Overview

• In the past 25 years, major advance were achieved in the nasography

of cardiomyopathies, influencing the definition and taxonomy of this
important chapter of cardiovascular disease.
Overview
• Nearly, 50 % of patients dying suddenly in childhood or adolescence
or undergoing cardiac transplantation are affected by
cardiomyopathies.
Overview
• Novel cardiomyopathies have been discovered arrhythmogenic,
Restrictive, Non compacted and added to the update of the world
health organization classification.

• Myocarditis has also been named inflammatory cardiomyopathy

Overview
• Cardiomyopathies are easily diagnosed but treated only with
palliative pharmacological or invasive therapy.
Overview
• 1899
• The concept of isolated, non ischemic disease of the myocardium was
proposed by Fiedler in Dresden
• Published an article entitled ‘’Acute interstitial myocarditis’’
• He described a series of fatal cases in young adults with cardiomegaly
and heart failure
• This condition named Fiedler’s myocarditis
• Characterized by sudden onset of was originally thought to be
malarial fever probably of microbial origin, often fatal in 5-17 days
Overview
• In 1949
• Evans reported on families with
unexplained cardiomegaly
Overview
• 1957
• Brigden first use the term
cardiomyopathy.
• Non coronary cardiomyopathies
to describe patients with
idiopathic myocardial disease

Wallace Brigden's grave at St Andrew's church,

Totteridge.
Overview
• 1980
• Task force of the world health
organization
• Chaired by John Goodwin
• Presented the first classification
of cardiomyopathies
• Dilated cardiomyopathy
• Hypertrophic cardiomyopathy
• Restrictive cardiomyopathies
John Goodwin (1918–2001)
Overview
• 1990
• Discovery of mutation gene
• B-myosin heavy chain gene
• Disease causing gene
Overview
• 1996
• World health organization classification
of cardiomyopathies
• The “unknown was deleted”.
• Cardiomyopathies was redefined as
“diseases of the myocardium associated
with cardiac dysfunction”.
Overview
• 1996
• Arrhythmogenic right ventricular
cardiomyopathy (ARVC) was added to the
classification.
• Several unclassified cardiomyopathies were
high lightened including left ventricular non-
compaction.
• Myocarditis included secondary myocarditis.
Overview
• 2006
• Writing committee of the American
heart association presented
classification.
• 2 distinct classification were
proposed.
• Primary and secondary classification.
Overview
• 2006
• In this scientific statement,
• The cardiomyopathies were considered
to be PRIMARY when the disease is
sole predominantly of the heart
muscle.
• SECONDARY when myocardial
involvement is associated with
multisystem disorder (eg Fabrys’s
disease, sarcoidosis, amyloidosis).
Overview
• 2006
• The primary cardiomyopathies
were classified as
• Genetic (HCM, ARVC)
• Acquired (Myocarditis)
• Mixed (both genetic and
acquired DCM)
Definition
• Cardiomyopathies are diseases of the heart muscle
• Genetic defects
• Cardiac myocyte injury
• Infiltration of myocardial tissue
Definition
• As of 2006, cardiomyopathies are defined as:
“A heterogeneous group of diseases of the myocardium associated
with mechanical and/or electrical dysfunction that usually (but not
invariably) exhibit inappropriate ventricular hypertrophy or dilatation
and are due to a variety of causes that frequently are genetic”.
Epidemiology
• Accounts for 5–10% of the 5–6
million patients already
diagnosed with heart failure in
the United States.
GENERAL PRESENTATION
• Exertional intolerance
• Breathlessness/shortness of
breath/dyspnea
• Fatigue
• chest pain
• Orthopnea
• peripheral edema
• atrial and ventricular
tachyarrhythmias
GENERAL PRESENTATION
• Exertional intolerance
• Breathlessness/shortness of
breath/dyspnea
• Fatigue
• chest pain
• Orthopnea
• peripheral edema
• atrial and ventricular
tachyarrhythmias
GENERAL PRESENTATION
• Exertional intolerance
• Breathlessness/shortness of
breath/dyspnea
• Fatigue
• chest pain
• Orthopnea
• peripheral edema
• atrial and ventricular
tachyarrhythmias
GENERAL PRESENTATION
• Restrictive cardiomyopathy can overlap in presentation, gross
morphology, and etiology with both hypertrophic and dilated
cardiomyopathies
ETIOLOGIES OF CARDIOMYOPATHY
ETIOLOGIES OF CARDIOMYOPATHY
• GENETIC
Estimates for the prevalence of genetic etiology for cardiomyopathy
continue to rise.
Most familial cardiomyopathies are inherited in an autosomal
dominant pattern.
occasional autosomal recessive and X-linked inheritance
Well-recognized in hypertrophic cardiomyopathy, heritability is also
present in at least 30%of dilated cardiomyopathy
ETIOLOGIES OF CARDIOMYOPATHY:
GENETICS
ETIOLOGIES OF CARDIOMYOPATHY:
VIRUS
ETIOLOGIES OF CARDIOMYOPATHY:
VIRUS
COMMON VIRUS AND OTHER
PATHOGENS ASSOCIATED WITH
MYOCARDITIS
ETIOLOGIES OF CARDIOMYOPATHY:
AUTOIMMUNE
ETIOLOGIES OF CARDIOMYOPATHY:
AUTOIMMUNE
Two general theories
1. Viral components incorporate into
the cardiac myocyte membrane
stimulating an antigenic response.

