Management of hypertrophic cardiomyopathy

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  • A FEW IMPORTANT UNDERLYING MECHANISMS IN THE DISEASE PROCESS WILL BE COVERED WHILE GOING THRU THE INVERSTIGATIONS…..
  • Few years back imaging studies ld ve meant only echo, but now…..
  • Most cases not diagnocsed cos of mild symptomology, less of family screening,
    70 % these genes..others inculde trop T,I
    80 names
    UNIQUE ENTITY WHEREIN DYNAMIC OBSTRUCTION –VARIES ACCORDING TO LOADING CONDITONS-PRE AND AFTER AND STATE OF LV CONTRACTILITY…..A RESULT OF VARIOUS MECHANISMS ARE PRESENT
  • HTN- old age, genetics, excessive thickening, family h/o,
    < 12 years children-2 sd age sex height
  • CORRABORATIVE EVIDENCE NEEDED…NO STRICT GUIDELINES FOR THICKNESS LIMITS….AGE, GENETIC ANALYSIS….
  • BETTER BE TERMED SYNDROMES WITH LV HYPERTROPHY RATHER THAN HCM……TERM HCM BE CONFINED TO USE ONLY AS A DISTINCT ENTITY….
  • SUDDEN DEATH
    HEART FAILURE-DIASTOLIC-SYMTPOMS
    END SATGE…
    AF…20-30%
    STABLE MOST…USUAL SYMPTOMS….>.
    CAN BE ALTERED BY A NUMBER OF THERPAEUTIC INETERVENTIONS WHICH IS THE SOLE PURPOSE OF ANY MANAGEMENT STRATEGY…
    PROGNOSIS OF THE DISEASE…..
  • COMING TO THE PROPER OF MY TOPIC…THE FIRST PART-DIAGNOSIS WILL BE COVERED IN THE ORDER OF OUR PRACTICAL WORK UP….FIRST THE SUSPICION IS MADE FROM CLINICAL SYMPTOMS AND EXAMINATION AND BY ORDERING AN ECG….THEN THE DIAGNOSIS IS MADE BY IMAGING STUDIES…EDISGNOSIS IS MAD EBY ECHOCARDIOGRAPHY RISK STARTIFYING PATIENTS FORMING A VERY IMPROTANT PART OF THE WORK UP OF A PATIENT OF HCM….
    GENETUC TESTING AND FAMILY SCREENING FORMING THE LAST PART OF THE WORK UP…
  • WHAT WE ALL KNOW IS..THIS WILL ADD TO THE CLINICAL SUSPICION OF HCM….
    BUT WHAT WE NEED TO CONCENTRATE IS RULING OUT WPW PATTERNS….REASON BEING..
    CANNOT DETERMINE THE SEVERITY WITH ECG…OTHER THAN APICAL VARIANT WE CANNOT DETERMINE THE LOCATION OF HCM;…
  • INCREASED VOLTAGES ARE ONLY WEAKLY CORRELATED WITH MAGNITUDE OF LV HT AND DO NOT DISTINGUISH OBS FROM NON OBS FROMS
  • 3 DANON S DISEASE PATIENTS
  • Venturi effect
  • Distance e-septum and duration of contact
  • WELL ESTABLISHED GUIDELINES
  • Mechanism of mr
  • MITRAL INFLOW VELOCITIES…MECHANISM OF DIASTOLIC DYSFUNCTION-MYO ISCHEMIA AND FIBROSIS, SYSTOLIC OVERLOAD, DELAYED RELAXATION, NONUNIFROM CONTARCTION AND RELAXATION…..MV MORPHOLOGYABNORMAL INSERTION OF PAPILLARY MUSCLE, MVP, FLAIL LEAFLETS—IMP FOR JUDGEMENT OF SURGERY VS NON SURGERY
  • Assessment of LV diastolic function in a patient with HCM with elevated LV end-diastolic pressure but normal LA pressure.
