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Hypertrophic Hypertrophic CardiomyopathyCardiomyopathy
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Learning Objectives Learning Objectives Understand the prevalence of hypertrophic cardiomyopathy and Understand the prevalence of hypertrophic cardiomyopathy and
the genetic basis for the disease the genetic basis for the disease Appreciate the pathophysiology of hypertrophic cardiomyopathy Appreciate the pathophysiology of hypertrophic cardiomyopathy Be able to identify the various clinical presentations of the Be able to identify the various clinical presentations of the
disease disease Learn how to diagnose hypertrophic cardiomyopathy using Learn how to diagnose hypertrophic cardiomyopathy using
different modalities different modalities Understand the typical natural history of the disease Understand the typical natural history of the disease Recognize the importance of implantable cardioverter Recognize the importance of implantable cardioverter
defibrillators in the prevention of sudden cardiac death in this defibrillators in the prevention of sudden cardiac death in this population population
Appreciate emerging therapeutic strategies and current Appreciate emerging therapeutic strategies and current research research
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CaseCase17 yo male professional basketball player with no 17 yo male professional basketball player with no known PMH collapses on the playing floor during known PMH collapses on the playing floor during practice and subsequently arrests. He had been practice and subsequently arrests. He had been having some exertional dyspnea for a few months having some exertional dyspnea for a few months prior to this incident but it did not affect his activity prior to this incident but it did not affect his activity level. He was told growing up that he had a “heart level. He was told growing up that he had a “heart murmur” that was never formally investigated. He murmur” that was never formally investigated. He was taking no medications, and there was no family was taking no medications, and there was no family history of cardiac disease in his family. An autopsy history of cardiac disease in his family. An autopsy later revealed that the patient had hypertrophic later revealed that the patient had hypertrophic cardiomyopathy. cardiomyopathy.
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Background Background
Prevalence of HCM: 1:500 to 1:1000 Prevalence of HCM: 1:500 to 1:1000 individuals individuals
This occurrence is higher than previously thought, This occurrence is higher than previously thought, suggesting a large number of affected but undiagnosed suggesting a large number of affected but undiagnosed people people
Men and African-Americans affected by almost Men and African-Americans affected by almost 2:1 ratio over women and Caucasians 2:1 ratio over women and Caucasians
Global disease with most cases reported from Global disease with most cases reported from USA, Canada, Western Europe, Israel, & Asia USA, Canada, Western Europe, Israel, & Asia
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Historical Perspective Historical Perspective
HCM was initially described by Teare in HCM was initially described by Teare in 1958 1958
Found massive hypertrophy of ventricular septum in Found massive hypertrophy of ventricular septum in small cohort of young patients who died suddenly small cohort of young patients who died suddenly
Braunwald was the first to diagnose HCM Braunwald was the first to diagnose HCM clinically in the 1960s clinically in the 1960s
Many names for the disease Many names for the disease Idiopathic hypertrophic subaortic stenosis (IHSS)Idiopathic hypertrophic subaortic stenosis (IHSS)Muscle subaortic stenosis Muscle subaortic stenosis Hypertrophic obstructive cardiomyopathy (HOCM)Hypertrophic obstructive cardiomyopathy (HOCM)
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Genetic Basis of HCM Genetic Basis of HCM
Causes: Inherited, Causes: Inherited, acquired, unknown acquired, unknown
Autosomal dominant Autosomal dominant inheritance pattern inheritance pattern
>450 mutations in 13 >450 mutations in 13 cardiac sarcomere & cardiac sarcomere & myofilament-related myofilament-related genes identified genes identified
?? Role for ?? Role for environmental factors environmental factors
Alcalai et al. J Cardiovasc Electrophysiol. 19(1): Jan 2008.
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Genetics of HCMGenetics of HCM
Alcalai et al. J Cardiovasc Electrophysiol 2008;19:105.