2. Anti heart antibodies are generated

as a result of myocardial damage as
opposed to being the proximate
caused
Classification of Cardiomyopathies
• Two distinct classifications

1. American Heart Association classification.

2. 2008 European Society of Cardiology classification.

American Heart Association
American Heart Association
American Heart Association
American Heart Association
American Heart Association
European Society of Cardiology
Classification
European Society of Cardiology
Classification
European Society of Cardiology
Classification
European Society of Cardiology
Classification
European Society of Cardiology
Classification
 DILATED CARDIOMYOPAHY

•Dilatation and impaired contraction of the left ventricle or both ventricle

•Caused by familial genetics, viral, immune, alcoholic toxic, or unknown
factor or is associated with recognized cardiovascular disease.
 HYPERTROPHIC CARDIOMYOPAHY

• Left and or right ventricular hypertrophy, often asymmetric

• usually involves the interventricular septum
 RESTRICTIVE CARDIOMYOPATHY

• Mutation in sarcoplasmic proteins

• Restricted filling and reduced diastolic size of either ventricle or both ventricles
with normal or near normal systolic function
 ARRHYTHMOGENIC RIGHT VENTRICULAR CARDIOMYOPATHY

• Progressive fibrofatty replacement of the right and some degree of left

ventricular myocardium
Specific cardiomyopathies
(Specific Etiologies)
• Ischemic Ischemic: thinned, scarred tissue

• Valvular
• Hypertensive
• Inflammatory
• Metabolic
• Inherited
• Toxic reactions
• Peripartum
Ischemic cardiomyopathies

• Arises as dilated cardiomyopathy

with depressed ventricular
function not explained by the
extent of coronary artery,
obstruction or ischemic changes
Valvular cardiomyopathies

• Arises as ventricular dysfunction

that is out of proportion to the
abnormal loading condition
produced by valvular stenosis
and or regurgitation.
Hypertensive cardiomyopathy

• Arises with ventricular

hypertrophy with features of
cardiac failure related to systolic
or diastolic dysfunction.
Inflammatory cardiomyopathy

• Cardiac dysfunction as
consequence of myocarditis.
Metabolic cardiomyopathy

• Includes a wide variety of causes,

including endocrine abnormalities,
glycogen storage disease,
deficiencies such as hypokalemia,
and nutritional disorder.
• Hemochromatosis. Microscopic
image of an endomyocardial biopsy
showing extensive iron deposition
within the cardiac myocytes
General systemic disease

• Includes connective tissue

disorder and infiltrative diseases
such as sarcoidosis and
leukemia.
• Microscopic image of an
endomyocardial biopsy showing
a noncaseating granuloma and
associated interstitial fibrosis
typical of sarcoidosis.
Muscular dystrophies

• Includes Duchenne, Becker type and myotonic dystrophies.

