Cardiomyopathies are diseases of the heart muscle that are classified based on etiology and functional characteristics. The main types are dilated, hypertrophic, restrictive, and arrhythmogenic right ventricular dysplasia. Symptoms vary depending on the type but often include heart failure symptoms like shortness of breath. Treatment focuses on managing underlying causes, controlling symptoms, preventing worsening of the disease, and reducing complications through medications, lifestyle changes, and procedures.

1. CARDIOMYOPATHY

2. Cardiomyopathies constitute a group of diseases,
in which the dominant feature is involvement of
the heart muscle itself.

3. • WHO classification (etiologic classification)
– Primary
• Idiopathic (D,R,H)
• Familial (D,R,H)
• Eosinophilic endomyocardial disease (R)
– Secondary or specific
• Infective (D)
• Metabolic (D)
• Deficiency (D)
• Connective tissue disorders (D)
• Infiltrations and granulomas (R,D)
• Neuromuscular (D)
• Sensitivity and toxic reactions (D)
• Peripartum heart disease (D)

4. Functional classification
• Dilated (formerly called congestive)
The most common form, characterized by ventricular dysfunction
and often symptoms of CHF.
• Hypertrophic
Recognized by inappropriate left ventricular hypertrophy, often
with asymmetrical involvement of the interventricular septum,
usually with preserved or enhanced contractile function.
• Restrictive
The most common form in western countries, marked by impaired
diastolic filling, in some cases with endocardial scarring of the
ventricle.
• Arrhythmogenic right ventricular dysplasia (ARVD)
• Obliterative cardiomyopathy

6. Risk factors
 Having a family history of CM, heart failure or sudden cardiac death.
 Having a disease or condition that can lead to CM such as:
• CAD, previous heart attack, myocarditis
• Disease that can damage the heart (e.g. hemochromatosis,
sarcoidosis or amyloidosis)
 Long term alcoholism
 Long term high blood pressure
 Diabetes and other metabolic diseases
 Obesity (increases the risk of developing heart failure, as does sleep
apnea).

7. Causes
• Idiopathic (10), especially in children.
• Inherited or due to any other disease or condition.
Dilated CM
 Inherited
 Due to certain diseases or conditions such as:
• CAD and heart attacks (ischemic CM)
• Infections (myocarditis)
• Alcohol (alcoholic CM)
• Pregnancy (Peripartum CM)
• Certain toxins such as cobalt
• Certain drugs such as cocaine, amphetamines, doxorubicin.
• Diseases such as diabetes and thyroid diseases.

8. Hypertrophic CM
Inherited
Can develop over time (because of high BP or aging)
Other causes are unknown
Restrictive CM
Hemochromatosis
Amyloidosis
Sarcoidosis
Connective tissue disorder

9. Pathophysiology, clinical features and
examination

10. Dilated CM
• Dilated cardiomyopathy is the most common form of cardiomyopathy.
• Generally occurs in adults aged 20 to 60 years.
• Men are more likely than women to develop dilated cardiomyopathy.
• Dilated cardiomyopathy usually starts in the left ventricle, where the heart
muscle begins to dilate or stretch and become thinner. This leads to
enlargement of the inside of the ventricle. The problem often spreads to the
right ventricle and then to the atria as the disease gets worse.
• When the chambers dilate, the heart can't pump blood very well (i.e. LV/RV
systolic pump function is impaired), leading to progressive cardiac enlargement
and hypertrophy, a process called remodelling. Symptoms of CHF typically
appear only after remodelling has been ongoing for sometime (months or
years).
• Dilated cardiomyopathy also can lead to heart valve problems, arrhythmias, and
blood clots in the heart. Having advanced dilated cardiomyopathy is a common
reason for needing a heart transplant.

12. Clinical features
• Some people with cardiomyopathy never have symptoms, and others
have no symptoms in the early stages of the disease.
• As cardiomyopathy progresses and the heart weakens, signs and
symptoms of heart failure usually appear. These signs and symptoms
include:
– Tiredness
– Weakness
– Shortness of breath after exercise or even at rest
– Swelling of the abdomen, legs, ankles, and feet
• Other signs and symptoms can include dizziness, lightheadedness,
fainting during exercise, abnormal heart rhythms (arrhythmias), and an
extra or unusual sound heard during the heartbeat (heart murmur).

13. Examination
• In patients with advanced disease:
– Pulse pressure is narrow
– Jugular venous pressure is elevated.
– Third and fourth heart sounds are common
– Mitral or tricuspid regurgitation may occur.
• Chest X-ray : Moderate to marked cardiac silhouette enlargement.
Pulmonary venous hypertension.
• ECG : ST-segment and T-wave abnormalities.
• Echo : Left ventricular dilatation and dysfunction.