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Pathophysiology of HCMPathophysiology of HCM
The pathophysiology of HCM involves 4 The pathophysiology of HCM involves 4 interrelated processes:interrelated processes:
Left ventricular outflow obstruction Left ventricular outflow obstruction Diastolic dysfunction Diastolic dysfunction Myocardial ischemia Myocardial ischemia Mitral regurgitation Mitral regurgitation
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LV Outflow Obstruction in HCMLV Outflow Obstruction in HCM
Long-standing LV outflow obstruction is a Long-standing LV outflow obstruction is a major determinant for heart failure major determinant for heart failure symptoms and death in HCM patients symptoms and death in HCM patients
Subaortic outflow obstruction is caused by Subaortic outflow obstruction is caused by systolic anterior motion (SAM) of the mitral systolic anterior motion (SAM) of the mitral valve – leaflets move toward the septumvalve – leaflets move toward the septum
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LV Outflow Obstruction in HCMLV Outflow Obstruction in HCM
Physiological Consequences of Physiological Consequences of Obstruction Obstruction Elevated intraventricular pressures Elevated intraventricular pressures Prolongation of ventricular relaxation Prolongation of ventricular relaxation Increased myocardial wall stress Increased myocardial wall stress Increased oxygen demand Increased oxygen demand Decrease in forward cardiac output Decrease in forward cardiac output
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Massive left ventricular hypertrophy, mainly confined to the septum
Histopathology showing significant myofiber disarray and interstitial fibrosis
Cell Research. 2003;13(1):10.
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Maron MS et al. NEJM. 2003;348:295.
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Pathophysiology of HCMPathophysiology of HCM
Diastolic Dysfunction Diastolic Dysfunction Contributing factor in 80% of patients Contributing factor in 80% of patients Impaired relaxation Impaired relaxation
High systolic contraction load High systolic contraction load Ventricular contraction/relaxation not uniform Ventricular contraction/relaxation not uniform
Accounts for symptoms of exertional dyspneaAccounts for symptoms of exertional dyspneaAbnormal diastolic filling à increased pulmonary Abnormal diastolic filling à increased pulmonary
venous pressure venous pressure
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Pathophysiology of HCMPathophysiology of HCM
Myocardial Ischemia Myocardial Ischemia Often occurs without atherosclerotic coronary Often occurs without atherosclerotic coronary
artery disease artery disease Postulated mechanismsPostulated mechanisms
Abnormally small and partially obliterated intramural Abnormally small and partially obliterated intramural coronary arteries as a result of hypertrophy coronary arteries as a result of hypertrophy
Inadequate number of capillaries for the degree of Inadequate number of capillaries for the degree of LV mass LV mass
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Pathophysiology of HCMPathophysiology of HCM
Mitral Regurgitation Mitral Regurgitation Results from the systolic anterior motion of the Results from the systolic anterior motion of the
mitral valve mitral valve Severity of MR directly proportional to LV Severity of MR directly proportional to LV
outflow obstruction outflow obstruction Results in symptoms of dyspnea, orthopnea in Results in symptoms of dyspnea, orthopnea in
HCM patients HCM patients
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Integrated PathophysiologyIntegrated Pathophysiology
Braunwald. Atlas of Heart Diseases: Cardiomyopathies, Myocarditis, and Pericardial Disease. 1998.
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Clinical Presentation Clinical Presentation
Dyspnea on exertion (90%), orthopnea, Dyspnea on exertion (90%), orthopnea, PND PND
Angina (70-80%) Angina (70-80%) Syncope (20%), Presyncope (50%) Syncope (20%), Presyncope (50%)
outflow obstruction worsens with increased outflow obstruction worsens with increased contractility during exertional activities contractility during exertional activities
Sudden cardiac deathSudden cardiac deathHCM is most common cause of SCD in young HCM is most common cause of SCD in young
people, including athletes people, including athletes
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Physical Examination Physical Examination
Carotid PulseCarotid PulseBifid – short upstroke & prolonged systolic ejection Bifid – short upstroke & prolonged systolic ejection
Jugular Venous Pulse Jugular Venous Pulse Prominent Prominent a wavea wave – decreased ventricular – decreased ventricular
compliance compliance
Apical Impulse Apical Impulse Double or triple Double or triple
Heart SoundsHeart SoundsS4 usually present due to hypertrophy S4 usually present due to hypertrophy
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Physical Examination Physical Examination
MurmurMurmurMedium-pitch crescendo-decrescendo systolic Medium-pitch crescendo-decrescendo systolic
murmur along LLSB without radiation murmur along LLSB without radiation Dynamic maneuvers Dynamic maneuvers
Murmur intensity increases with decreased Murmur intensity increases with decreased preload (i.e. Valsalva)preload (i.e. Valsalva)
Murmur intensity decreases with increased Murmur intensity decreases with increased preload (i.e. squatting, hand grip) preload (i.e. squatting, hand grip)
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Physical Examination in HCMPhysical Examination in HCM
Braunwald E. Atlas of Internal Medicine. 2007.