Sensitivity and toxic reaction

• Includes reaction to alcohol,

catecholamines, anthracyline,
irradiation.
Peripartum cardiomyopathy

• First become manifested in the peripartum period, but is probably

heterogenous group.
 Functional classification of cardiomyopathies
Dilated Restrictive Hypertrophic

SYMPTOMS

congestive heart failure Dyspnea and fatigue; Dyspnea; angina pectoris;

particularly left sided; Right sided congestive Fatigue, syncope,
Fatigue and weakness heart failure palpitation
Systemic or pulmonary Sign and symptom of
emboli systemic disease ( eg
amyloidosis; iron storage
disease)
 Functional classification of cardiomyopathies
Dilated Restrictive Hypertrophic
PHYSICAL EXAMINATION
Moderate to severe Mild to moderate Mild cardiomegaly;
cardiomegaly; S3 S4; cardiomegaly, S3 or S4; Apical systolic thrill and
Atrioventricular valve atrioventricular valve heave;
regurgitation, especially regurgitation, inspiratory Brisk carotid upstroke, S4
mitral increase in venous is common;
pressure (kussmaul sign) Systolic murmur that
increases with Valsalva
maneuver
 Functional classification of cardiomyopathies
Dilated Restrictive Hypertrophic
ELECTROCARDIOGRAPHY
Sinus tachycardia; Low voltage; Left ventricular
Atrial ventricular Intraventricular hypertrophy;
arrhythmias; conduction defect; ST segment T wave
ST segment T wave Atrioventricular abnormalities;
abnormalities; conduction defect Abnormal q wave;
Intraventricular Atrial and ventricular
conduction defect arrhythmias
 Functional classification of cardiomyopathies
Dilated Restrictive Hypertrophic
ECHOCARDIOGRAPHY
Left ventricular dilation Increase left ventricular Asymmetric septal
and dysfunction; wall thickness and mass; hypertrophy;
Abnormal mitral valve Small or normal size left Narrow left ventricular
motion secondary to ventricular cavity, normal outflow tract; systolic
abnormal compliance and systolic function; anterior motion of the
filling pressure Pericardial effusion mitral valve;
small or normal sized left
ventricle
 Functional classification of cardiomyopathies
Dilated Restrictive Hypertrophic
CHEST RADIOGRAPHY
Moderate to marked Mild cardiac enlargement; Mild to moderate cardiac
cardiac enlargement, Pulmonary venous enlargement; left atrial
especially left ventricular; congestion enlargement
Pulmonary venous
congestion
 Chest radiograph
Hypertrophic cardiomyopathy Dilated cardiomyopathy Restrictive cardiomyopathy
 DILATED
CARDIOMYOPATHY
 DILATED CARDIOMYOPATHY

 Most common
 Enlargement of ventricles and systolic dysfunction
 25% of congestive heart failure
 50% with specific cause
 50% idiopathic  diagnosis of exclusion
 Enlarged left ventricle with decreased systolic function
 DILATED CARDIOMYOPATHY
Major Causes
Inflammatory Myocarditis Toxic Metabolic
Infective

Viral (coxsackie,a adenovirus,a HIV, hep C) Alcohol Nutritional deficiencies

Parasitic (T. cruzi—Chagas’ disease, Catecholamines Electrolyte deficiencies
trypanosomiasis, toxoplasmosis) Chemotherapeutic agents Pheochromocytoma
Bacterial (diphtheria) Heavy metals: lead, mercury Diabetes
Spirochetal (Lyme disease) Occupational exposure Obesity
Rickettsial (Q fever)
Fungal (with systemic infection)

Non-infective

Sarcoidosis
Giant cell myocarditis
 DILATED CARDIOMYOPATHY
Inflammatory Infective VIRAL

Myocarditis: injure the myocardium through direct invasion, production of cardiotoxic

substances, or chronic inflammation with or without persistent infection
Most commonly associated with viruses and the protozoan Trypanosoma cruzi.

Viruses gain entry through the respiratory or gastrointestinal tract, they can infect organs possessing specific
receptors, such as the coxsackie-adenovirus receptor on the heart  Viral infection and replication can cause
myocardial injury and lysis  viral replication and infection through degradation of the myocyte protein
dystrophin, which is crucial for myocyte stability  activation of viral receptor proteins can also activate host
tyrosine kinases, which modify the cytoskeleton to facilitate further viral entry  Ongoing cytokine release
activates matrix metalloproteinases that can disrupt the collagen and elastin scaffolding of the heart, potentiating
ventricular dilation
 DILATED CARDIOMYOPATHY
Inflammatory Infective VIRAL

Myocarditis: injure the myocardium through direct invasion, production of cardiotoxic

substances, or chronic inflammation with or without persistent infection
Most commonly associated with viruses and the protozoan Trypanosoma cruzi.

