Cardiology An Introduction: 12 Cardiomyopathy With Joseph S. Alpert, M.D

Cardiology An introduction
12 Cardiomyopathy
With Joseph S. Alpert, M.D.
DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

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Cardiomyopathy
What is Cardiomyopathy?
Cardio = heart
Myo = muscle
Pathy = disease
• Thus, a cardiomyopathy is a disease of heart muscle
• Potential complications: heart failure, arrhythmias, sudden death
• In the past few cardiomyopathies were thought to be genetic;

Now at least 1/3 are known to be genetic

Cardiomyopathy
What is Cardiomyopathy?

Cardiomyopathy
Diseases of the Heart Muscle
• Cardiomyopathy is the 2nd most common cause of sudden death

(** Ischemic heart disease is #1**)
• Prognosis for Dilated Cardiomyopathy is poor especially when

diagnosed late in the course when heart failure signs and symptoms
are present
Two types of cardiomyopathy
1. Primary type: heart muscle disease predominantly involving the

myocardium and of unknown etiology
2. Secondary type: myocardial disease of known cause/ associated

with a systemic disease, for example, amyloidosis, chronic
alcoholic use

Cardiomyopathy
Primary cardiomyopathies
Mixed
Idiopathic dilated cardiomyopathy
Restrictive cardiomyopathy
Genetic Acquired
• Hypertrophic • Inflammatory (myocarditis)
• Arrhythmogenic right ventricular dyslapsia • Peripartum
• Glycogen storage disorders • Tachycardia-induced
• Mitochondrial myopathies
Secondary cardiomyopathies
• Infiltrative including amyloidosis • Neuromuscular/neurological

• Storage including hemochromatosis • Autoimmune including systemic
• Toxicity including drugs, chemotherapeutic agents lupus erythematosis, rheumatoid arthritis,
• Inflammatory including sarcoidosis scleroderma
• Endocrine including hypo- or hyperthyroidism diabetes

Cardiomyopathy
In 2006, the American Heart Association Primary cardiomyopathies

defined cardiomyopathies as
a heterogeneous group of diseases of the Mixed

myocardium associated with mechanical Idiopathic dilated cardiomyopathy
and/or electrical dysfunction that usually (but
Genetic
not invariably) exhibit inappropriate ventricular • Hypertrophic
Acquired
• Inflammatory (myocarditis)
• Arrhythmogenic right ventricular dyslapsia • Peripartum
hypertrophy or dilatation and are due to a • Glycogen storage disorders • Tachycardia-induced
• Mitochondrial myopathies
variety of causes that frequently are genetic
Secondary cardiomyopathies
• Infiltrative including amyloidosis

• Storage including hemochromatosis
• Toxicity including drugs, chemotherapeutic agents
• Inflammatory including sarcoidosis
• Endocrine including hypo- or hyperthyroidism diabetes
• Neuromuscular/neurological
• Autoimmune including systemic lupus erythematosis, rheumatoid arthritis, scleroderma

Cardiomyopathy
Diagramatic representation of different cardiomyopathies

Cardiomyopathy
Risk factors
Hypertension
African
Americans of Pregnancy
both genders
More common Viral Infections

in males
Alcohol abuse

Cardiomyopathy
Dilated cardiomyopathy
Characteristics
• Dilation of the cardiac chambers
• Reduction in ventricular contractile function
• Epidemiology
• The most common form of CM
• Responsible for 10,000 death and 46,000

hospitalizations per year in USA

Cardiomyopathy
Pathophysiology
• Results from a variety of factors that impair

myocardial contractile function
• Dilation is directly related to compensatory

mechanisms responding to decreased
myocardial function
• Associated with atrial and ventricular

arrhythmias in many patients (probably
responsible for the high incidence of sudden
death in the population)
• Tricuspid and Mitral regurgitation result from

chamber dilatation leading to dilated valve
structure

Cardiomyopathy
Normal Heart Heart with dilated

cardiomyopathy

Cardiomyopathy
Normal Heart Heart with dilated

Aorta Aorta cardiomyopathy
Enlarged left
atrium
Left
atrium
Right
atrium Enlarged right
atrium
Left
Right ventricle
ventricle
Enlarged right
ventricle
Thinned wall Enlarged left ventricle

Cardiomyopathy
Statistics
• Affects 5 to 8 of 100,000 people in US
• 50% of all deaths resulting from DCM

occur suddenly
• In a study conducted in 1988, 5-year

mortality rate of 50% was reported for DCM
of various etiologies with LV ejection
fraction < 50%
• A more recent study in 2000 showed

a 5-year survival rate of 75%

Cardiomyopathy
Secondary Dilated cardiomyopathy
Types
• Ischemic
• Hypertensive
• Valvular Long standing mitral or aortic

regurgitation
• Anthracycline chemotherapy
• Peripartum
• Alcohol, chronic and in high dose