    Mitral inflow shows a short mitral A duration at the level of the mitral annulus, whereas the Ar velocity in pulmonary venous flow is
    increased in amplitude and duration. Lateral annular e0 velocity is normal, and the ratio of peak E velocity (at the level of mitral
    tips) to e0 velocity is <8, consistent with normal LA pressure.
  • Assessment of LV diastolic function in a patient with HCM with elevated LA pressure. Mitral inflow shows a restrictive inflow
    pattern (E velocity, 140 cm/sec). The arrow points to an L velocity in middiastole, which is observed in the presence of impaired relaxation
    and increased filling pressures. Lateral annular and septal annular tissue Doppler (TD) velocities (both e0 and a0) are markedly
    reduced consistent with severely impaired LV relaxation. The markedly increased E/e0 ratio is consistent with increased LA pressure >
    20 mm Hg. The reduced mitral A velocity with its short deceleration time and the severely reduced a0 velocity are consistent with
    increased LV end-diastolic pressure.
  • MAIN PURPOSE OF THE NEW STUDIES WERE TO “QUANTIFY” ABONRMALITIES IN EARLY RELAXATION, DETCTING PRECLINICAL DISEASE, DISTINGUISHING HCM FROM OTHER FORMS OF LV HYPERTROPHY
    APICAL HT MISSED IN 10 % OF ROUTINE ECHOES, LV APICAL ANEURYSMS…ALSO LV CONTRAST…
  • 4 c view
  • Short axis view
  • PERFLUTREN BASED LIPID SPHERES CONTRAST, MECHAICAL INDEX, ADVISED LOW MECHANICAL INDEX TO DELINEATE THE EXACT LOCATION OF SUPPLY
  • EXTENSIVE WORK BEING CARRIED OUT IN THIS AREA….EXTENT OF THICKNESS HAS A LINEAR ASSOCIATION WITH RISK OF DEATH
    6 % PATIENTS WITH NORMAL ECHO BUT CLINICAL SUSPICION OF HCM WENT ON TO SHOW THICKNESS IN LV IN 1 PARTICULAR REGION IN MRI..
  • DEOMONSATRATING LGE
  • DIFFUSE, BASAL SEPTUM, APEX, EXTERME HT, LGE, MID CAVITARY
  • PACEMAKER
  • WITH NUMBER OF IMAGING MODALITIES INCREASING, IT IS OBVIOUS TO TRY TESTING EACH OF THEM TO DIAGNOSIS OF HCM…AND HENCE EVOLVED NUCLEAR SCANA AND CT SCANS….CHEST DISCOMFORT IS A COMMON COMPLAINT IN HCM DUE A NUMBER OF POSTULATED MECHANISMS…ASSOCITAED CAD/MICROVASC. DYSFUNC….IMPORTANT CONCEPT….INDUCIBLE DEFECTS CLD BE SEC TO EPICARDIAL OR MICROVASC DISEASE….COMPLEMENTARY…WHETHER EXTENT OF DEFECTS BOTH FIXED AND INDUCIBLE ARE RISK MARKERS FOR SCD OR NOT IS AN AREA OF EXTENSIVE RESEASRCH…ROLE OF CT ANGIO IS CONFINED TO RULING OUT EPICARDIAL DISEASE….
  • Single photon-emission computed tomographic perfusion imaging from a patient with HCM. Septal (Sep) thickness is increased,
    as is the count activity (hot spot) in the septum relative to lateral (Lat) wall. The computer analysis software registered a fixed
    perfusion defect (scar) (PDS) in the lateral and apical regions upon normalization to the septum.
  • Talk a lot abt the mechanism of various phenomena within hcm
  • MECHANISM OF AUTONOMIC DYSFUNCTION !!!....
  • MECHANISM OF AUTONOMIC DYSFUNCTION !!!....
