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DR.VINOD.G.V

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DEFINITION 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 given patient.

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HCM is a common genetic cardiovascular disease Prevalance estimated to be 1:500

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GENETICS Caused by autosomal dominant mutations in genes encoding protein components of the sarcomere and its constituent myofilament elements. 1400 mutations identified among at least 8 genes.

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HCM GENES AND THEIR FREQUENCIES GENECHROMOSOMEFREQUENCY % Beta MHC14q135-50 MYBP C11q1115-20 Cardiac troponin T1q315-20 Alpha tropomyosin15q2

CMR - Poor Prognostic factors Markedly elevated LV mass index (men > 91 g/m 2, women > 69 g/m 2 ) - sensitive(100%) Maximal wall thickness of more than 30 mm specific (91%) for cardiac deaths

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Type 1:Anterior segment of septum(10%) Type 2:Both anterior and posterior segment(20%) Type 3:Septum and anterolateral free wall(52%) Type 4:Other regions including apical HCM (18%)

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Right ventricular (RV) hypertrophy Myocardial edema by T2-weighted imaging LGE has been associated with Ventricular arrhythmias Progressive ventricular dilation

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BURNTOUT HCM 3% manifest the end stage- systolic dysfunction (ejection fraction

Athlete's Heart Vs Hypertrophic Cardiomyopathy HCM Can be asymmetric Wall thickness: > 15 mm LA: > 40 mm LVEDD :< 45 mm Diastolic function: always abnormal Athletic heart Concentric & regresses < 15 mm < 40 mm > 45 mm Normal

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MANAGEMENT

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SUDDEN CARDIAC DEATH Most commonly in adolescents and young adults

Age 12 to 1821 y Every 1218 mo Age >1821 y At onset of symptoms or at least every 5 y More frequent intervals are appropriate in families with late-onset HCM.

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Genotype-Positive/Phenotype-Negative Patients CLASS 1 Serial ECG, TTE, and clinical assessment at periodic intervals 12 to 18 months in children and adolescents Every 5 years in adults based on the patients age and change in clinical status

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MEDICAL THERAPY Initial therapeutic approach to relieving symtoms with obstructive HCM. Beta -Adrenergic blocking agents are the initial drug of choice

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Beta Blockers Advantages of beta blockers include 1. Decreased heart rate response to exercise 2. Decreased outflow tract gradient with exercise 3.Relief of angina by a decrease in myocardial oxygen demand 4 Improvement in diastolic filling

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Dosage should be titrated to symptom relief or to obtain a resting heart rate of

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Calcium Channel Blockers specifically verapamil and diltiazem decrease inotropy and chronotropy also improve abnormal diastolic relaxation by preventing calcium influx Verapamil -used most frequently due to its minimal effect on afterload.

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In contrast to beta blocking drugs an improvement in diastolic filling occurred with verapamil. CCBs may improve angina to a greater degree than beta blockers. verapamil - sustained symptomatic improvement in

SEPTAL ABLATION NYHA class III-IV symptoms despite maximal medical therapy Septal thickness >18mm Subaortic gradient >50mmHg due to mitral septal contact Absence of papillary muscle or mitral valve anomalies

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Absence of significant coronary arterial disease Compatible septal perforator branch arterial anatomy. Relative contraindications to surgical myectomy(age,comorbidity)

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Initial results reported successful short- term outcomes The outflow tract gradient is reduced from a mean of 60 to 70 mm Hg often to

5.All are indications of alcohol septal ablation except a.NYHA class III-IV symptoms despite maximal medical therapy b.Septal thickness >18mm c.Subaortic gradient >30mmHg d.Absence of papillary muscle or mitral valve anomalies.

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6.True regarding alcohol septal ablation except a.10ml of absolute alcohol is injected b.Immediate reduction of LVOT gradient c.Increased incidence of complete heart block d.Predisposes to SCD

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7.All are true regarding medical management of HCM except a.Betablockers are the initial drug of choice b.CCBS cause better angina control than betablockers c.Diltiazem can precipitate acute pulmonary edema d.Combination of betablockers and CCBS more effective than single drug

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8.ECHO features of HCM all except a.Septal hypertrophy >15mm b.Dagger shaped doppler spectrum c.SAM of anterior mitral valve. d.Increased mitral annular velocity on DTI

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9.All are true regarding athlets heart except a.Wall thickness 45mm c.Diastolic function is normal d.LVH does not regress on deconditioning

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10.All are potential arbitrators for SCD except a.End stage phase b.LV apical aneurysm c.CMR Late gadolinium enhancement d.Unexplained syncope of recent oncet

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References Braunwalds Heart disease 9 th edition Hurst 13 th edition ACC AHA 2011 Guidelines Topol interventional cardiology 5 th Feigenbaums Echocardiography 7 th Bonita Anderson echocrdiography NEJM 2004 march 25,Rick A,Nishimura

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JAMA 2002 287,HOCM Barry J Maron Circulation 2008,118,131-139 Circulation 2001,104,2113-2116 JACC 2005 no 3,volume 46 JACC 2009,NO3,volume 54 JACC 2011,No 10 volume 56 JACC 2011 no 5 volume 57