Hypertrophic Cardiomyopathy

Harry M. Lever, MD

Asymmetrical hypertrophic cardiomyopathy Asymmetrical hypertrophy of the heart Asymmetrical septal hypertrophy (ASH) Brock’ Brock’s disease Diffuse muscular subaortic stenosis Diffuse subvalvular aortic stenosis Dynamic hypertrophic subaortic stenosis Dynamic muscular subaortic stenosis Familial hypertrophic subaortic stenosis Familial hypertrophic cardiomyopathy (FHC) Familial muscular subaortic stenosis Familial myocardial disease Functional aortic stenosis Functional hypertrophic subaortic stenosis Functional obstructive cardiomyopathy Functional obstruction of the left ventricle Functional obstructive subvalvular aortic stenosis Functional subaortic stenosis Hereditary cardiovascular dysplasia Hypertrophic cardiomyopathy (HCM) Hypertrophic constrictive cardiomyopathy Hypertrophic hyperkinetic cardiomyopathy Hypertrophic infundibular aortic stenosis Hypertrophic nonobstructive cardiomyopathy Hypertrophic obstructive cardiomyopathy (HOCM) Hypertrophic stenosing cardiomyopathy Hypertrophic subaortic stenosis Idiopathic hypertrophic cardiomyopathy Idiopathic hypertrophic obstructive cardiomyopathy Idiopathic hypertrophic subaortic stenosis (IHSS)

Idiopathic hypertrophic subvalvular stenosis Idiopathic muscular hypertrophic subaortic stenosis Idiopathic muscular stenosis of the left ventricle Idiopathic myocardial hypertrophy Idiopathic stenosis of the flushing chamber of the left ventricle Idiopathic ventricular septal hypertrophy Irregular hypertrophic cardiomyopathy Left ventricular muscular stenosis Low subvalvular aortic stenosis Muscular aortic stenosis Muscular hypertrophic stenosis of the left ventricle Muscular stenosis of the left ventricle Muscular subaortic stenosis (MSS) Muscular subvalvular aortic stenosis NonNon-dilated cardiomyopathy Nonobstructive hypertrophic cardiomyopathy Obstructive cardiomyopathy Obstructive hypertrophic aortic stenosis Obstructive hypertrophic cardiomyopathy Obstructive hypertrophic myocardiopathy Obstructive myocardiopathy Pseudoaortic stenosis Stenosing hypertrophy of the left ventricle Stenosis of the ejection chamber of the left ventricle Subaortic hypertrophic stenosis Subaortic idiopathic stenosis Subaortic muscular stenosis Subvalvular aortic stenosis of the muscular type Teare’ Teare’s disease

Intensive Review of Cardiology

Asymmetrical hypertrophic cardiomyopathy Asymmetrical hypertrophy of the heart Asymmetrical septal hypertrophy (ASH) Brock’ Brock’s disease Diffuse muscular subaortic stenosis Diffuse subvalvular aortic stenosis Dynamic hypertrophic subaortic stenosis Dynamic muscular subaortic stenosis Familial hypertrophic subaortic stenosis Familial hypertrophic cardiomyopathy (FHC) Familial muscular subaortic stenosis Familial myocardial disease Functional aortic stenosis Functional hypertrophic subaortic stenosis Functional obstructive cardiomyopathy Functional obstruction of the left ventricle Functional obstructive subvalvular aortic stenosis Functional subaortic stenosis Hereditary cardiovascular dysplasia Hypertrophic cardiomyopathy (HCM) Hypertrophic constrictive cardiomyopathy Hypertrophic hyperkinetic cardiomyopathy Hypertrophic infundibular aortic stenosis Hypertrophic nonobstructive cardiomyopathy Hypertrophic obstructive cardiomyopathy (HOCM) Hypertrophic stenosing cardiomyopathy Hypertrophic subaortic stenosis Idiopathic hypertrophic cardiomyopathy Idiopathic hypertrophic obstructive cardiomyopathy Idiopathic hypertrophic subaortic stenosis (IHSS)

