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  • Heart FailureHeart FailureCardiomyopathyCardiomyopathy

    Sudden Cardiac DeathSudden Cardiac Death

    Dr Deirdre WardDr Deirdre WardConsultant CardiologistConsultant Cardiologist

  • Heart failureHeart failureBroad descriptive term which includes:Broad descriptive term which includes:

    Left ventricular systolic impairmentLeft ventricular systolic impairmentLeft ventricular diastolic impairmentLeft ventricular diastolic impairmentBiventricular systolic impairmentBiventricular systolic impairmentRight ventricular systolic impairmentRight ventricular systolic impairment

    Prevalence Prevalence approx 1% total populationapprox 1% total population44--5% of population > 70 years5% of population > 70 years> 40,000 patients > 40,000 patients Prevalence probably increasing as mortality from Prevalence probably increasing as mortality from heart disease reducesheart disease reducesMost common discharge diagnosis in > 65s in USAMost common discharge diagnosis in > 65s in USA

  • Heart Failure Heart Failure -- OutcomeOutcome

    Annual mortality is 15Annual mortality is 15--20%20%From time of diagnosis 50% 5 year From time of diagnosis 50% 5 year survivalsurvivalMorbidity highMorbidity high

    Of those discharged from hospital with heart Of those discharged from hospital with heart failure 30% will be readmitted in 3 monthsfailure 30% will be readmitted in 3 months

  • Causes of Heart FailureCauses of Heart FailureIschaemic heart diseaseIschaemic heart diseaseValvular heart diseaseValvular heart diseaseHypertensionHypertensionInherited cardiomyopathyInherited cardiomyopathyCongenital heart diseaseCongenital heart diseaseInfectionInfection

    Myocarditis, endocarditis, rheumatic feverMyocarditis, endocarditis, rheumatic feverPericardial diseasePericardial diseaseInfiltrative (Infiltrative (sarcoidsarcoid, TB, haemochromatosis, metastatic , TB, haemochromatosis, metastatic cancers) cancers) Other : Other :

    TachycardiaTachycardiaPeriPeri--partumpartumCOPD / OSACOPD / OSA

  • Symptoms of heart failureSymptoms of heart failure

    SymptomsSymptomsFatigueFatigueShortness of breath on exertionShortness of breath on exertionOrthopnoeaOrthopnoeaParoxysmal nocturnal dyspnoeaParoxysmal nocturnal dyspnoeaPalpitationsPalpitationsDizinessDiziness, syncope, syncopeAngina Angina

  • NYHA Classification of SymptomsNYHA Classification of Symptoms

    Class I Class I no limitation of physical activityno limitation of physical activityClass II Class II slight limitation of physical slight limitation of physical activity, comfortable at restactivity, comfortable at restClass III Class III marked limitation of physical marked limitation of physical activity, comfortable at restactivity, comfortable at restClass IV Class IV symptoms at restsymptoms at rest

  • Signs of left heart failureSigns of left heart failure

    Gallop rhythmGallop rhythmLung Lung crepitationscrepitationsDisplaced apex beatDisplaced apex beatPleural effusionsPleural effusionsSigns of underlying causeSigns of underlying cause

    Valvular heart disease, hypertension etcValvular heart disease, hypertension etc

  • Signs of right heart failureSigns of right heart failure

    Elevated JVPElevated JVPAnkle oedema (JVP should be up 4cms)Ankle oedema (JVP should be up 4cms)

    Cave venous insufficiency, Ca antagonist RxCave venous insufficiency, Ca antagonist Rx

    AscitesAscites

  • Diagnosis of Heart FailureDiagnosis of Heart FailureClinical historyClinical historyECGECG

    Often nonOften non--specific changes or fairly normalspecific changes or fairly normalPrevious MI, acute ischaemic changesPrevious MI, acute ischaemic changesHypertensive changes or cardiomyopathyHypertensive changes or cardiomyopathyTachyarrhythmia Tachyarrhythmia

  • Echo in Heart failureEcho in Heart failure

    LV sizeLV sizeSystolic functionSystolic functionRegional Regional vsvs global wall motion abnormalityglobal wall motion abnormalityValvular heart diseaseValvular heart diseaseLV diastolic functionLV diastolic function

    Mitral valve inflowMitral valve inflowPulmonary vein flowPulmonary vein flowTissue Doppler velocityTissue Doppler velocity

    Right heart size and functionRight heart size and function

  • Other investigationsOther investigations

    Chest XChest X--rayrayBlood testsBlood tests

    BNP (proBNP (pro--BNP, BNP, NTproBNPNTproBNP))AnaemiaAnaemiaThyroid function testsThyroid function testsRenal profileRenal profileDiabetes Diabetes Liver profile, coagulation (right heart failure)Liver profile, coagulation (right heart failure)