Cardiomyopathy
Ischemic Dilated Cardiomyopathy
• The most common type of dilated cardiomyopathy
• Caused by coronary artery disease
• About 15% to 45% of patients who have a myocardial infarction will

develop dilatation of the left ventricle with a decrease in ejection
fraction
• Prognosis is worse for ischemic cardiomyopathy, than for

non-ischemic cardiomyopathies

Cardiomyopathy
Ischemic Dilated Cardiomyopathy Diagnosis
Clinical presentation Physical exam
• Symptoms of heart failure: dyspnea, fatigue • Enlarged and displaced apex impulse
• Some patients are asymptomatic • Pulse pressure is narrow
• ECG reveals nonspecific changes • Jugular venous pressure is increased
• Sinus tachycardia/atrial fibrillation • 3rd or 4th heart sound are common
• Ventricular arrhythmias • Mitral/ tricuspid regurgitation may be heard
• Left atrial abnormality

Cardiomyopathy
Ischemic Dilated Cardiomyopathy Diagnosis
Diagnostic testing
• Confirm abnormal cardiac structure with echocardiography or radionuclide

ventriculography
• MRI is being used increasingly to define underlying metabolic abnormalities and

the degree of myocardial damage and fibrosis
• Echo is helpful to differentiate from other forms of cardiomyopathy
• Angiography reveals dilated, hypokinetic left ventricle with normal coronary

arteries and often with some degree of mitral regurgitation

Cardiomyopathy
Ischemic Dilated Cardiomyopathy Therapy
• Therapeutic strategies include beta

blockade, renin-angiotensin axis blockade,
diuretics, and mineralocorticoid blockade
• Implantation of a special form of pacemaker

which re-coordinates left ventricular
contraction is effective in patients with a
wide QRS complex on the EKG
• Ventricular assist devices, artificial heart, and

cardiac transplantation are performed in
highly selected patients

Cardiomyopathy
Hypertrophic cardiomyopathy
Hypertrophic Cardiomyopathy (HCM)
• Usually genetic with autosomal dominance
• Most common cause of sudden death in young athletes in

North America
• Prevalence approx. 100 per 100,000 population
• Characterized by inappropriate and elaborate LV hypertrophy with

misalignment of the myocardial fibers
• Hypertrophy may be generalized or confined largely to

interventricular septum

Cardiomyopathy
Hypertrophic Cardiomyopathy (HCM)

Pathophysiology
• Due to single point mutation in one of the

many genes that encode myocardial
contractile proteins
• There are now over 1000 mutations

documented that can lead to this form of CM
• In many individuals, HCM is benign and

consistent with a long life
• Some forms are associated with a high risk

for sudden death

Cardiomyopathy
Normal Hypertrophic
Heart cardiomyopathy
Enlargement of
the heart muscle
Right ventricle Left ventricle

Cardiomyopathy
• Primarily affects diastolic function (**filling***)
• Features of HCM
• Asymmetrical LV hypertrophy
• Hypertrophy of interventricular
septum (HOCM) **
• Rapid, forceful contractions of LV
• Impaired relaxation
• Obstruction of LV outflow **

Cardiomyopathy
Symptoms
• Exertional angina
• Dyspnea on effort; Syncope on effort
• Sudden death
Signs
• Very brisk pulse occasionally with a double impulse
• Palpable left ventricular heave
Symmetric or
concentric
• Double impulse at apex hypertrophy
• Mid-systolic murmur that increases with standing or Valsalva maneuver
• Pansystolic murmur of mitral regurgitation is sometimes heard

Cardiomyopathy
Treatment
• B-blockers, verapamil (calcium channel
blocker), disopyramide, amiodarone have
been found useful
• Beta blockers in high dose is the #1 therapy
to be tried
• No pharmacological treatment is known to
improve prognosis

Cardiomyopathy
Treatment
• Atrial and ventricular arrhythmias are
common and often respond to anti-
arrhythmic drug therapy including beta
blockade
• Outflow tract obstruction can be improved by
• Partial surgical resection (myectomy)
or
• Iatrogenic infarction of basal septum
(septal ablation with alcohol)
• Implantable cardiac defibrillation (ICD) for
patients with risk of sudden death