Viruses gain entry through the respiratory or gastrointestinal tract, they can infect organs possessing specific
receptors, such as the coxsackie-adenovirus receptor on the heart  Viral infection and replication can cause
myocardial injury and lysis  viral replication and infection through degradation of the myocyte protein
dystrophin, which is crucial for myocyte stability  activation of viral receptor proteins can also activate host
tyrosine kinases, which modify the cytoskeleton to facilitate further viral entry  Ongoing cytokine release
activates matrix metalloproteinases that can disrupt the collagen and elastin scaffolding of the heart, potentiating
ventricular dilation
 DILATED CARDIOMYOPATHY
Inflammatory Infective VIRAL
Patients with recent or ongoing viral syndromes can be classified
into three levels of diagnosis:
1. Possible subclinical acute myocarditis: typical viral syndrome but no cardiac symptoms, with one or
more of the following:
• Elevated biomarkers of cardiac injury (troponin or CK-MB)
• ECG findings suggestive of acute injury
• Reduced left ventricular ejection fraction or regional wall motion
• Abnormality on cardiac imaging, usually echocardiography
2. Probable acute myocarditis is diagnosed when the above criteria are met and accompanied also by cardiac
symptoms, such as shortness of breath or chest pain, which can result from pericarditis or myocarditis.
When clinical findings of pericarditis (pleuritic chest pain, ECG abnormalities, pericardial rub or effusion) are
accompanied by elevated troponin or CK-MB or abnormal cardiac wall motion
3. Definite myocarditis is diagnosed when there is histologic or immunohistologic evidence of inflammation on
endomyocardial biopsy and does not require any other laboratory or
clinical criteria.
 DILATED CARDIOMYOPATHY
Inflammatory Infective BACTERIAL

 Direct invasion and abscess formation

 Depress contractility in severe infection and sepsis
 Diphtheria: the bacillus releases a toxin that impairs protein synthesis
and may particularly affect the conduction system
 Streptococcal infection: inflammation and fibrosis of cardiac valves and
systemic connective tissue
 Tuberculosis can involve the myocardium directly
 DILATED CARDIOMYOPATHY
Inflammatory Infective

CLINICAL PRESENTATION

 Symptoms and signs of heart failure

 Chest pain
 Atrial or ventricular tachyarrhythmias, or by pulmonary or systemic emboli
 from intracardiac thrombi
 Progressive dyspnea and weakness within a few days to weeks after a viral
syndrome that was accompanied by fever and myalgias
 DILATED CARDIOMYOPATHY
Inflammatory Non- Infective

CLINICAL PRESENTATION

LUNGS
Rapid-onset heart failure and ventricular tachyarrhythmias, conduction block, chest pain
syndrome
Ocular involvement
Skin rash
Nonspecific febrile illness
 DILATED CARDIOMYOPATHY
Toxic

Alcohol: most common toxin

Polymorphisms of the genes encoding alcohol dehydrogenase and the

angiotensin-converting enzyme
Five to six drinks (about 4 ounces of pure ethanol) daily for 5–10 years, but frequent binge
drinking may also be sufficient
Reversible after cessation of alcohol consumption
 DILATED CARDIOMYOPATHY
Metabolic

 Diabetes: insulin resistance an increased advanced-glycosylation

end products, which impair both systolic and diastolic function
 Obesity: impaired excretion of excess volume load  increased
wall stress and secondary adaptive neurohumoral responses
DILATED CARDIOMYOPATHY
IDIOPATHIC SPECIFIC

Genetic or Stress (Tako-tsubo

or Broken Heart
previous Syndrome)
environmental Peripartum
insults Cardiomyopathy

Tachycardia-induced
Cardiomyopathy

Alcoholic
Cardiomyopathy
Factors associated with an adverse outcome
CLINICAL NON-INVASIVE
NYHA Class III/IV Elevated serum BNP Low LV ejection
fraction
Increasing age Elevated uric acid
Marked LV dilatation
Low exercise peak oxygen Decreased serum
Low LV mass
consumption sodium
> Moderate mitral
Marked intraventricular Protodiastolic gallop regurgitation
conduction delay (S3)
Abnormal diastolic
Complex Ventrciular Evidence of excessive function
Arrythmias sympathetic stimulation
Abnormal Contractile
reserve
High LV filling
INVASIVE pressures RV dilatation or
dysfunction
Pathology
Pathology
Pathology
DILATED CARDIOMYOPATHY
DIAGNOSIS
Echocardiography