    Coronary angiographyCoronary angiography? Viral screen, family evaluation etc? Viral screen, family evaluation etc

  • Management Management systolic impairmentsystolic impairmentAcute management Acute management stable patientstable patient

    OxygenOxygenDiuretic therapy (loop +/Diuretic therapy (loop +/-- thiazidethiazide))Treat any treatable causes (ischaemia, Treat any treatable causes (ischaemia, TFTsTFTs anaemia)anaemia)ACE inhibitorACE inhibitor+/+/-- morphine and nitratemorphine and nitrate

    Acute management Acute management unstable patientunstable patientPositive airway pressure oxygenPositive airway pressure oxygenDiureticDiuretic+/+/-- morphine and nitratemorphine and nitrateConsider intubation and ventilationConsider intubation and ventilation

  • LongLong--term management term management systolic systolic impairmentimpairment

    Symptom reliefSymptom reliefDiuretics Diuretics loop +/loop +/-- thiazidethiazideDigoxinDigoxin even in sinus rhythmeven in sinus rhythm

    Prognostic benefitPrognostic benefitACE inhibitors (ACE inhibitors (uptitrateuptitrate slowly)slowly)BetaBeta--blockers (start after diuretics reduced or blockers (start after diuretics reduced or withdrawn, withdrawn, uptitrateuptitrate gradually)gradually)AldosteroneAldosterone antagonistantagonistAngiotensinAngiotensin receptor blockers (metareceptor blockers (meta--analysis analysis suggests not in combination with ACE i)suggests not in combination with ACE i)HydralazineHydralazine and nitratesand nitrates

  • LongLong--term management term management other other issuesissues

    Diastolic dysfunctionDiastolic dysfunctionSymptomatic improvement with diuretic therapySymptomatic improvement with diuretic therapyCaution not to overCaution not to over--diuresediureseNo drug therapy of proven prognostic benefitNo drug therapy of proven prognostic benefit

    Right heart failureRight heart failureDiuretic therapy Diuretic therapy -- spironolactonespironolactoneRemove exacerbating elements (eg OSA)Remove exacerbating elements (eg OSA)Pleural / Pleural / asciticascitic taptap

  • NonNon--pharmacological Rxpharmacological Rx

    ExerciseExerciseRestrict salt intakeRestrict salt intake? Reduce fluid intake (controversial)? Reduce fluid intake (controversial)Reduce excessive weightReduce excessive weightHeart failure clinicsHeart failure clinics

  • Invasive therapyInvasive therapy

    Biventricular pacemaker (CRT)Biventricular pacemaker (CRT)Broad QRSBroad QRSNYHA Class III or IV (? Less now)NYHA Class III or IV (? Less now)dyssynchronydyssynchrony echoecho

    ICD ICD often with aboveoften with aboveEF < 30EF < 30--35% after 4 (or 12) weeks optimal therapy35% after 4 (or 12) weeks optimal therapy

    LVADLVADCardiac TransplantationCardiac Transplantation

  • Cardiac resynchronisation therapyCardiac resynchronisation therapy

  • Inherited cardiomyopathyInherited cardiomyopathy

    Sarcomeremutation

    Nuclear envelopeCytoskeletonSarcomere

    Cell adhesion gene

  • PrevalencePrevalence Estimated noEstimated noaaffected in Irelandffected in Ireland

    HCM 1:500HCM 1:500 9,0009,000ARVC 1:1,000 ARVC 1:1,000 -- 10,00010,000 900900DCMDCM 1:3,000 1:3,000 -- 5,0005,000 900900--15001500

  • HCM HCM under a microscopeunder a microscope

  • Genetic causesGenetic causes

  • Genetics of HCMGenetics of HCM

    90 % cases are Autosomal Dominant90 % cases are Autosomal DominantAutosomal = not on X or Y chromosomesAutosomal = not on X or Y chromosomesDominant = only one Dominant = only one abnormalabnormal copy of gene copy of gene required to cause conditionrequired to cause condition50 % chance of affected person passing on 50 % chance of affected person passing on abnormalabnormal gene to each of their childrengene to each of their children

    Incomplete penetranceIncomplete penetranceNot everyone who inherits gene will develop Not everyone who inherits gene will develop symptoms and signs of the conditionsymptoms and signs of the condition

    10 % 10 % SporadicSporadic ie no family history of HCMie no family history of HCM

