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MANAGEMENT OF HYPERTROPHIC CARDIOMYPATHY

Management of hypertrophic cardiomyopathy

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Page 1: Management of hypertrophic cardiomyopathy

MANAGEMENT OF HYPERTROPHIC CARDIOMYPATHY

Page 2: Management of hypertrophic cardiomyopathy

MANAGEMENT OF HCM

• INTRODUCTION

• MANAGEMENT -INVESTIGATIONS (work up of a clinically suspected case of HCM)

-TREATMENT (medical, surgical and nonsurgical interventions)

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MANAGEMENT OF HCM

DIAGNOSIS• ECG• IMAGING - ECHOCARDIOGRAPHY - CARDIAC MRI - OTHER MODALITIES• CATH DATA• TESTS TO RISK STRATIFY PATIENTS• GENETIC TESTING• FAMILY SCREENING

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MANAGEMENT OF HCM

TREATMENT

• NONOBSTRUCTIVE HCM -MEDICAL MANAGEMENT• OBSTRUCTIVE HCM -MEDICAL MANAGEMENT -SURGICAL MANAGEMENT -NON SURGICAL MANAGEMENT• PREVENTION OF SCD WITH ICDs• END STAGE HCM

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INTRODUCTION“HCM is a disease state characterized by unexplained LV hypertrophy, associated with nondilated ventricular chambers, in the absence of another cardiac or systemic disease that itself would be capable of producing the magnitude of hypertrophy evident in a given patient”

POINTS TO NOTE- •obstruction not necessary, •presence of extra cardiac features goes against the diagnosis, •basis for diagnosis is imaging studies•Genetics not needed to diagnose

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• Prevalence : 1 in 500

• Mendelian inheritance- AD “variable penetrance”

• At least 11 genes and > 1400 mutations- ßMHC, MBPc

• “DYNAMIC OBSTRUCTION”

• Obsolete names- IHSS, HOCM

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MANAGEMENT OF HCM

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MANAGEMENT OF HCM

Criteria • ≥ 15 mm for Adults• > 2 SD for age, sex and height for Children < 12

years• Queries- - Is it necessary to be ‘Diffuse’? -RV involvement? -13 to14 mm, the Grey zone? -can a person have both HTN and HCM? - for children?

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Differential diagnoses

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MANAGEMENT OF HCM

Athlete’s heart

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MANAGEMENT OF HCM

Systemic HTN

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Metabolic disorders

• PRKAG2 MUTATIONS• DANON’S DISEASE-LAMP MUTATION

• NOONAN SYNDROME• INFANTS OF DIABETIC MOTHERS• POMPE’S DISEASE• FRIDERICK’S ATAXIA• FABRY’S DISEASE• LEOPARD SYNDROME

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MANAGEMENT OF HCM

NATURAL HISTORY

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MANAGEMENT OF HCM

DIAGNOSIS• ECG• IMAGING - ECHOCARDIOGRAPHY - CARDIAC MRI - OTHER MODALITIES• CATH DATA• TESTS TO RISK STRATIFY PATIENTS• GENETIC TESTING• FAMILY SCREENING

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ECG

• ST-T CHANGES IN LATERAL PRECORDIAL LEADS• DEEP T WAVE INVERSIONS IN LATERAL LEADS• LAE• DEEP NARROW Q WAVES• ABNORMAL IN 75-95%• NOT RELIABLE FOR LOCALISING/QUANTIFYING

HYPERTROPHY

• TO LOOK FOR WPW PATTERNS, LOW VOLTAGE COMPLEXES

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ECG MIMICKING HCM

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IMAGING

• ECHOCARDIOGRAPHY• CARDIAC MRI• OTHER MODALITIES- -NUCLEAR SCANS, -CT SCANS

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ECHOCARDIOGRAPHY

• M MODE ECHO • 2 D ECHOCARDIOGRAPHY• DOPPLER STUDIES• PROVOCATIVE TESTING - EXERCISE - DRUGS• ROLE OF MYOCARDIAL CONTRAST ECHO• ROLE OF TOE

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MANAGEMENT OF HCM

M MODE ECHO

• SAM• GRADING OF SAM• AORTIC VALVE

FLUTTERING

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MANAGEMENT OF HCM

ECHO GRADING OF SAM

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DOPPLER STUDIES• LATE SYSTOLIC ‘PEAK’ GRADIENTS• PROVOCATION-EXERCISE, NITRATE, VALSALVA