Radionuclide Imaging
Cardiac Magnetic Resonance Imaging and Multidetector Computed
Tomography

Endomyocardial Biopsy
DILATED CARDIOMYOPATHY
MANAGEMENT

Pharmacologic and Device Therapy

Surgery

Emerging Specific Therapies

DILATED CARDIOMYOPATHY
Studies and new updates
RESTRICTIVE
CARDIOMYOPATHY
RESTRICTIVE CARDIOMYOPATHY

Increase in stiffness of the ventricular walls

Systolic function may be normal in early stages

Most common cause: Amyloidosis

RESTRICTIVE CARDIOMYOPATHY
Classification of Types of Restrictive Cardiomyopathy
according to cause

Non-infiltrative Infiltrative Storage Disease Endomyocardial

 Idiopathic  Amyloidosis  Hemochroma

 Endomyocardial
 Familial  Sarcoidosis tosis fibrosis
 Hypertrophic  Gaucher  Fabry  Radiation
 Scleroderma disease disease  Toxic effects of
 Pseudoxantho  Hurler  Glycogen anthracyline
ma elasticum disease storage
 Diabetic  Fatty disease
infiltration
RESTRICTIVE CARDIOMYOPATHY
Restrcitive Cardiomyopathy Constrcitive Pericarditis
CARDIAC CATHETERIZATION
 Equalization of diastolic  Equalization of diastolic
pressure vary pressure (pressures differ by
 Pulmonary hypertension is no more than 5 mmHg)
worse with systolic
pulmonary pressures often
exceeding 50 mmHg
Pathology
Pathology
Pathology
RESTRICTIVE CARDIOMYOPATHY
Pathophysiology and cause
RESTRICTIVE CARDIOMYOPATHY
Diagnosis and treatment
RESTRICTIVE CARDIOMYOPATHY
Diagnosis and treatment based on history and pe
Imaging modalities (xray ecg 2decho) histopath with picture and
description
RESTRICTIVE CARDIOMYOPATHY
Studies and new updates
HYPERTROPHIC
CARDIOMYOPATHY
HYPERTROPHIC CARDIOMYOPATHY
• Defined as left ventricular hypertrophy that develops in the absence
of causative hemodynamic factors, such as hypertension, aortic valve
disease, or systemic infiltrative or storage diseases.
HYPERTROPHIC CARDIOMYOPATHY
• The first contemporary reports on HCM in 1958 are from Brock in the
cardiac catherezation laboratory and from teare, who described at
autopsy “asymmetric hyperthropy of the heart” as responsible for
cardiac death in small group of young people.
HYPERTROPHIC CARDIOMYOPATHY
Previously been termed:
• Hypertrophic obstructive cardiomyopathy (HOCM)
• Asymmetric septal hypertrophy (ASH)
• Idiopathic hypertrophic subaortic stenosis (IHSS)
• The accepted terminology is now hypertrophic cardiomyopathy with
or without obstruction.
HYPERTROPHIC CARDIOMYOPATHY:
Epidemiology
• Prevalence in North America, Japan, and China is about 1:500.
• Several epidemiologic studies have reported a similar prevalence of
HCM phenotype in general population about 1:500.
• It is the leading cause of sudden death in the young and is an
important cause of heart failure.
HYPERTROPHIC CARDIOMYOPATHY:
Genetic Basis
• Autosomal dominant pattern of inheritance
HYPERTROPHIC CARDIOMYOPATHY:
Genetic Basis
HYPERTROPHIC CARDIOMYOPATHY:
Morphology