  • How do people presentHow do people present

    Symptoms :Symptoms :Chest painChest painShortness of breathShortness of breathPalpitationsPalpitationsPrePre--syncope / syncopesyncope / syncopeCardiac arrestCardiac arrest

    No symptoms :No symptoms :Incidental findingIncidental findingFamily screeningFamily screening

  • How is HCM diagnosedHow is HCM diagnosed

    HistoryHistoryPhysical exam (Physical exam (jerkyjerky pulse, systolic murmur left pulse, systolic murmur left sternal edge)sternal edge)ECG (LVH +/ECG (LVH +/-- ST segment and TST segment and T--wave changes)wave changes)Transthoracic EchoTransthoracic Echo+/+/-- Cardiopulmonary exercise test Cardiopulmonary exercise test +/+/-- MRIMRI+/+/-- Genetic testingGenetic testing

  • Complications of HCMComplications of HCM

    NoneNoneChest pain Chest pain LVOT obstructionLVOT obstructionAtrial fibrillationAtrial fibrillationThromboThrombo--embolic events (strokes)embolic events (strokes)Heart failureHeart failureCardiac arrestCardiac arrest

  • Management of HCMManagement of HCM

    Symptom controlSymptom controlBB--blockersblockersVerapamilVerapamilNegative Negative inotropesinotropes (LVOT obstruction)(LVOT obstruction)Heart failure therapyHeart failure therapyAnticoagulation Anticoagulation Septal reduction therapy (alcohol septal ablation Septal reduction therapy (alcohol septal ablation vsvssurgical myectomy)surgical myectomy)TransplantTransplant

  • HCM ManagementHCM Management

    Genetic aspectsGenetic aspectsFirst degree relatives evaluated with ECG and First degree relatives evaluated with ECG and EchoEcho1212--18 year18 year--old annuallyold annuallyThereafter 2Thereafter 2--5 yearly5 yearlyGenetic testingGenetic testing

    Sudden death riskSudden death risk

  • Percentage SCD

    in HCM

    1.4%

    Causes of SCDin the Young

    50 % HCM

    Magnitude of Sudden Cardiac Death in HCM

  • Who is at risk?Who is at risk?Features that may indicate increased risk :Features that may indicate increased risk :

    Previous cardiac arrestPrevious cardiac arrestFamily history premature sudden deathFamily history premature sudden deathUnexplained blackoutsUnexplained blackoutsAbnormal rhythm on exercise test or Holter monitor Abnormal rhythm on exercise test or Holter monitor (even 3 beats of ventricular tachycardia)(even 3 beats of ventricular tachycardia)Blood pressure fails to rise normally with exerciseBlood pressure fails to rise normally with exerciseSevere thickening of the heart (>3 cms or almost 3x Severe thickening of the heart (>3 cms or almost 3x normal)normal)

    Some more significant at younger ageSome more significant at younger agePresence of LVOTO may also increase riskPresence of LVOTO may also increase riskPresence of fibrosis as indicated by late Presence of fibrosis as indicated by late enhancement post contrast on cardiac MRIenhancement post contrast on cardiac MRI

  • Inherited DCMInherited DCM

    77--25% of 25% of IdiopathicIdiopathic DCM may be familialDCM may be familialSometimes associated with more Sometimes associated with more generalised generalised myopathiesmyopathies (eg muscular (eg muscular dystrophies)dystrophies)Genetic causes may exceed 100 genesGenetic causes may exceed 100 genesPresence of conduction disease may Presence of conduction disease may suggest specific subtypessuggest specific subtypesManagement same as for heart failureManagement same as for heart failure

  • Arrhythmogenic right ventricular Arrhythmogenic right ventricular cardiomyopathy (ARVC)cardiomyopathy (ARVC)

    Commonest cause of SCD in athletes in Commonest cause of SCD in athletes in Northern Italy.Northern Italy.Prevalence 1:1000 (or 1 in 5000).Prevalence 1:1000 (or 1 in 5000).Familial in 30Familial in 30--40% of cases (or > 90%)40% of cases (or > 90%)Fibrofatty replacement of the right Fibrofatty replacement of the right ventricle.ventricle.Fatal ventricular arrhythmias of right Fatal ventricular arrhythmias of right ventricular origin.ventricular origin.