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COLOR DOPPLER

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“THE DAGGER”

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A comprehensive echo evaluation report

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LVEF

• Usually normal or increased • Can have small LV end-diastolic volumes and

therefore reduced stroke volumes despite having normal EFs

• Overt LV systolic dysfunction, termed the ‘‘dilated or progressive phase of HCM,’’ ‘‘end-stage HCM,’’ or ‘‘burnt-out HCM,’’ is usually defined as an LV EF < 50% and occurs in a minority (2%–5%) of patients

• Prognosis is worse in the presence of LV systolic dysfunction

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2D ECHOCARDIOGRAPHY• ≥15 mm AT ANY REGION OF LV• SAM OF AML, THE SEPTAL-MITRAL CONTACT• MITRAL REGURGITATION• LV MASS NOT A NECESSARY CRITERION• BEWARE OF MORPHOLOGICAL SUBTYPES-

APICAL LV HYPERTROPHY, NEUTRAL/REVERSE/SIGMOID HYPERTROPHY OF IVS, MIDCAVITARY HYPERTROPHY

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TO LOOK FOR!oLV DIMENSIONS-NOT BE DILATEDoLA VOLUMESoLV SYSTOLIC FUNCTIONS oDIASTOLIC FUNCTIONSoAORTIC AND SUBAORTIC STENOSISoMV MORPHOLOGY, SEVERITY AND DIRECTION OF MR JEToREGIONAL INVOLVEMENT-SHOULD NOT MISS OUT ON APICAL AND MID CAVITARY HYPERTROPHYoAMYLOIDOSIS, HTN, EXTRA SYSTEMIC FEATURES

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REGIONAL INVOLVEMENT

• ASYMMETRICAL SEPTAL HT MOST COMMON PATTERN

• DIFFUSE HT (70-75%)• BASL SEPTAL HT (10-15%)• CONCENTRIC (5%)• APICAL HT (<5%)• LATERAL WALL HT (2%)

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MITRAL VALVE APPARATUS

• GRADING OF MR• THICKENING OF AML• MV PROLAPSE• COAPTATION• CHORDAL APPARATUS• PAPILLARY MUSCLES

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MANAGEMENT OF HCM

LV DIASTOLIC FUNCTIONS

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MANAGEMENT OF HCM

LV DIASTOLIC DYSFUNCTION

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WHATS NEW IN 2D ECHO!

• MYOCARDIAL DEFORMATION STUDIES-STRAIN ANALYSIS, TISSUE TRACKING, SPECTRAL TRACKING

• CONTRAST ECHO -INTRAPROCEDURAL MYOCARDIAL CONTRAST -LV CONTRAST

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CONTRAST ECHO

• INTRAPROCEDURAL-DURING ASA

• ECHOCONTRAST / AGITATED XRAY CONTRAST• ENSURE SITE AND SIZE OF

INFARCTION• HIGHER SUCCESS RATES,

LESS INFARCT SIZE

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LV contrast echo

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ROLE OF TOE

• CLARIFICATION OF SUSPICIOUS SUBAORTIC MEMBRANE

• STUDY OF MV MORPHOLOGY AND MR

• INTRAOP GUIDANCE FOR MYECTOMY

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TOE

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CARDIAC MRI

• TO CLARIFY THE DIAGNOSIS-INCONCLUSIVE ECHO STUDY

• TO GET ADDITIONAL INFO-MORPHOLOGY AND EXTENT OF THICKNESS

• TO CLARIFY ABNORMALITIES IN MV APPARATUS

• LATE GADOLINIUM ENHANCEMENT- PATTERN OF ENHANCEMENT & SCD RISK STRATIFICATION

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MRI IMAGES

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CARDIAC CT !