• Clinical diagnosis of HCM is usually made with two dimentional
echocardiography (2D-echo).
• More recently cardiovascular magnetic resonance imaging (CMR).
HYPERTROPHIC CARDIOMYOPATHY:
Morphology
Gross specimen
• symmetric septal hypertrophy
(septum much thicker than left
ventricular free wall) with the
septum bulging into the left
ventricular outflow tract causing
obstruction. There is patchy
replacement fibrosis, and small
thick-walled arterioles can be
appreciated grossly, especially in
the interventricular septum.
HYPERTROPHIC CARDIOMYOPATHY:
2D-Echo
• Asymmetric hypertrophy of the
septum compared to the lateral
wall of the left ventricle.
• The mitral valve (MV) is moving
anteriorly toward the
hypertrophied septum in systole.
• The left atrium (LA) is enlarged.
HYPERTROPHIC CARDIOMYOPATHY:
2D-Echo
• Hyperthrophy is frequently
diffuse involving portion of
ventricular septum and left
ventricle free wall.
• However, in about 50% of
patients LV hypertrophy is non
diffuse.
 HYPERTROPHIC CARDIOMYOPATHY:
CMR
• Cardiac magnetic resonance imaging
(MRI) uses a powerful magnetic field,
radio waves and a computer to produce
detailed pictures of the structures
within and around the heart
• CMR is complementary to
echocardiography by clarifying
technically ambiguous LV wall thickness
by visualizing abnormalities often not
identifiable with echocardiography
HYPERTROPHIC CARDIOMYOPATHY:
CMR
HYPERTROPHIC CARDIOMYOPATHY:
Histopathology
• In HCM, cardiac muscle cell (myocytes)
in both ventricular septum and LV free
wall show increased transverse diameter
and bizarre shapes often maintaining
intercellular connections with several
adjacent cells.
HYPERTROPHIC CARDIOMYOPATHY:
Histopathology
• Myocytes and Myofilaments
arrange in chaotic disorganized
pattern
HYPERTROPHIC CARDIOMYOPATHY:
ECG
ECG is abnormal in more than 90% of probands with HCM and in about
75% of asymptomatic relatives.
HYPERTROPHIC CARDIOMYOPATHY:
ECG
• Most common abnormalities includes increased in voltages consistent
with LV hypertrophy, tall R waves or Deep S wave
• ST-T changes including marked T wave inversion in lateral precordial
leads
• Left atrial enlargement
• Deep and narrow Q waves
• Diminished R wave in lateral precordial leads.
HYPERTROPHIC CARDIOMYOPATHY:
ECG
• Left ventricular hypertrophy results in increased precordial voltages
and non-specific ST segment and T-wave abnormalities.
• Asymmetrical septal hypertrophy produces deep, narrow (“dagger-
like”) Q waves in the lateral (V5-6, I, aVL) and inferior (II, III, aVF)
leads.
HYPERTROPHIC CARDIOMYOPATHY:
ECG
HYPERTROPHIC CARDIOMYOPATHY:
ECG
HYPERTROPHIC CARDIOMYOPATHY:
Microvascular
• Myocardial ischemia due to microvascular dysfunction occurs in HCM
and is important pathophysiologic component of the disease process,
promoting LV myocardial scarring and remodeling.
Family Screening Strategies