Idiopathic hypertrophic subvalvular stenosis Idiopathic muscular hypertrophic subaortic stenosis Idiopathic muscular stenosis of the left ventricle Idiopathic myocardial hypertrophy Idiopathic stenosis of the flushing chamber of the left ventricle Idiopathic ventricular septal hypertrophy Irregular hypertrophic cardiomyopathy Left ventricular muscular stenosis Low subvalvular aortic stenosis Muscular aortic stenosis Muscular hypertrophic stenosis of the left ventricle Muscular stenosis of the left ventricle Muscular subaortic stenosis (MSS) Muscular subvalvular aortic stenosis NonNon-dilated cardiomyopathy Nonobstructive hypertrophic cardiomyopathy Obstructive cardiomyopathy Obstructive hypertrophic aortic stenosis Obstructive hypertrophic cardiomyopathy Obstructive hypertrophic myocardiopathy Obstructive myocardiopathy Pseudoaortic stenosis Stenosing hypertrophy of the left ventricle Stenosis of the ejection chamber of the left ventricle Subaortic hypertrophic stenosis Subaortic idiopathic stenosis Subaortic muscular stenosis Subvalvular aortic stenosis of the muscular type Teare’ Teare’s disease

Hypertrophic Cardiomyopathy (HCM)

Intensive Review of Cardiology

Prevalence of HCM and Less Common Diseases Known to Cause Death and Disability

Hypertrophic Cardiomyopathy(HCM)

1 : 500

Multiple Sclerosis(MS)

1 : 700

Marfan Syndrome

1 : 5,000

Long QT Syndrome(LQTS)

1: 5,000

Cystic Fibrosis(CF)

1: 10,000

ALS

1: 10,000

Muscular Dystrophy (MD)

1: 16,000

Intensive Review of Cardiology

Abnormal Muscle Cells

Normal

HCM Intensive Review of Cardiology

Symptoms of LV Outflow Obstruction SVAS VAS MSAS HSAS

Dyspnea Angina Palpitations Syncope-dizziness Sudden death

Intensive Review of Cardiology

Asymptomatic Presentations • Heart murmur • Unexplained LVH with or without a murmur • EKG - old myocardial infarction

Intensive Review of Cardiology

Physical Findings • Bifid or bisferiens carotid pulse • Sustained apical impulse • S4 gallop • Systolic murmur

Intensive Review of Cardiology

Physiological and Pharmacologic Maneuvers Ventricular Volume

Gradient

Murmur

Valsalva

↓

↑

↑

Amyl Nitrite

↓

↑

↑

Isoproterenol

↓

↑

↑

Hand Grip

↑

↓

↓

Phenylephrine

↑

↓

↓

Beta Blocker

↑

↓

↓

Intensive Review of Cardiology

Aortic Root Aortic Valve IV Septum Mitral Valve Phono ECG

Aortic Pressure LV Pressure

Intensive Review of Cardiology

Anterior LVFW Hypertrophy a

b

Posterior VS Hypertrophy

c

d

Maron BJ et al.

Intensive Review of Cardiology

a

Anterolateral LVFW Hypertrophy b

c

d

Maron BJ et al.

Intensive Review of Cardiology

Intensive Review of Cardiology

Intensive Review of Cardiology

58.1%

30.6%

9.7%

1.6% Spirito P et al.

Intensive Review of Cardiology

Stewart et al.

Intensive Review of Cardiology

CW Doppler Flow Profiles

Aortic Flow

LVOT Flow

MR & LVOT Flow

Intensive Review of Cardiology

Predictor of LVOT Gradient in HCM 3D assessment of LVOT area

Qin et al.

Intensive Review of Cardiology

Aortic Stenosis vs. HCM

Sherrid, M., Cardiology in Review;6:1988

Intensive Review of Cardiology

Cavity Obliteration

D’Cruz IA., Echocardiographic Anatomy

Intensive Review of Cardiology

Intracavitary Gradient

Pearson et al., JACC;12:1988

Intensive Review of Cardiology

Hypertrophic Cardiomyopathy Brock’s Disease vs Teare’s Disease

Intensive Review of Cardiology

Hypertrophic Cardiomyopathy Is This a Congenital Disease? or Is This an Acquired Disease?

Intensive Review of Cardiology

Intensive Review of Cardiology

Intensive Review of Cardiology

Intensive Review of Cardiology

Incidence of Hypertrophic Disease in the Aged Early Studies Hardarson

1 of 119

Over 60

Adelman and Wigle

1 of 60

Over 70

Frank and Braunwald

2 of 126

Over 60

1.3%

Over 55

Intensive Review of Cardiology

Incidence of Hypertrophic Disease in the Aged

Later Studies Whiting

14 of 44

Over 60

Hamby

9 of 35

Over 70

Krasnow

15 of 20

Over 60

Berger

26 of 43

Over 60

45%

Over 60

Intensive Review of Cardiology

2-D Echo Findings

Ovoid Left Ventricle Reversed Septal Curvature Proximal Septal Bulge Right Ventricular Hypertrophy Mitral Anular Calcification

Elderly

Young

24 (86%)

7 (25%) p50% relief of the gradient – 4%