14. • Radionuclide ventriculogram : Left ventricular dilatation and
dysfunction.
• Cardiac catheterization : Left ventricular dilatation and dysfunction.
Elevated left and often right sided filling pressures. Diminished cardiac
output

15. Hypertrophic CM
• Hypertrophic cardiomyopathy occurs when the heart muscle thickens
abnormally. The thickening generally happens in the left ventricle.
• This type of cardiomyopathy can affect people of any age.
• Hypertrophic cardiomyopathy can be obstructive or nonobstructive.
• In the obstructive type, the septum thickens and bulges into the left
ventricle. This bulge blocks the flow of blood out of the ventricle. The
ventricle must work much harder to pump blood past the blockage and
out to the body. Symptoms can include chest pain, dizziness, shortness
of breath, or fainting.
• Obstructive hypertrophic cardiomyopathy also can affect the heart's
mitral valve, causing blood to leak backward through the valve. This is
due to abnormal systolic anterior motion of the anterior mitral valve
leaflet.

17. • In nonobstructive hypertrophic cardiomyopathy, the thickened heart
muscle does not block the flow of blood out of the ventricle. The entire
ventricle may become thicker (symmetric ventricular hypotrophy) or it
may happen only at the bottom of the heart (apical hypertrophy). The
right ventricle also may be affected.
• In both kinds of hypertrophic cardiomyopathy, the thickened muscle
makes the inside of the left ventricle smaller so that it holds less blood.
The walls of the ventricles also may become stiff. As a result, they are
less able to relax and fill with blood. This causes increased pressure in
the ventricles and the blood vessels of the lungs. Changes also occur to
the cells in the damaged heart muscle. This may interfere with the
heart's electrical signals, leading to arrhythmias.

18. Clinical features
• The clinical course is highly variable.
• Patients are asymptomatic or mildly symptomatic.
• 1st clinical manifestation of the disease may be sudden death in children
and young adults (during or after physical exertion).
• In symptomatic patients : dyspnea, angina pectoris, fatigue, syncope.

19. Examination
• A double or triple apical precordial impulse.
• Rapidly rising carotid arterial pulse.
• Fourth heart sound.
• Hallmark of obstructive HCM is a systolic murmur (best heard at lower
left sternal border).
• Dynamic pressure gradient (may change between examinations and even
from beat to beat). This can be because of
1) increased left ventricular contractility, 2) decreased preload, 3)
decreased afterload.

20. • Chest X-ray : Mild to moderate cardiac silhouette enlargement.
• ECG : ST-segment and T-wave abnormalities. Left ventricular
hypertrophy. Abnormal Q waves.
• Echo : Asymmetric septal hypertrophy. Systolic anterior motion of the
mitral valve.
• Radionuclide ventriculogram : Vigorous systolic function. Perfusion
defect.
• Cardiac catheterization : Vigorous systolic function. Dynamic left
ventricular outflow obstruction. Elevated left and right sided filling
pressures.

21. Restrictive CM
• Restrictive cardiomyopathy mostly affect older adults.
• The hallmark of the RCM is abnormal diastolic function.
• In this cardiomyopathy, the ventricles become stiff and rigid due to
replacement of the normal heart muscle with abnormal tissue, such as
scar tissue. As a result, the ventricles cannot relax normally and expand
to fill with blood, which causes the atria to become enlarged.
• Eventually, blood flow in the heart is reduced, and complications such
as heart failure or arrhythmias occur.

23. Clinical features
• Exercise intolerance
• Dyspnea
• Dependent edema, ascites
• Enlarged, tender and often pulsatile liver

24. Examination
• Dependent edema, ascites
• Enlarged, tender and often pulsatile liver.
• Elevated JVP (it may rise with inspiration i.e. Kussmaul’s sign).
• 3rd and 4th heart sounds.
• Easily palpable apex impulse.

25. • Chest X-ray : Mild cardiac silhouette enlargement.
• ECG : Low voltage conduction defects.
• Echo : Increased left ventricular wall thickness. Normal or mildly reduced
systolic function.
• Radionuclide ventriculogram : Normal or mildly reduced systolic
function.
• Cardiac catheterization : Normal or mildly reduced systolic function.
Elevated left and right sided filling pressures.

26. Treatment
• Not everyone with cardiomyopathy needs treatment. People who have
no symptoms may not need treatment. In some cases, dilated
cardiomyopathy that comes on suddenly may even go away on its own.
• For other people with cardiomyopathy, treatment is necessary.
• Specific treatments depend on the type of cardiomyopathy, how severe
the symptoms and complications are, and the age and overall health of
the person.