• Clinical screening of relatives in HCM families is performed in two

dimentional echocacardiography ad 12 lead electrocardiography also
CMR as well as by history taking and physical examination

• Screening evaluation are usually performed on 12-18 months basis at

the age of 12 years
Family screening strategies
HYPERTROPHIC CARDIOMYOPATHY:
Physical examination
• PE findings in patients with HCM are variable and related in large
measure to hemodynamic state.
• Initial clinical suspicion of HCM my be triggered by recognition of a
heart murmur.
HYPERTROPHIC CARDIOMYOPATHY:
Symptoms
• Dyspnea
• Fatigue
• Orthopnea
• Paroxysmal nocturnal dyspnea
• Chest pain
• Syncope/ lightheadedness
Athletes Heart and Hypertrophic
Cardiomyopathy
• Long term athletic training can increase LV diastolic cavity dimension,
wall thickness and calculated mass
Athletes Heart and Hypertrophic
Cardiomyopathy
• A diagnostic dilemma may arise in distinguishing clinically benign and
physiologic hypertrophy (consequence of athletic training) from
pathologic condition such as HCM.
Athletes Heart and Hypertrophic
Cardiomyopathy
• Clinical parameters that favor the diagnosis of HCM in trained
athletes in ambiguous “gray zone” of overlap between two condition (
maximum LV wall thickness 13mm-15mm)
• Parameters favoring physiologic athletes heart include regression of
wall thickness after a short (4-6 weeks) period of deconditioning
assessed with CMR and enlarged LV cavity size exceeding 55mm
Athletes heart vs. Hypertrophic
Cardiomyopathy
Athletes heart vs. Hypertrophic
Cardiomyopathy
Risk Stratification and Sudden Death
• Sudden death in HCM occurs at wide range of ages but most
commonly in adolescents and young adults <30 to <35 years.
• Caused by primary ventricular tachycardia and ventricular fibrillation.
Risk Stratification and Sudden Death
• Sudden death in HCM occurs at wide range of ages but most
commonly in adolescents and young adults <30 to <35 years.
• Caused by primary ventricular tachycardia and ventricular fibrillation.
Prevention of Sudden Death
• An
implantable cardioverter defibrilla
tor (ICD)
is a small device that uses
electrical shocks to help control
life-threatening, irregular
heartbeats. A common diagnosis
that results in a defibrillator is
called Sudden Cardiac Arrest or
SCA which is when the heart
suddenly stops beating. This is a
critical diagnosis that can result in
death if not managed quickly
Treatment
• Treatment for heart failure
• Treatment for atrial fibrillation
• Surgery
• Dual chamber pacing
• Alcohol septal ablation
Treatment
• Heart failure
1. Beta blockers- slowing heart rate and reducing force LV contraction,
augmenting ventricular filling and relaxation and decreasing
myocardial oxygen consumption
Propranolol, atenolol, metoprolol, nadolol-commonly used
Treatment
• Heart failure
2. Verapamil improves symptoms and exercise capacity, in patients
without marked obstruction to LV outflow probably because beneficial
effects on ventricular relaxation and filling
Treatment
• Heart failure
3. Disopyramide- some investigator favor disopyramide as third option (
in combination of beta blockers) to ameliorate symptoms when other
drugs fail to achieve symptom control
4. Diuretics-may judiciously administer in conjunction with beta
blockers or verapamil to reduce pulmonary congestion and LV filling
pressure
Treatment
• Heart failure
5. Angiotensin converting enzyme inhibitors
6. Angiotensin receptor blocker
Treatment
• Atrial fibrillation
- The most common sustained arrhythmia in HCM
- Occurs in about 20% of HCM, increasing incidence with age and linked
to enlargement of the left atrium.
Treatment
• Atrial fibrillation
1. Anticoagulant- Because of potential for clot and embolization, a low
threshold for initiation of anticoagulant therapy with warfarin is
prudent.
2. Amiodarone is considered the most effective drug in reducing AF
recurrences
3. Beta blockers and verapamil- usually administer to control heart rate
in chronic AF
4. Radio frequency ablation therapy- treatment of refractory AF
Surgery

• On the basis of extensive worldwide experienced during more than 45

years, surgical septal myomectomy remains the preferred treatment
option for patients with severe drug refractory heart failure symptoms
and marked functional disability ( NYHA classes III and IV)
Surgery
The primary objective of surgical myomectomy is reduction in heart
failure symptoms and improve quality life, by virtue of relieving outflow
obstruction

95% of patients undergoing myomectomy experienced permanent

abolition of the basal outflow gradient, without compromise of global
LV function
Surgery
At present , surgical myectomy is not recommended for asymptomatic (
or mildly asymptomatic) patients, as conclusive evidence is lacking that
prophylactic relief of obstruction is of benefit long term
Dual Chamber Pacing
• About 20 years ago, permanent dual chamber pacing was promoted
as an alternative to myomectomy for obstructive HCM patients with
refractory heart failure symptoms
Dual Chamber Pacing
Several randomized studies demonstrated that the subjectively
perceived symptomatic benefit from pacing was nor accompanied by
objective evidence of improved exercise capacity and appeared to be a
placebo effect
Alcohol Septal Ablation
• Percutaneous alcohol septal ablation, an alternative to myomectomy
involves introduction of 1- 3 ml 95% alcohol into a major septal
perforator coronary artery to create necrosis and permanent
transmural myocardial infarction in the proximal ventricular septum
Alcohol Septal Ablation
• Alcohol ablation resolves heart failure symptoms in many patients
although follow up is short compared with myomectomy, and even in
the expert center, it may be associated with procedural mortality and
complication rates exceeding those of myomectomy. Non randomized
comparative data show that gradient reduction after alcohol ablation
is similar to myomectomy than with ablation. About 20% o patient
required repeated ablation because of unsatisfactory hemodynamic
and symptomatic result o permanent pacing for complete heart block.