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NEW AREAS IN IMAGING

• SPECT SCAN -MYOCARDIAL PERFUSION IMAGING -FIXED DEFECTS -INDUCIBLE DEFECTS ”MICROVASCULAR DYSFUNCTION”

• CT CORONARY ANGIOGRAPHY

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MANAGEMENT OF HCM

SPECT

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Important HP slides

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STRESS TESTING

• TMT -EXERCISE CAPACITY -BP RESPONSE -PROVOCATION FOR DYNAMIC GRADIENT• STRESS ECHO (exercise and not dobutamine) -USED AS A PROVOCATION FOR STUDY OF

GRADIENTS -75 % FALSE POSITIVE RWMA, HENCE NOT USED TO

DETECT/EXCLUDE CAD IN HCM

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STRESS TESTING

• TMT -EXERCISE CAPACITY -BP RESPONSE -PROVOCATION FOR DYNAMIC GRADIENT• STRESS ECHO (exercise and not dobutamine) -USED AS A PROVOCATION FOR STUDY OF

GRADIENTS -75 % FALSE POSITIVE RWMA, HENCE NOT USED TO

DETECT/EXCLUDE CAD IN HCM

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METABOLIC STRESS TESTING

• STUDYING THE OXYGEN AND ENERGY REQUIREMENTS OF MYOCARDIUM AT REST AND CHANGES IN VARIOUS DISEASE STATES LIKE HCM

• VERY EARLY STAGES OF RESEARCH

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ROLE OF AMBULATORY ECG• RISK STRATIFICATION

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CATH DATA• ESTABLISHMENT OF HEMODYNAMICS,

GRADIENTS• PROVOCATIVE TESTING

• ROLE OF LV ANGIOGRAPHY

• ROLE OF CORONARY ANGIOMUST ACCOMPANY CATH STUDY STUDY CORONARY ANATOMY, MYOCARDIAL

BRIDGINGRULE OUT EPICARDIAL CADDONE ‘AFTER’ CATH STUDY

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PROVOCATION

• INCONCLUSIVE/EQUIVOCAL RESULTS IN ECHO• VPCs-BROKENBROUGH PHENOMENON• ISOPRENALINE• NTG/AMYL NITRATE • DOBUTAMINE

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MANAGEMENT OF HCM

RISK STRATIFICATION

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GENETIC TESTING

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DISTINCT ENTITY

• “GENOTYPE POSITIVE-PHENOTYPE NEGATIVE”

• WARRANTS PERIODIC SCREENING FOR LVH

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SCREENING STRATEGY

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TREATMENT

• NONOBSTRUCTIVE HCM -MEDICAL MANAGEMENT• OBSTRUCTIVE HCM -MEDICAL MANAGEMENT -SURGICAL MANAGEMENT -NON SURGICAL MANAGEMENT• PREVENTION OF SCD WITH ICDs• END STAGE HCM

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MEDICAL MANAGEMENT• BETA BLOCKERS• VERAPAMIL• DISOPYRAMIDE

• DIURETICS

• DILTIAZEM, ACE-i/ARBs, STATINS

• NIFEDIPINE, DIGOXIN, INOTROPES

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Beta blockers (class I)

• Drugs of choice- negative inotropic properties and enhanced relaxation—>improved diastolic performance

• Titrate and target maximum doses

• Look for hypotension and AV block

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Titration of beta blockers

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Verapamil (class I) • Add on therapy to beta blockers if high doses of

beta blockers are not tolerated• First choice when beta blockers are

contraindicated • Maximal doses of 280 mg/day• AVOID in NYHA class IV dyspnoea and

hypotension• When used as add-on therapyto look for high

grade AV block

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Rosing DR, Kent KM, Maron BJ, et al. Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy,II: effects on exercise capacity and symptomatic status.Circulation. 1979;60:1208 –13.

Swanton RH, Brooksby IA, Jenkins BS, et al. Hemodynamic studies ofbeta blockade in hypertrophic obstructive cardiomyopathy. EurJ Cardiol. 1977;5:327– 41.

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DISOPYRAMIDE (class II A)

• NOT USED AS A SOLO THERAPY

• ALWAYS AS AN ADD ON THERAPY TO BB AND VERAPAMIL

• INCREASED AV CONDUCTION, PROLONG QT INTERVAL, ANTICHOLINERGIC SIDE EFFECTS

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Diltiazem !

• Less studies to justify use !