  • CLINICAL APPLICATION WILL TAKE A LONG TIME BUT CAN BE LOOKED FORWARD TO GIVING NEW INSIGHTS INTO THE PATHOPHYSIOLOGY OF THE DISEASE PROCESS AND POSSIBLE NEW INTERVENTIONS IN THE FORM OF DRUGS OR ANY OTHERS….
  • WILL BE FURTHER DISCUSSED UNDER RISK SRTAIFICATION….
  • LV ANGIO-LV MORPHOLOGY, QUANTIFY MR,
    CORONARY ANGIO SHOULD ACCOMPANY CATH STUDY,
  • THE NEXT STEP AFTER CONVINCINGLY DIAGNOSING HCM IS TO RISK STRATIFY THE PATIENTS….AND THIS IS A VERY IMPORTANT STEP IN THE SENSE THAT WE ARE INETRVENING WITH THE NATURAL HISTORY OF A DISEASE ESPECIALLY THE SCD PART, THIS DISEASE IS OTHERWISE PROVEN TO HAVE A PROGNOSIS SIMILAR TO THAT OF THE GENERAL POPULATION…SO BY RISK STRATIFICATION WE MEAN SCD RISK STARTFICATION…THSE ARE THE VARIOUS VARIABLES STUDIED AND ESTABLISHED AS TARGETS FOR PREVENTION OF SCD…WE OBSERVE THAT A METICULAOUS HISTORY CAN HELP ELUCIDATE THE THE FIRST 3 VARIABLES…..THE DEFINITIONS OF EACH….
    HOLTER TESTING, TMT TSTIN, ECHO IMAGING, MRI IMAGING, CORONARY ANGIOGRAPHY HELP IN ELUCIDATING THE REST….
  • FIRST DEGREE RELATIVES…PRE CLINICAL DISEASE DETECTION AN AREA OF RESEARCH…
  • MEDICAL MANAGEMENT WITH CERTAIN PRECATIONS…NON SURGICAL IS ALMOST SYNONYMOUS WITH ALCOHOL SEPTAL ABLTAION FOR NOW….DUAL CAHMBER PACING BECOME A STARTEGY OF THE PAST….
  • NO ROLE OF ANY DRUGS / INTERVENTIONS IN TREATMENT OF ASYMPTOMATIC PATIENTS REGARDLESS OF PRESENCE OR ABSENCE OR SEVERITY OF OBSTRUCTION….HOWEVER THEY MUST BE SUBJECT TO TESTS FOR RISK STRATIFICATION AND IF FOUND….ICD…..
  • COS HCM WITH AFINCREASED AV CONDUCTION, PROLONG QT INTERVAL, ANTICHOLINERGIC SIDE EFFECTS…WHEN WE ARE EXHAUSTED WITH THE MAXIMUM DOSES OF BB AND VERAPAMIL AND PT STILL NOT RESPONDING….
  • ANIMALM STUDIES SHOWING REGRESSION OF HYPERTROPHY
  • THEN HOW DO WE MANAGE HYPOTENSION IN HOCM….
  • FOR THIS WE NEED TO UNDERSTAND THE PATHOPHYSIOLOGY OF HCM THOROUGHLY
  • Surgical reduction has been the gold standard described in lit….but the procedure has been seriously challenged by the increasing no of ASA being performed world wide….
  • Not for dual chamber pacing…
  • WELL ESTABLISHED GUIDELINES
  • 50 years ago
  • MECHANISM OF DEVELOPMENT OF BBB WILL BE DEALT ALONG WITH ASA WHEREIN ALSO WE LD EXPECT A SIMILAR COMPLICATION…
  • Many alternatives to surgery even in india…in 1994 the same year when…
  • HAVE EXCEEDED THE NUBER OF MYECTOMIES PERFORMED LAST 50 YEARS….ONCE THE OPERATOR KNOWS WHEN AND WHERE TO PERFORM THE PROCEDURE, THEN THE COMPLICATION RATES AND OUTCOMES ARE COMPARABLE TO SURGERY..
