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Hypertrophic Cardiomyopathy Guidelines
Summary from the:ACC/ESC Clinical Expert Consensus Statement on Hypertrophic Cardiomyopathy
Maron BJ, et al. J Am Coll Cardiol 2003; 42: 1687-713
And ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm
AbnormalitiesEpstein AE et al. Heart Rhythm 2008;5:934-55
Hypertrophic CardiomyopathyGuidelines
Joseph Blackshear, MDMayo Clinic
Jacksonville, FL
www.HRSonline.org
Hypertrophic Cardiomyopathy: LVH ≥ 15 mm, without hypertension, aortic stenosis etc25% with LVOT obstruction
Maron BJ et al. JACC 2003;42:1687
www.HRSonline.org
HCM, epidemiology and genetics
1:500 persons
600,000 persons in US
Most common cause of SCD, age < 40 yr
Most common cause of SCD, competitive athletes
Data on management derived from registries, practice
With no SCD risk factors: 90 % predictive value for freedom from SCD, BUT 3-
5% may still suffer SCD
Heritable: 11 genes, > 1,000 mutations
60% have identifiable abnormal genotype
Predominantly autosomal dominant inheritance
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Approach to Hypertrophic Cardiomyopathy
Symptomatic treatment
Exertional dyspnea
Angina
Arrhythmias, including leading to SCD
Prevention of sudden cardiac death
ICD for primary or secondary prevention
Screening of relatives
History, exam, ECG, echo, MRI
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Symptomatic treatment
Class I: no drugs.
Class II: beta blocker, verapamil, or disopyramide.
possibly low dose diuretics. Avoid vasodilators and
inotropes.
Class III/IV: despite maximal medical management,
surgical septal myectomy; consideration of alcohol
septal ablation (or pacing) in selected patients.
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Prevention of Sudden Cardiac Death
Data from Registry, mean age 42, n=506, 4 yr follow up.
Resuscitated cardiac arrest or sustained VT: 11%
appropriate shocks per year after ICD implantation.
Primary prevention, ≥ 1major risk factor: 4% appropriate
shocks per year after ICD implantation.
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Risk Factors for SCD in HCM
Major, established
Cardiac arrest/ Sustained VT
Family history of SCD
Unexplained syncope
LV thickness ≥ 30 mm
Abnormal BP response to
exercise
NSVT on Holter
Possible
LV outflow obstruction ≥ 30 mm Hg
Intense physical exertion (competitive
sports)
Late gadolinium enhancement (scar) on
contrast MRI
Alcohol septal ablation
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Registry of 506 patients who received ICDs at 42 medical centers in US, Europe, and AustraliaMean age 42 yearsFunctional class: I: 55%, II: 32%, III/IV: 13%≥ 30 mm Hg mean gradient: 25%3.7 year mean follow upComplications: 27 % inappropriate shocks, 4% infection, 2% hemorrhage or thrombosis, 7% lead fracture
Maron BJ et al JAMA 2007; 298: 405
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Indications for ICD
Secondary protection
Cardiac arrest
Spontaneous sustained VT
Primary prevention
One or more major risk factors
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Genetic Testing and Screening (Irrelevant for risk stratification)
Only a few mutations predict a high risk for sudden death or
rapid progression to terminal CHF.
If (+) for causal mutations, relatives may be ruled in/out by
testing.
If (–) for causal mutation, clinical screen of family of affected
proband: begin at age 12, annually with echo, ECG, Holter,
exam. If negative by age 18, continue Q 5 yr.
Recommended