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Diagnostic Evaluation Diagnostic Evaluation
ElectrocardiogramElectrocardiogramEchocardiogram Echocardiogram Catheterization Catheterization
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Electrocardiogram in HCMElectrocardiogram in HCM
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Echocardiography in HCMEchocardiography in HCM
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Transesophageal EchoTransesophageal Echo
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Cardiac Catheterization Cardiac Catheterization
Hyperdynamic systole function results in almost complete obliteration of the LV cavity
Coronary angiography is not typically necessary in HCM
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Natural History of HCMNatural History of HCMCumulative Survival After Initial Diagnostic Evaluation Among Patients Diagnosed
as Having HCM at 20 Years or Older
Maron, BJ et al. JAMA 1999;281:650-655
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Disease Progression in HCMDisease Progression in HCM
ACC Consensus Document. J Am Coll Cardiol. 2003;42(9):1693.
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Sudden Cardiac Death in HCM Sudden Cardiac Death in HCM Most frequent in young Most frequent in young
adults <30-35 years oldadults <30-35 years oldPrimary VF/VT Primary VF/VT Tend to die during or Tend to die during or
just following vigorous just following vigorous physical activity physical activity
Often is 1Often is 1stst clinical clinical manifestation of manifestation of disease disease
HCM is most common HCM is most common cause of SCD among cause of SCD among young competitive young competitive athletes athletes
J Am Coll Cardiol. 2003;42(9):1693.
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SCD in Competitive Athletes SCD in Competitive Athletes
Maron B. Atlas of Heart Diseases. 1996
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Natural History of HCM Natural History of HCM
Heart Failure Heart Failure Only 10-15% progress Only 10-15% progress
to NYHA III-IV to NYHA III-IV Only 3% will become Only 3% will become
truly end-stage with truly end-stage with systolic dysfunction systolic dysfunction
Endocarditis Endocarditis 4-5% of HCM patients 4-5% of HCM patients Usually mitral valve Usually mitral valve
affected affected
Atrial Fibrillation Atrial Fibrillation Prevalent in up to 30% of Prevalent in up to 30% of
older patientsolder patients Dependent on atrial kick Dependent on atrial kick
– CO decreases by 40% – CO decreases by 40% if AF present if AF present
Autonomic Dysfunction Autonomic Dysfunction 25% of HCM patients 25% of HCM patients Associated with poor Associated with poor
prognosis prognosis
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Influence of Gender & Race Influence of Gender & Race
Women often remain underdiagnosed and Women often remain underdiagnosed and are clinical recognized after they develop are clinical recognized after they develop more pronounced symptomsmore pronounced symptoms11
HCM clinically underrecognized in African-HCM clinically underrecognized in African-Americans Americans Most athletes with SCD due to HCM are Most athletes with SCD due to HCM are undiagnosed African-Americansundiagnosed African-Americans22
11 Olivotto I et al. Olivotto I et al. J Am Coll CardiolJ Am Coll Cardiol 2005;46:480. 2005;46:480. 2 2 Maron BJ et al. Maron BJ et al. J Am Coll CardiolJ Am Coll Cardiol 2003;41:974. 2003;41:974.
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Treatment of HCM Treatment of HCM
Medical therapy Medical therapy Device therapy Device therapy Surgical septal myectomy Surgical septal myectomy Alcohol septal ablationAlcohol septal ablation
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ACC Consensus Document. J Am Coll Cardiol. 2003;42(9):1693.
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Medical Therapy Medical Therapy
Beta-blockersBeta-blockersIncrease ventricular diastolic filling/relaxation Increase ventricular diastolic filling/relaxation Decrease myocardial oxygen consumption Decrease myocardial oxygen consumption Have not been shown to reduce the incidence of Have not been shown to reduce the incidence of
SCD SCD Verapamil Verapamil
Augments ventricular diastolic filling/relaxation Augments ventricular diastolic filling/relaxation Disopyramide Disopyramide
Used in combination with beta-blocker Used in combination with beta-blocker Negative inotrope Negative inotrope
Diuretics Diuretics
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Dual-Chamber Pacing Dual-Chamber Pacing
Proposed benefit: pacing the RV apex will Proposed benefit: pacing the RV apex will decrease the outflow tract gradient decrease the outflow tract gradient
Several RCTs have found that the improvement Several RCTs have found that the improvement in subjective measures provided by dual-chamber in subjective measures provided by dual-chamber pacing is likely a placebo effect pacing is likely a placebo effect
Objective measures such as exercise capacity Objective measures such as exercise capacity and oxygen consumption are not improved and oxygen consumption are not improved
No correlation has been found between pacing No correlation has been found between pacing and reduction of LVOT gradient and reduction of LVOT gradient
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Surgical Septal MyectomySurgical Septal Myectomy
Nishimura RA et al. NEJM. 2004. 350(13):1320.