  • ARBITRARY…JUDGEMENT OF THE PHYSICIAN….
  • The target septal branch may occasionally
    originate from the left main coronary artery, an intermediate
    or diagonal branch or the posterior descending coronary
    artery.
  • HIGHLY VARIABLE DEPENDING UPON THE OPERATOR EXPERIENCE AND COMPETENCE
  • ARRYTHMOGENIC SUBSTRATE
  • PSTIENTS CLOICE REMAINS IMPORTANT….
  • FOR NOW ACCORDING TO THE LATST GUIDELINES….BUT WE CAN EXPECT ASA TO MOVE UP THE LADDER SOONER THAN LATER,…..AND IT IS TO BE NOTED THAT NO OF ASA IN LAST 15 YARS HAVE OUTNUMBERED THE NUMBER OF SURGERIES DONE IN THE PAST 50 YEASR…
  • OPERATOR
  • INITIAL INTERETS
  • Expalin each…
  • Long term compli.- venous obstruction, lead fracture, growth rates cause strain on leads
    Can pace as well as defibrillate…
  • LVOT OBSTRUCTION, AF, FAMILY H/O ARE RISK FACTORS
  • 20-30% RISK IN HCM, IRREPECTIVE OF LVOT OBS/THICKNESS….LA VOLUMES ARE RISK FACTORS….
  • Examples like golf, bowling,brisk walking… Well defined in acc 2011 guifddlines on what sports are permitted and a=what not…
  • Potential for new information in every field right from genetics to investigation to treatment modalitites…Plenty of areas…Not a bad idea…this will help in diagnosing more no of cases and ll give a direction to advance in this field
  • Not far behind when surgery will be overtaken by abation procedures but ofcourse only in a selct group of patients, subject to anatomy….
  • Not a bad idea….
  • Management of hypertrophic cardiomyopathy

    1. 1. MANAGEMENT OF HYPERTROPHIC CARDIOMYPATHY
    2. 2. MANAGEMENT OF HCM • INTRODUCTION • MANAGEMENT -INVESTIGATIONS (work up of a clinically suspected case of HCM) -TREATMENT (medical, surgical and nonsurgical interventions)
    3. 3. MANAGEMENT OF HCM DIAGNOSIS • ECG • IMAGING - ECHOCARDIOGRAPHY - CARDIAC MRI - OTHER MODALITIES • CATH DATA • TESTS TO RISK STRATIFY PATIENTS • GENETIC TESTING • FAMILY SCREENING
    4. 4. 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
    5. 5. MANAGEMENT OF HCM 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
    6. 6. MANAGEMENT OF HCM • Prevalence : 1 in 500 • Mendelian inheritance- AD “variable penetrance” • At least 11 genes and > 1400 mutations- ßMHC, MBPc • “DYNAMIC OBSTRUCTION” • Obsolete names- IHSS, HOCM
    7. 7. MANAGEMENT OF HCM
    8. 8. 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?