  • Treatment of ARVCTreatment of ARVC

    Asymptomatic, normal pump function, no Asymptomatic, normal pump function, no abnormal rhythms detected : No abnormal rhythms detected : No TreatmentTreatmentPalpitations : BetaPalpitations : Beta--blockers or Amiodaroneblockers or AmiodaroneReduced pump function : ACE inhibitors, Reduced pump function : ACE inhibitors, SpironolactoneSpironolactone, diuretics etc, diuretics etcHigh risk : Implantable defibrillatorHigh risk : Implantable defibrillatorEndEnd--stage pump failure : transplantstage pump failure : transplant

  • Sudden Cardiac DeathSudden Cardiac DeathSudden Cardiac Death = Sudden Cardiac Death = deathdeath from from definite or probable cardiac causes within definite or probable cardiac causes within 1 hour of symptom onset1 hour of symptom onsetIncidence from International StudiesIncidence from International Studies

    1 to 3 per 100,000 in those 1 to 35 yrs of age1 to 3 per 100,000 in those 1 to 35 yrs of age10 to 75 per 100,000 in those 35 to 64 yrs10 to 75 per 100,000 in those 35 to 64 yrs

    Incidence in Ireland unknownIncidence in Ireland unknownExtrapolation from other studies suggestExtrapolation from other studies suggest

    > 5,000 SCD annually > 5,000 SCD annually RoIRoI, >2000 NI , >2000 NI > 60 deaths < 35 yrs (> 60 deaths < 35 yrs (RoIRoI), >25 (NI)), >25 (NI)

  • Causes of SCDCauses of SCD

    Over 35 yrs of age, Coronary Heart Over 35 yrs of age, Coronary Heart Disease is most common causeDisease is most common causeUnder 35 yrs Under 35 yrs

    CardiomyopathiesCardiomyopathiesCongenital Heart DiseaseCongenital Heart DiseaseStructurally Normal HeartStructurally Normal Heart (ion channel (ion channel disorders, conduction disease)disorders, conduction disease)Anomalous coronariesAnomalous coronaries

  • Sudden Adult Death SyndromeSudden Adult Death Syndrome

    Cause not apparent on PMCause not apparent on PMCave potentially spurious causesCave potentially spurious causes

    NonNon--obstructive coronary disease with no obstructive coronary disease with no infarctinfarctLVHLVH with normal heart weightwith normal heart weightsudden death in epilepsysudden death in epilepsy

    40% of families have inherited cause 40% of families have inherited cause identified (mostly LQT and Brugada)identified (mostly LQT and Brugada)

  • Long QT syndromeLong QT syndrome

    Inherited form 1 in 5000Inherited form 1 in 5000Up to 7 genes associatedUp to 7 genes associatedUp to 75 % have LQT1(35Up to 75 % have LQT1(35--40%),2 (3040%),2 (30--35) or 3 35) or 3 (10%)(10%)Patients present with syncope or sudden death Patients present with syncope or sudden death due to polymorphic ventricular tachycardia due to polymorphic ventricular tachycardia ((torsadestorsades de pointes)de pointes)LQT1 events occur with exercise or emotionLQT1 events occur with exercise or emotion

    swimmingswimmingLQT2 events occur with LQT2 events occur with startlestartleLQT3 events occur at rest or during sleepLQT3 events occur at rest or during sleep

  • Management LQTManagement LQT

    Avoid precipitantsAvoid precipitantsMedicationsMedicationsStimulantsStimulantsExercise (LQT types 1 and 2)Exercise (LQT types 1 and 2)

    BB--blockersblockersLQT type 1 and 2LQT type 1 and 2

    ICDICD

  • Brugada SyndromeBrugada Syndrome

    Prevalence unknownPrevalence unknownMay be significant regional variationMay be significant regional variationAssociation of incomplete RBBB in right Association of incomplete RBBB in right precordial leads with ST segment elevation and precordial leads with ST segment elevation and sudden deathsudden deathConcealedConcealed cases may be unmasked by cases may be unmasked by provocation testsprovocation testsManagementManagement

    At risk if syncope or spontaneously abnormal ECGAt risk if syncope or spontaneously abnormal ECGICD currently only available treatmentICD currently only available treatment

  • Other causes SCDOther causes SCD

    MyocarditisMyocarditisCatecholaminergicCatecholaminergic Polymorphic VTPolymorphic VTCongenital heart diseaseCongenital heart diseaseQuestionable causesQuestionable causes

    Anomalous coronariesAnomalous coronariesMVPMVPSudden death in epilepsySudden death in epilepsy

  • SummarySummary

    Heart failure widespread Heart failure widespread Prognosis still poor, but intensive Prognosis still poor, but intensive management improvesmanagement improvesSCD in the young is rare but causes SCD in the young is rare but causes underunder--recognisedrecognisedSpecialist centre for evaluation of and Specialist centre for evaluation of and management of those at risk in Tallaghtmanagement of those at risk in Tallaght

    Made possible by your hard work!! Made possible by your hard work!!