• STATINS

• ACE-i/ARBs- -SYSTOLIC DYSFUNCTION

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DRUGS THAN CAN HARM(class III)

• NIFEDIPINE-POTENT VASODILATOR AND HENCE AVOIDED

• DIGOXIN, DOBUTAMINE, NORADRENALINE, DOPAMINE-POSITIVE INOTROPES

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ROLE OF DIURETICS

• USEFUL DRUGS WHEN WE KNOW WHEN TO USE

• AVOIDED IN HOCM WITH PRESERVED SYSTOLIC FUNCTION (class II B)

• CAN BE USED IN HOCM WITH SYSTOLIC DYSFUNCTION (class II B), NONOBSTRUCTIVE HCM (ONLY AS ADD ON THERAPY-class II A)

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MANAGEMENT OF ACUTE HYPOTENSION IN HOCM

• i.v FLUIDS (class I)

• PHENYLEPHRINE (class I)-vasopressor

• Positive inotropes can cause harm (class III)

• MANAGEMENT OF HOCM WITH “DEPRESSED SYSTOLIC FUNCTION” IS A SEPERATE ENTITY

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INTERVENTIONAL STRATEGIES

• SURGICAL REDUCTION

• NON SURGICAL REDUCTION - ALCOHOL SEPTAL ABLATION - DUAL CHAMBER PACING

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ELIGIBILITY CRITERIA

Clinical: Severe dyspnea or chest pain (NYHA functional classes III or IV) / exertional symptoms (such as syncope), despite optimal medical therapyHemodynamic: Dynamic LVOT gradient at rest orwith physiologic provocation 50 mm Hg associated with septal hypertrophy and SAM of the mitral valveAnatomic: Targeted anterior septal thickness sufficient to perform the procedure safely and effectively in the judgment of an experienced operator

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PROVOCATION-EXERCISE, NITRATE, VALSALVA, VPCs, ISOPROTERENOL

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SURGICAL TREATMENT

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MYECTOMY

• MORROW’S PROCEDURE-traditional

• About 3 cm long resection of septum

• Transaortic approach

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EXTENDED MYECTOMY

• Current era-Method of choice• At least 7 cm long resection of septum ±

papillary muscle ± lateral wall done• Resection increases as we move towards apex• Trough created• Potential for mitral valve repair/replacement,

repositioning or resection of papillary muscles

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Better surgical candidates

• Younger age• Greater septal thickness (≥30 mm)• Concomitant cardiac diseases -severe mitral regurgitation/intrinsic MV

pathology -severe papillary muscle hypertrophy -coronary artery disease (CABG planned)

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Outcomes

• Almost total abolition of LVOT gradient and MR

• Technical success 90-95%

• Chances of repeat procedure is very less

• increased treadmill time, maximum workload, peak oxygen consumption, and improved myocardial oxygen demand, metabolism, and coronary flow

• Periop mortality < 1%

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Complications

• VSD <1%

• LBBB, CHB-2 %(pts with preexisting RBBB)

• MV/AV injury < 1%

• SCD risk persists though reduced, SCD risk/ICD discharges < 1%

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SEPTAL ABLATION

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Med Hypotheses. 1994 Sep;43(3):141-4.Percutaneous radiofrequency ablation of the left bundle branch: an alternative modality of treatment for patients with hypertrophic obstructive cardiomyopathy.Dalvi B.SourceDepartment of Cardiology, King Edward VII Memorial Hospital, Parel, Bombay, India

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BACKGROUND

• 1994-SIGWART

• INCREASING NUMBER OF PROCEDURES

• A SERIOUS CHALLENGE TO SEPTAL MYECTOMY IN A SELECT GROUP OF PATIENTS

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SELECTION OF PATIENTS

• ELIGIBILITY CRITERIA• NOT INDICATED IN CHILDREN• THICKNESS 20-30 mm• PREEXISTING LBBB-HIGH RISK• ANATOMY IS THE MOST IMPORTANT (MV,

PAPILLARY MUSCLE)• ‘RIVER-RIVER BED’SEPTAL PERFORATOR

AND ITS TERRITORY

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THE PROCEDURE

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• B-BLOCKER THERAPY SHOULD BE DISCONTINUED AND INTRAVENOUS FLUID BOLUSES AVOIDED IN ORDER TO ALLOW FOR OPTIMAL ASSESSMENT OF THE LVOT GRADIENT

• VERIFICATION OF RESTING/PROVOCABLE GRADIENTS WITH CATH STUDIES

• VERIFICATION OF CORONARIES- CAD, ANATOMY

• TRANS VENOUS PACEMAKER IN SITU LEAST TILL 48 HRS AFTER PROCEDURE

• ASPIRIN AND HEPARIN 50 U/kg

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• ROUTINE GUIDING CATHETERS, PTCA WIRES