Cardiomyopathy
Alcohol Septal Ablation

Cardiomyopathy
• Rare condition
• Ventricular filling is impaired because

ventricles are stiff
• Leads to high atrial pressure with atrial

hypertrophy, dilatation and later atrial
fibrillation
• High atrial pressure leads to lung congestion

and dyspnea

Cardiomyopathy
• Amyloidosis is the most common cause
• Other forms
• Idiopathic perimyocyte fibrosis
• Familial restrictive cardiomyopathy

Cardiomyopathy
Normal Restrictive
Heart cardiomyopathy

Cardiomyopathy
Diagnosis is often difficult Therapy
• Difficult to distinguish from constrictive • Diuretics to combat pulmonary and

pericarditis systemic congestion
• Echo/doppler and MRI can make the • Usually the prognosis is poor
diagnosis many times, BUT
endomyocardial biopsy may need to be • Cardiac transplantation is indicated for
done to confirm the diagnosis patients with severe heart failure secondary
to restrictive cardiomyopathy

Cardiomyopathy
Arrhythmogenic Right Ventricular Dysplasia (ARVD)
Characteristics
• Patches of the right ventricular myocardium

are replaced with fibrous and fatty tissue
• Inherited as autosomal dominant trait
• Uncommon
• Dominant clinical problems are:

ventricular arrhythmias, sudden death, and
right sided heart failure
• ECG shows inverted T waves in the right

precordial leads
• MRI is the most useful diagnostic tool

Cardiomyopathy
Therapy
Left Ventricular Assist Device (LVAD)

Cardiomyopathy
Therapy
Cardioverter-Defibrillator

Cardiomyopathy
Therapy
Heart Transplant

List of references
1. Lange, Veronika: BASICS Kardiologie, 3. Aufl. 2013.
2. Krautzig, Steffen, Renz-Polster, Herbert: Basislehrbuch Innere Medizin, 5.Aufl.
3. Spes, Christoph, Klauss, Volker: Facharztprüfung Kardiologie.
4. Classen, Meinhard, Diehl, V., Kochsiek, K., Hallek, Michael, Böhm, Michael, Schmiegel, Wolff:
Innere Medizin, 6.A.
5. Stierle, Ulrich, Hartmann, Franz: Klinikleitfaden Kardiologie, 5. Aufl.

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Cardiology An introduction

With Joseph S. Alpert, M.D.

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

• Thus, a cardiomyopathy is a disease of heart muscle

• Potential complications: heart failure, arrhythmias, sudden death

• In the past few cardiomyopathies were thought to be genetic;

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

• Cardiomyopathy is the 2nd most common cause of sudden death

• Prognosis for Dilated Cardiomyopathy is poor especially when

Two types of cardiomyopathy

1. Primary type: heart muscle disease predominantly involving the

2. Secondary type: myocardial disease of known cause/ associated

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

• Infiltrative including amyloidosis • Neuromuscular/neurological

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

In 2006, the American Heart Association Primary cardiomyopathies

a heterogeneous group of diseases of the Mixed

• Infiltrative including amyloidosis

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

Diagramatic representation of different cardiomyopathies

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

More common Viral Infections

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

• Dilation of the cardiac chambers

• Reduction in ventricular contractile function

• The most common form of CM

• Responsible for 10,000 death and 46,000

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

• Results from a variety of factors that impair

• Dilation is directly related to compensatory

• Associated with atrial and ventricular

• Tricuspid and Mitral regurgitation result from

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

Normal Heart Heart with dilated

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

Normal Heart Heart with dilated

Thinned wall Enlarged left ventricle

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

• Affects 5 to 8 of 100,000 people in US

• 50% of all deaths resulting from DCM

• In a study conducted in 1988, 5-year

• A more recent study in 2000 showed

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

• Valvular Long standing mitral or aortic

• Alcohol, chronic and in high dose

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

Ischemic Dilated Cardiomyopathy

• The most common type of dilated cardiomyopathy

• Caused by coronary artery disease

• About 15% to 45% of patients who have a myocardial infarction will

• Prognosis is worse for ischemic cardiomyopathy, than for

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

Ischemic Dilated Cardiomyopathy Diagnosis

Clinical presentation Physical exam

• Some patients are asymptomatic • Pulse pressure is narrow

• ECG reveals nonspecific changes • Jugular venous pressure is increased

• Sinus tachycardia/atrial fibrillation • 3rd or 4th heart sound are common

• Ventricular arrhythmias • Mitral/ tricuspid regurgitation may be heard

• Left atrial abnormality

DARRYL JOHN PASAMBA, pasambad1306@uerm.edu.ph

Ischemic Dilated Cardiomyopathy Diagnosis

• Confirm abnormal cardiac structure with echocardiography or radionuclide

• Primarily affects diastolic function (filling*)