27. • Goals of Treatment
The main goals of treating cardiomyopathy are to:
– Manage any conditions that cause or contribute to the
cardiomyopathy
– Control symptoms so that the person can live as normally as possible
– Stop the disease from getting worse
– Reduce complications and the chance of sudden cardiac death
Specific Types of Treatment
Treatments for cardiomyopathy may include medicines, surgery,
nonsurgical procedures, and lifestyle changes.

28. • Medicines
– Diuretics, remove excess fluid and sodium from the body.
– Angiotensin-converting enzyme (ACE) inhibitors, lower BP and reduce stress on
the heart.
– Beta-blockers, slow the heart rate, also lower BP.
– Calcium channel blockers, slow heartbeat, also lower BP.
– Digoxin, increases the force of heart contractions and slows the heartbeat.
– Anticoagulants, prevent blood clots & are often used in the treatment of dilated
cardiomyopathy.
– Antiarrhythmia medicines, keep the heart beating in a normal rhythm.
– Antibiotics help to prevent endocarditis
– Corticosteroids, reduce inflammation.

29. • Surgery
– Septal myectomy - Hypertrophic obstructive CM and severe symptoms.
Used in younger patients and when medicines aren't working well.
(In septal myectomy, a surgeon removes part of the thickened septum that
is bulging into the left ventricle. This widens the pathway in the ventricle
that leads to the aortic valve and improves blood flow through the heart
and out to the body. The tissue that is removed does not grow back. If
necessary, the mitral valve can be repaired or replaced at the same time).
– Surgically implanted devices. Pacemaker, biventricular pacemaker
– A left ventricular assist device (LVAD) can be used as a long-term therapy or
as a short-term treatment for people who are waiting for a heart
transplant.
– An implantable cardioverter defibrillator (ICD) is used in people who are at
risk of life-threatening arrhythmia or sudden cardiac death.
– Heart transplant. It is a last resort for people with heart failure when all
other treatments have failed.

30. • Nonsurgical Procedure
– Alcohol septal ablation.
In this procedure, ethanol (a type of alcohol) is injected through a
catheter into the small artery that supplies blood to the thickened
area of heart muscle. The alcohol kills the cells and the thickened
tissue shrinks to a more normal size. Blood can flow freely through
the pathway in the ventricle that leads to the aortic valve, and
symptoms improve.

31. • Lifestyle Changes
These changes may help reduce symptoms. Lifestyle changes may
include:
• Quitting smoking
• Losing excess weight
• Eating a low-salt diet
• Getting moderate exercise, such as walking, and avoiding strenuous
exercise
• Avoiding the use of alcohol and illegal drugs
• Getting enough sleep and rest
• Reducing stress
• Treating underlying conditions, such as diabetes and high blood pressure

32. Arrhythmogenic Right Ventricular Dysplasia
• Arrhythmogenic right ventricular dysplasia (ARVD) is a rare type of
cardiomyopathy.
• It is a unique familial CM marked by progressive replacement of the
right ventricular wall with adipose tissue and with scar tissue.
• This process causes problems in the heart's electrical signaling, resulting
in arrhythmias.
• Symptoms include a feeling of strong or irregular heartbeats
(palpitations) and fainting after exercise.

33. • ARVD usually develops in teens or young adults and is often the cause
of sudden cardiac death in young athletes.
• ARVD is thought to be an inherited disease.
• Catheter ablation of arrhythmia sites and insertion of an implantable
cardioverter - defibrillator are often employed.

34. Obliterative Cardiomyopathy
• This disease involves the endocardium of one or both ventricles. The
pathogenesis is not clear but some form of endocardial damage appears
to trigger thrombosis and elaborate fibrosis which gradually obliterates
the ventricular cavities.
• The mitral and tricuspid valves are often involved and may become
regurgitant.
• Heart failure and pulmonary and systemic embolism are prominent
features.
• In temperate climates the endocardial damage is usually attributable to
some form of eosinophilia (e.g. eosinophilic leukemia) but this does not
seem to be the case in the tropical countries, where the disease can be
responsible for up to 10% of cardiac deaths.

35. • Treatment is unsatisfactory and mortality is high (50% at 2 years).
• Anticoagulation and antiplatelet therapy are usually advisable and
diuretics may help to treat the symptoms of heart failure.
• Surgery (tricuspid and/or mitral valve replacement with decortication of
the endocardium) may be helpful in selected cases.