• DESIRED 45 DEGREE BEND IN THE PTCA WIRE • SHORT, OVER-THE-WIRE ANGIOPLASTY BALLOON CATHETERS

• MYOCARDIAL CONTRAST ECHO

• ENGAGEMENT OF SEPTAL PERFORATOR OR ANY OF BRANCHES OF SEPTAL PERFORATOR

• BALLOON INFLATIONTEST FOR REDUCTION IN GRADIENTS

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• REMOVAL OF GUIDE WIRE

• ADMINISTRATION OF CORONARY CONTRASTLOOK FOR REFLUX INTO CORONARY CIRCULATION

• 1-3 ML OF 96% ALCOHOL OVER 10 MINUTES TILL DESIRED RESULT

• SEPTAL INFARCTION

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MCE

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VIDEO

Liyakat Ali, 44/M 2013701926

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The haemodynamic objective is a decrease in the gradient to < 10 mmHg at rest in patients with resting gradientsor a decrease by >50% of a provocable gradient

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TRIPHASIC RESPONSE• REDUCTION IN GRADIENT PERSISTS FOR 48 HRS

• MYOCARDIAL EDEMA, STUNNINGRECURRENCE OF GRADIENT

• 3 MONTHSSCAR RETRACTION, SUBSIDING OF EDEMAREDUCTION OF GRADIENT

• 6 MONTHS AND LATERLV AND LA REMODELLING WITH PROGRESSIVE IMPROVEMENT IN ALL PARAMETERS

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TRIPHASIC RESPONSE

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DISCHARGE AT 5 DAYS

• RISK OF CHB IN 10-20% CASES

• CHANCES OF NEW ONSET RBBB, HIGH GRADE AV BLOCK, CHB

• PTS WITH PREEXISITING LBBB

• ON TPI FOR 48 HRS POST PROCEDURE

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OUTCOMES

• TECHNICAL SUCCESS > 90%• PATIENT SELECTION- MOST IMPORTANT STEP• PERIPROCEDURAL MORTALITY 0-4%

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COMPLICATIONS

• CARDIAC TAMPONADE• CORONARY DISSECTION• VSD• AV BLOCK (10-20%)• “CONCERN” FOR LONG TERM ARRYTHMIAS

(4.9%)

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OTHER THAN ALCOHOL!!!• polyvinyl alcohol foam particles,• microspheres, • absorbable gelatin sponges, • septal coils• Gross CM, Schulz-Menger J, Kramer J, Siegel I, Pilz B, Waigand J, Friedrich MG, Uhlich F, Dietz R.

Percutaneous transluminal septal artery ablation using polyvinyl alcohol foam particles for septal hypertrophy in patients with hypertrophic obstructive cardiomyopathy: acute and 3-year outcomes. J Endovasc Ther2004;11:705–711.

• Llamas-Esperon GA, Sandoval-Navarrete S. Percutaneous septal ablation with absorbable gelatin sponge in hypertrophic obstructive cardiomyopathy. Catheter Cardiovasc Interv 2007;69:231–235.

• Lafont A, Durand E, Brasselet C, Mousseaux E, Hagege A, Desnos M. Percutaneous transluminal septal coil embolisation as an alternative to alcohol septal ablation for hypertrophic obstructive cardiomyopathy. Heart2005;91:92

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ASA VS SURGERY

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META ANALYSIS

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“WITH PROPER SELECTION OF PATIENTS AND OPERATOR COMPETENCE AND EXPERIENCE, ASA CAN PROVE AS GOOD AS SURGERY, BUT FOR THE HIGH RATES OF AV BLOCKS”

