reconstructionSeptal defect closureTetralogy of FallotEarly stenosisTract reconstructionConduit placementDefect closureComplete repairSuccessful placement
2014
Anomalous Left Coronary Artery in Hypertrophic Cardiomyopathy
Penciu OM, Mojibian H, Sugeng L, Cleman M, Brennan J, DePasquale E, McKenna W, Bonde P, Jacoby D. Anomalous Left Coronary Artery in Hypertrophic Cardiomyopathy. The Annals Of Thoracic Surgery 2014, 97: 2190-2193. PMID: 24882306, DOI: 10.1016/j.athoracsur.2013.07.122.Peer-Reviewed Case Reports and Technical NotesConceptsCoronary arterySignificant left ventricular outflow tract obstructionLeft ventricular outflow tract obstructionVentricular outflow tract obstructionRight ventricular outflow tractSeptal reduction therapyOutflow tract obstructionSingle coronary arteryVentricular outflow tractSudden cardiac deathOpposite sinusAnomalous originTract obstructionRight sinusSeptal myectomyCardiac catheterizationCardiac deathMedical therapyTomography angiogramOutflow tractHypertrophic cardiomyopathyCommon causeReduction therapyImplantable cardioverterArteryManagement of Hypertrophic Cardiomyopathy
Enriquez AD, Goldman ME. Management of Hypertrophic Cardiomyopathy. Annals Of Global Health 2014, 80: 35-45. PMID: 24751563, DOI: 10.1016/j.aogh.2013.12.004.Peer-Reviewed Original ResearchConceptsSudden cardiac deathHypertrophic cardiomyopathyHeart failureAtrial fibrillationHCM patientsManagement of HCMCommon genetic cardiovascular diseaseUnexplained myocardial hypertrophyAlcohol septal ablationOutflow tract obstructionRhythm control strategyPathophysiology of HCMRisk of thromboembolismMajority of patientsGeneral adult populationGenetic cardiovascular diseaseAutosomal dominant diseaseFamily membersSurgical myectomyRefractory symptomsRestrictive physiologySeptal ablationTract obstructionYounger patientsCardiac death
2011
Mutational analysis of JAG1 gene in non-syndromic Tetralogy of Fallot children
Kola S, Koneti NR, Golla JP, Akka J, Gundimeda SD, Mundluru HP. Mutational analysis of JAG1 gene in non-syndromic Tetralogy of Fallot children. Clinica Chimica Acta 2011, 412: 2232-2236. PMID: 21893051, DOI: 10.1016/j.cca.2011.08.017.Peer-Reviewed Original ResearchConceptsTetralogy of FallotTOF patientsRight ventricular outflow tract obstructionVentricular outflow tract obstructionJAG1 geneRight-sided heart diseaseOutflow tract obstructionSided heart diseaseRight ventricular hypertrophyVentricular septal defectSecond epidermal growth factorCell fate decisionsInter-species conservationNon-syndromic tetralogyCell-cell interactionsBidirectional DNA sequencingAortic dextropositionFallot childrenTract obstructionVentricular hypertrophySeptal defectFate decisionsHealthy controlsHeart diseaseIndian cohort
1986
Accessory Mitral Valve Tissue Causing Left Ventricular Outflow Obstruction (Two-Dimensional Echocardiographic Diagnosis and Surgical Approach)
Ascuitto R, Ross-Ascuitto N, Kopf G, Kleinman C, Talner N. Accessory Mitral Valve Tissue Causing Left Ventricular Outflow Obstruction (Two-Dimensional Echocardiographic Diagnosis and Surgical Approach). The Annals Of Thoracic Surgery 1986, 42: 581-584. PMID: 3778009, DOI: 10.1016/s0003-4975(10)60590-5.Peer-Reviewed Original ResearchConceptsAccessory mitral valve tissueVentricular outflow tract obstructionVentricular outflow obstructionOutflow tract obstructionTwo-dimensional echocardiographyMitral valve tissueNative mitral valveDoppler flow analysisTract obstructionOutflow obstructionPreoperative evaluationRare causeMitral valveIntraoperative techniquesValve tissueAccessory tissuesObstructionTissueEchocardiographySurgery
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