    9. 9. MANAGEMENT OF HCM Differential diagnoses
    10. 10. MANAGEMENT OF HCM Athlete’s heart
    11. 11. MANAGEMENT OF HCM Systemic HTN
    12. 12. MANAGEMENT OF HCM 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
    13. 13. MANAGEMENT OF HCM
    14. 14. MANAGEMENT OF HCM NATURAL HISTORY
    15. 15. MANAGEMENT OF HCM
    16. 16. MANAGEMENT OF HCM DIAGNOSIS • ECG • IMAGING - ECHOCARDIOGRAPHY - CARDIAC MRI - OTHER MODALITIES • CATH DATA • TESTS TO RISK STRATIFY PATIENTS • GENETIC TESTING • FAMILY SCREENING
    17. 17. MANAGEMENT OF HCM 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
    18. 18. MANAGEMENT OF HCM
    19. 19. MANAGEMENT OF HCM
    20. 20. MANAGEMENT OF HCM ECG MIMICKING HCM
    21. 21. MANAGEMENT OF HCM IMAGING • ECHOCARDIOGRAPHY • CARDIAC MRI • OTHER MODALITIES- -NUCLEAR SCANS, -CT SCANS
    22. 22. MANAGEMENT OF HCM ECHOCARDIOGRAPHY • M MODE ECHO • 2 D ECHOCARDIOGRAPHY • DOPPLER STUDIES • PROVOCATIVE TESTING - EXERCISE - DRUGS • ROLE OF MYOCARDIAL CONTRAST ECHO • ROLE OF TOE
    23. 23. MANAGEMENT OF HCM M MODE ECHO • SAM • GRADING OF SAM • AORTIC VALVE FLUTTERING
    24. 24. MANAGEMENT OF HCM ECHO GRADING OF SAM
    25. 25. MANAGEMENT OF HCM
    26. 26. MANAGEMENT OF HCM DOPPLER STUDIES • LATE SYSTOLIC ‘PEAK’ GRADIENTS • PROVOCATION-EXERCISE, NITRATE, VALSALVA
    27. 27. MANAGEMENT OF HCM COLOR DOPPLER
    28. 28. MANAGEMENT OF HCM “THE DAGGER”
    29. 29. MANAGEMENT OF HCM A comprehensive echo evaluation report
    30. 30. MANAGEMENT OF HCM 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
    31. 31. MANAGEMENT OF HCM 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
    32. 32. MANAGEMENT OF HCM TO LOOK FOR! oLV DIMENSIONS-NOT BE DILATED oLA VOLUMES oLV SYSTOLIC FUNCTIONS oDIASTOLIC FUNCTIONS oAORTIC AND SUBAORTIC STENOSIS oMV MORPHOLOGY, SEVERITY AND DIRECTION OF MR JET oREGIONAL INVOLVEMENT-SHOULD NOT MISS OUT ON APICAL AND MID CAVITARY HYPERTROPHY oAMYLOIDOSIS, HTN, EXTRA SYSTEMIC FEATURES
    33. 33. MANAGEMENT OF HCM 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%)
    34. 34. MANAGEMENT OF HCM
    35. 35. MANAGEMENT OF HCM MITRAL VALVE APPARATUS • GRADING OF MR • THICKENING OF AML • MV PROLAPSE • COAPTATION • CHORDAL APPARATUS • PAPILLARY MUSCLES
    36. 36. MANAGEMENT OF HCM
    37. 37. MANAGEMENT OF HCM LV DIASTOLIC FUNCTIONS
    38. 38. MANAGEMENT OF HCM LV DIASTOLIC DYSFUNCTION
    39. 39. MANAGEMENT OF HCM WHATS NEW IN 2D ECHO! • MYOCARDIAL DEFORMATION STUDIES-STRAIN ANALYSIS, TISSUE TRACKING, SPECTRAL TRACKING • CONTRAST ECHO -INTRAPROCEDURAL MYOCARDIAL CONTRAST -LV CONTRAST
    40. 40. MANAGEMENT OF HCM
    41. 41. MANAGEMENT OF HCM
    42. 42. MANAGEMENT OF HCM
    43. 43. MANAGEMENT OF HCM CONTRAST ECHO • INTRAPROCEDURAL- DURING ASA • ECHOCONTRAST / AGITATED XRAY CONTRAST • ENSURE SITE AND SIZE OF INFARCTION • HIGHER SUCCESS RATES, LESS INFARCT SIZE
    44. 44. MANAGEMENT OF HCM LV contrast echo
    45. 45. MANAGEMENT OF HCM ROLE OF TOE • CLARIFICATION OF SUSPICIOUS SUBAORTIC MEMBRANE • STUDY OF MV MORPHOLOGY AND MR • INTRAOP GUIDANCE FOR MYECTOMY
    46. 46. MANAGEMENT OF HCM TOE
    47. 47. MANAGEMENT OF HCM
    48. 48. MANAGEMENT OF HCM 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
    49. 49. MANAGEMENT OF HCM
    50. 50. MANAGEMENT OF HCM MRI IMAGES
    51. 51. MANAGEMENT OF HCM CARDIAC CT !