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DUAL CHAMBER PACING

• CLASS IIb-MAY BE CONSIDERED FOR SUBOPTIMAL CANDIDATES OF SEPTAL REDUCTION THERAPY

• RESIDUAL GRADIENT HIGH• 25-50% DECREASE IN GRADIENT• MUCH OF IMPROVEMENT WAS PROVED TO BE

DUE TO PLACEBO EFFECT, IN RCT ND META ANALYSIS

• OUT OF FAVOUR

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• RV PACINGTIMING OF SEPTAL CONTRACTIONAVOIDING SEPTAL-AML CONTACT

• OTHER UNKNOWN MECHANISMS INVOLVED LEADING TO INADEQUATE RESULT

• OPTIMISATION OF AV DELAY, POSITION OF RV LEAD

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PREVENTION OF SCD

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Selection of ICD type

• Single chamber preferred in children (IIa)- Lead strain and fracture as child grows- Lead extraction difficult- Additional lead placement may lead to venous

obstruction• Dual chamber preferred in (IIa) -Older patients -AF -Heart failure -concomitant LVOT gradients > 50 mmhg

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Results of ICD therapy

• For Class I indication, discharge rates10 % per year

• For Class II indications, discharge rates 4 % per year

• Relative weight of each of risk factors in predicting discharge rate not mentioned

• Number of risk factors not related to discharge rate

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Complications

• Failure rates 0.5-1 % per year• Complications : 4 % per yearEarly-Pneumothorax, Pocket infection, Pocket

hematoma, Pericardial effusion, lead dislodgmentLate-Venous thrombosis, lead dislodgment, infection,

high defibrillation threshold necessitating lead revision, inappropriate shocks-triggered by supraventricular

arrhythmias, sinus tachycardia, lead fracturesor dislodgment, oversensing, double counting, and

programming malfunctions

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Role of CRT

• Paucity of published data on the use of cardiac resynchronization therapy devices in patients with HCM and end-stage heart failure

• Might be useful (level B evidence)

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MANAGEMENT OF HCM WITH LV SYSTOLIC DYSFUNCTION

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• ACEi/ARBs, Diuretics-STANDARD HF TREATMENT (class I)

• DISCONTINUE VERAPAMIL, DILTIAZEM, DISOPYRAMIDE (class III)

• CAD, VALVULAR HEART DISEASE, METABOLIC DISORDERS TO BE RULED OUT

• ICDs TO BE CONSIDERED (class IIB)• ANTICOAGULATION IN PRESENCE OF AF/LV APICAL

ANEURYSMS• HEART TRANSPLANTATION FOR REFRACTORY NYHA

CLASS III/IV SYMPTOMS

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MANAGEMENT OF AF

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• ANTICOAGULATION IRRESPECTIVE OF LV FUNCTION (class I)

• AMIODARONE, DISOPYRAMIDE (class II B)• BETA BLOCKERS, VERAPAMIL, DILTIAZEM (II A)

• RADIOFREQUENCY ABLATION (II A)• MAZE PROCEDURE (II A)

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PREGNANCY/DELIVERY• High risk for- LVOT gradient > 50 mmhg• Class III for those with class III/IV systolic

dysfunction • No added risk for patients with controlled

symptoms (II A)• Continue drugs in prgnancy (class I)-watch or

fetal bradycardia and growth abnormalities in fetus

• Guard against post delivery volume loss

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OCCUPATION

The guidelines state that “irrespective of symptoms, a person should not be certified as a [commercial motor vehicle] driver if a firm diagnosis of HCM is made…”

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PHYSICAL ACTIVITIES

• LOW INTENSITY AEROBIC EXERCISES• AVOID DEHYDRATION• AVOID HEAVY MEALS• RISK OF SYNCOPE IN HIGH INTENSITY SPORTS• UNPREDICTABILITY OF SCDAN OTHER

REASON TO AVOID HIGH INTENSITY SPORTS

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What to expect in future!!!

• ASA OVERTAKING SURGERY

• ALTERNATIVES TO ALCOHOL

• INCLUSION OF CARDIAC MRI IN DIAGNOSIS AND RISK STRATIFICATION

• PRECLINICAL DIAGNOSIS WITH ECHO

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Future Directions

• Refining risk stratification criteria and definitions

• Genetic analysis• Management of AF

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ASA VS SURGERY

• Robust information about the types and frequency of adverse outcomes following alcohol septal ablation are needed

• Rigorous assessment of whether these events are intrinsic to the procedure or related to underlying hypertrophic substrate, concomitant coronary or other comorbid disease, or the advanced age at which patients receive this therapy versus myectomy

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HCM Program

“Every institution to have one such program to deal with the advancements, research needs, maintaining a registry of HCM patients and their follow-up”

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THANK YOU