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Alcohol Septal Ablation Alcohol Septal Ablation
Braunwald. Atlas of Heart Diseases: Cardiomyopathies, Myocarditis, and Pericardial Disease. 1998.
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Alcohol Septal Ablation Alcohol Septal Ablation
Before After
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Alcohol Septal Ablation Alcohol Septal Ablation
Successful short-term outcomesSuccessful short-term outcomesLVOT gradient reduced from a mean of 60-70 mmHg LVOT gradient reduced from a mean of 60-70 mmHg
to <20 mmHg to <20 mmHg Symptomatic improvements, increased exercise Symptomatic improvements, increased exercise
tolerance tolerance Long-term data not available yet Long-term data not available yet Complications Complications
Complete heart blockComplete heart blockLarge myocardial infarctionsLarge myocardial infarctions
No randomized efficacy trials yet for alcohol No randomized efficacy trials yet for alcohol septal ablation vs. surgical myectomy septal ablation vs. surgical myectomy
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Circulation. 2008; 18(2): 131-9.
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Efficacy of Therapeutic StrategiesEfficacy of Therapeutic Strategies
Nishimura et al. NEJM. 2004. 350(13):1323.
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Coil Embolization Coil Embolization
Case report of 20 patients Case report of 20 patients w/ drug-refractory HCM w/ drug-refractory HCM
Occlude septal perforator Occlude septal perforator branches branches
NYHA functional class NYHA functional class and peak oxygen and peak oxygen consumption improved at consumption improved at 6 months 6 months
Significant reduction in Significant reduction in septum thickness by echo septum thickness by echo
European Heart Journal 2008;29:350.
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Implantable Cardioverter Implantable Cardioverter
Defibrillators in HCMDefibrillators in HCM Primary & Secondary Prevention Primary & Secondary Prevention
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Maron BJ et al. NEJM 2000;342:365-73.
Appropriate discharges in Appropriate discharges in 23% of patients 23% of patients
Rate of appropriate Rate of appropriate discharges of 7% per year discharges of 7% per year
Of 21 patients for which Of 21 patients for which intracardiac electrograms intracardiac electrograms were available, 10 shocks were available, 10 shocks for VT, 9 shocks for VFfor VT, 9 shocks for VF
Suggested role for ICDs in Suggested role for ICDs in primary & secondary primary & secondary prevention of SCDprevention of SCD
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Risk Stratification – ICDs Risk Stratification – ICDs
Primary Prevention Risk Factors for SCD Primary Prevention Risk Factors for SCD Premature HCM-related sudden death in more Premature HCM-related sudden death in more
than 1 relative than 1 relative History of unexplained syncope History of unexplained syncope Multiple or prolonged NSVT on Holter Multiple or prolonged NSVT on Holter Hypotensive blood pressure response to exercise Hypotensive blood pressure response to exercise Massive LVH Massive LVH
How many risk factors warrant ICD How many risk factors warrant ICD placement?placement?
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JAMA. 2007;298(4): 405-12.
Multicenter registry Multicenter registry study w/ 506 pts from study w/ 506 pts from 1986-2003 1986-2003
Mean follow-up 3.7 yrsMean follow-up 3.7 yrsAverage age 41 years Average age 41 years
old old Primary Outcome: Primary Outcome:
appropriate ICD appropriate ICD interventions interventions terminating VF/VT terminating VF/VT
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JAMA. 2007;298(4): 405-12.
J Cardiovasc Electrophysiol 2008;19(10).
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J Am Coll Cardiol 2008;51(10):1033-9.
3500 asymptomatic elite 3500 asymptomatic elite athletes (75% male), mean athletes (75% male), mean age 20.5 +/- 5.8 years, no age 20.5 +/- 5.8 years, no family hx of HCM family hx of HCM
12-lead ECG, 2D-Echo 12-lead ECG, 2D-Echo 53 athletes (1.5%) had LVH 53 athletes (1.5%) had LVH 3 athletes (0.08%) had ECG 3 athletes (0.08%) had ECG
and echo features of HCMand echo features of HCM
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HCM vs. Athlete’s Heart HCM vs. Athlete’s Heart
Circulation 1995;91.
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Future Directions Future Directions
Identification of additional causative mutations Identification of additional causative mutations Risk stratification tools Risk stratification tools Determining more precise indications for ICDs Determining more precise indications for ICDs
Defining most appropriate role for alcohol Defining most appropriate role for alcohol
septal ablation septal ablation ?Gene therapy ?Gene therapy