    52. 52. MANAGEMENT OF HCM NEW AREAS IN IMAGING • SPECT SCAN -MYOCARDIAL PERFUSION IMAGING -FIXED DEFECTS -INDUCIBLE DEFECTS ”MICROVASCULAR DYSFUNCTION” • CT CORONARY ANGIOGRAPHY
    53. 53. MANAGEMENT OF HCM SPECT
    54. 54. MANAGEMENT OF HCM Important HP slides
    55. 55. MANAGEMENT OF HCM 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
    56. 56. MANAGEMENT OF HCM
    57. 57. MANAGEMENT OF HCM 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
    58. 58. MANAGEMENT OF HCM 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
    59. 59. MANAGEMENT OF HCM ROLE OF AMBULATORY ECG • RISK STRATIFICATION
    60. 60. MANAGEMENT OF HCM
    61. 61. MANAGEMENT OF HCM CATH DATA • ESTABLISHMENT OF HEMODYNAMICS, GRADIENTS • PROVOCATIVE TESTING • ROLE OF LV ANGIOGRAPHY • ROLE OF CORONARY ANGIO MUST ACCOMPANY CATH STUDY STUDY CORONARY ANATOMY, MYOCARDIAL BRIDGING RULE OUT EPICARDIAL CAD DONE ‘AFTER’ CATH STUDY
    62. 62. MANAGEMENT OF HCM
    63. 63. MANAGEMENT OF HCM
    64. 64. MANAGEMENT OF HCM PROVOCATION • INCONCLUSIVE/EQUIVOCAL RESULTS IN ECHO • VPCs-BROKENBROUGH PHENOMENON • ISOPRENALINE • NTG/AMYL NITRATE • DOBUTAMINE
    65. 65. MANAGEMENT OF HCM RISK STRATIFICATION
    66. 66. MANAGEMENT OF HCM GENETIC TESTING
    67. 67. MANAGEMENT OF HCM DISTINCT ENTITY • “GENOTYPE POSITIVE-PHENOTYPE NEGATIVE” • WARRANTS PERIODIC SCREENING FOR LVH
    68. 68. MANAGEMENT OF HCM SCREENING STRATEGY
    69. 69. 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
    70. 70. MANAGEMENT OF HCM MEDICAL MANAGEMENT • BETA BLOCKERS • VERAPAMIL • DISOPYRAMIDE • DIURETICS • DILTIAZEM, ACE-i/ARBs, STATINS • NIFEDIPINE, DIGOXIN, INOTROPES
    71. 71. MANAGEMENT OF HCM 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
    72. 72. MANAGEMENT OF HCM Titration of beta blockers
    73. 73. MANAGEMENT OF HCM 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 therapyto look for high grade AV block
    74. 74. MANAGEMENT OF HCM 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 of beta blockade in hypertrophic obstructive cardiomyopathy. Eur J Cardiol. 1977;5:327– 41.
    75. 75. MANAGEMENT OF HCM 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
    76. 76. MANAGEMENT OF HCM Diltiazem ! • Less studies to justify use ! • STATINS • ACE-i/ARBs- -SYSTOLIC DYSFUNCTION
    77. 77. MANAGEMENT OF HCM DRUGS THAN CAN HARM(class III) • NIFEDIPINE-POTENT VASODILATOR AND HENCE AVOIDED • DIGOXIN, DOBUTAMINE, NORADRENALINE, DOPAMINE-POSITIVE INOTROPES
    78. 78. MANAGEMENT OF HCM 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)
    79. 79. MANAGEMENT OF HCM 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
    80. 80. MANAGEMENT OF HCM INTERVENTIONAL STRATEGIES • SURGICAL REDUCTION • NON SURGICAL REDUCTION - ALCOHOL SEPTAL ABLATION - DUAL CHAMBER PACING
    81. 81. MANAGEMENT OF HCM ELIGIBILITY CRITERIA Clinical: Severe dyspnea or chest pain (NYHA functional classes III or IV) / exertional symptoms (such as syncope), despite optimal medical therapy Hemodynamic: Dynamic LVOT gradient at rest or with physiologic provocation 50 mm Hg associated with septal hypertrophy and SAM of the mitral valve Anatomic: Targeted anterior septal thickness sufficient to perform the procedure safely and effectively in the judgment of an experienced operator
    82. 82. MANAGEMENT OF HCM PROVOCATION-EXERCISE, NITRATE, VALSALVA, VPCs, ISOPROTERENOL
    83. 83. MANAGEMENT OF HCM SURGICAL TREATMENT
    84. 84. MANAGEMENT OF HCM MYECTOMY • MORROW’S PROCEDURE-traditional • About 3 cm long resection of septum • Transaortic approach
    85. 85. MANAGEMENT OF HCM
    86. 86. MANAGEMENT OF HCM 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
    87. 87. MANAGEMENT OF HCM
    88. 88. MANAGEMENT OF HCM 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)
    89. 89. MANAGEMENT OF HCM 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%
    90. 90. MANAGEMENT OF HCM Complications • VSD <1% • LBBB, CHB-2 %(pts with preexisting RBBB) • MV/AV injury < 1% • SCD risk persists though reduced, SCD risk/ICD discharges < 1%
    91. 91. MANAGEMENT OF HCM SEPTAL ABLATION
    92. 92. MANAGEMENT OF HCM 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. Source Department of Cardiology, King Edward VII Memorial Hospital, Parel, Bombay, India
    93. 93. MANAGEMENT OF HCM BACKGROUND • 1994-SIGWART • INCREASING NUMBER OF PROCEDURES • A SERIOUS CHALLENGE TO SEPTAL MYECTOMY IN A SELECT GROUP OF PATIENTS
    94. 94. MANAGEMENT OF HCM 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
    95. 95. MANAGEMENT OF HCM THE PROCEDURE
    96. 96. MANAGEMENT OF HCM • 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
    97. 97. MANAGEMENT OF HCM • 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 INFLATIONTEST FOR REDUCTION IN GRADIENTS
    98. 98. MANAGEMENT OF HCM • REMOVAL OF GUIDE WIRE • ADMINISTRATION OF CORONARY CONTRASTLOOK FOR REFLUX INTO CORONARY CIRCULATION • 1-3 ML OF 96% ALCOHOL OVER 10 MINUTES TILL DESIRED RESULT • SEPTAL INFARCTION
    99. 99. MANAGEMENT OF HCM MCE
    100. 100. MANAGEMENT OF HCM
    101. 101. MANAGEMENT OF HCM VIDEO Liyakat Ali, 44/M 2013701926
    102. 102. MANAGEMENT OF HCM The haemodynamic objective is a decrease in the gradient to < 10 mmHg at rest in patients with resting gradients or a decrease by >50% of a provocable gradient
    103. 103. MANAGEMENT OF HCM TRIPHASIC RESPONSE • REDUCTION IN GRADIENT PERSISTS FOR 48 HRS • MYOCARDIAL EDEMA, STUNNINGRECURRENCE OF GRADIENT • 3 MONTHSSCAR RETRACTION, SUBSIDING OF EDEMAREDUCTION OF GRADIENT • 6 MONTHS AND LATERLV AND LA REMODELLING WITH PROGRESSIVE IMPROVEMENT IN ALL PARAMETERS
    104. 104. MANAGEMENT OF HCM TRIPHASIC RESPONSE
    105. 105. MANAGEMENT OF HCM
    106. 106. MANAGEMENT OF HCM 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
    107. 107. MANAGEMENT OF HCM OUTCOMES • TECHNICAL SUCCESS > 90% • PATIENT SELECTION- MOST IMPORTANT STEP • PERIPROCEDURAL MORTALITY 0-4%
    108. 108. MANAGEMENT OF HCM COMPLICATIONS • CARDIAC TAMPONADE • CORONARY DISSECTION • VSD • AV BLOCK (10-20%) • “CONCERN” FOR LONG TERM ARRYTHMIAS (4.9%)
    109. 109. MANAGEMENT OF HCM 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
    110. 110. MANAGEMENT OF HCM ASA VS SURGERY
    111. 111. MANAGEMENT OF HCM
    112. 112. MANAGEMENT OF HCM META ANALYSIS
    113. 113. MANAGEMENT OF HCM
    114. 114. MANAGEMENT OF HCM
    115. 115. MANAGEMENT OF HCM
    116. 116. MANAGEMENT OF HCM “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”
    117. 117. MANAGEMENT OF HCM 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
    118. 118. MANAGEMENT OF HCM • RV PACINGTIMING OF SEPTAL CONTRACTIONAVOIDING SEPTAL-AML CONTACT • OTHER UNKNOWN MECHANISMS INVOLVED LEADING TO INADEQUATE RESULT • OPTIMISATION OF AV DELAY, POSITION OF RV LEAD
    119. 119. MANAGEMENT OF HCM PREVENTION OF SCD
    120. 120. MANAGEMENT OF HCM
    121. 121. MANAGEMENT OF HCM
    122. 122. MANAGEMENT OF HCM
    123. 123. MANAGEMENT OF HCM
    124. 124. MANAGEMENT OF HCM 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
    125. 125. MANAGEMENT OF HCM Results of ICD therapy • For Class I indication, discharge rates10 % 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
    126. 126. MANAGEMENT OF HCM Complications • Failure rates 0.5-1 % per year • Complications : 4 % per year Early-Pneumothorax, Pocket infection, Pocket hematoma, Pericardial effusion, lead dislodgment Late-Venous thrombosis, lead dislodgment, infection, high defibrillation threshold necessitating lead revision, inappropriate shocks-triggered by supraventricular arrhythmias, sinus tachycardia, lead fractures or dislodgment, oversensing, double counting, and programming malfunctions
    127. 127. MANAGEMENT OF HCM 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)
    128. 128. MANAGEMENT OF HCM MANAGEMENT OF HCM WITH LV SYSTOLIC DYSFUNCTION
    129. 129. MANAGEMENT OF HCM • 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
    130. 130. MANAGEMENT OF HCM MANAGEMENT OF AF
    131. 131. MANAGEMENT OF HCM • 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)
    132. 132. MANAGEMENT OF HCM 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
    133. 133. MANAGEMENT OF HCM 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…”
    134. 134. MANAGEMENT OF HCM PHYSICAL ACTIVITIES • LOW INTENSITY AEROBIC EXERCISES • AVOID DEHYDRATION • AVOID HEAVY MEALS • RISK OF SYNCOPE IN HIGH INTENSITY SPORTS • UNPREDICTABILITY OF SCDAN OTHER REASON TO AVOID HIGH INTENSITY SPORTS
    135. 135. MANAGEMENT OF HCM What to expect in future!!! • ASA OVERTAKING SURGERY • ALTERNATIVES TO ALCOHOL • INCLUSION OF CARDIAC MRI IN DIAGNOSIS AND RISK STRATIFICATION • PRECLINICAL DIAGNOSIS WITH ECHO
    136. 136. MANAGEMENT OF HCM Future Directions • Refining risk stratification criteria and definitions • Genetic analysis • Management of AF
    137. 137. MANAGEMENT OF HCM 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
    138. 138. MANAGEMENT OF HCM 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”
    139. 139. MANAGEMENT OF HCM THANK YOU

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