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CARDIOMYOPATHY
Cardiomyopathies constitute a group of diseases,
in which the dominant feature is involvement of
the heart muscle itself.
• WHO classification (etiologic classification)
– Primary
• Idiopathic (D,R,H)
• Familial (D,R,H)
• Eosinophilic endomyoca...
Functional classification
• Dilated (formerly called congestive)
The most common form, characterized by ventricular dysfun...
Risk factors
 Having a family history of CM, heart failure or sudden cardiac death.
 Having a disease or condition that ...
Causes
• Idiopathic (10), especially in children.
• Inherited or due to any other disease or condition.
Dilated CM
 Inher...
Hypertrophic CM
Inherited
Can develop over time (because of high BP or aging)
Other causes are unknown
Restrictive CM
...
Pathophysiology, clinical features and
examination
Dilated CM
• Dilated cardiomyopathy is the most common form of cardiomyopathy.
• Generally occurs in adults aged 20 to 60 ...
Clinical features
• Some people with cardiomyopathy never have symptoms, and others
have no symptoms in the early stages o...
Examination
• In patients with advanced disease:
– Pulse pressure is narrow
– Jugular venous pressure is elevated.
– Third...
• Radionuclide ventriculogram : Left ventricular dilatation and
dysfunction.
• Cardiac catheterization : Left ventricular ...
Hypertrophic CM
• Hypertrophic cardiomyopathy occurs when the heart muscle thickens
abnormally. The thickening generally h...
• In nonobstructive hypertrophic cardiomyopathy, the thickened heart
muscle does not block the flow of blood out of the ve...
Clinical features
• The clinical course is highly variable.
• Patients are asymptomatic or mildly symptomatic.
• 1st clini...
Examination
• A double or triple apical precordial impulse.
• Rapidly rising carotid arterial pulse.
• Fourth heart sound....
• Chest X-ray : Mild to moderate cardiac silhouette enlargement.
• ECG : ST-segment and T-wave abnormalities. Left ventric...
Restrictive CM
• Restrictive cardiomyopathy mostly affect older adults.
• The hallmark of the RCM is abnormal diastolic fu...
Clinical features
• Exercise intolerance
• Dyspnea
• Dependent edema, ascites
• Enlarged, tender and often pulsatile liver
Examination
• Dependent edema, ascites
• Enlarged, tender and often pulsatile liver.
• Elevated JVP (it may rise with insp...
• Chest X-ray : Mild cardiac silhouette enlargement.
• ECG : Low voltage conduction defects.
• Echo : Increased left ventr...
Treatment
• Not everyone with cardiomyopathy needs treatment. People who have
no symptoms may not need treatment. In some ...
• Goals of Treatment
The main goals of treating cardiomyopathy are to:
– Manage any conditions that cause or contribute to...
• Medicines
– Diuretics, remove excess fluid and sodium from the body.
– Angiotensin-converting enzyme (ACE) inhibitors, l...
• Surgery
– Septal myectomy - Hypertrophic obstructive CM and severe symptoms.
Used in younger patients and when medicines...
• Nonsurgical Procedure
– Alcohol septal ablation.
In this procedure, ethanol (a type of alcohol) is injected through a
ca...
• Lifestyle Changes
These changes may help reduce symptoms. Lifestyle changes may
include:
• Quitting smoking
• Losing exc...
Arrhythmogenic Right Ventricular Dysplasia
• Arrhythmogenic right ventricular dysplasia (ARVD) is a rare type of
cardiomyo...
• ARVD usually develops in teens or young adults and is often the cause
of sudden cardiac death in young athletes.
• ARVD ...
Obliterative Cardiomyopathy
• This disease involves the endocardium of one or both ventricles. The
pathogenesis is not cle...
• Treatment is unsatisfactory and mortality is high (50% at 2 years).
• Anticoagulation and antiplatelet therapy are usual...
Cardiomyopathy
Cardiomyopathy
Cardiomyopathy
Cardiomyopathy
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short description of cardiomyopathy, cardiomyopathy means the disease of the muscles of heart.

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Cardiomyopathy

  1. 1. CARDIOMYOPATHY
  2. 2. Cardiomyopathies constitute a group of diseases, in which the dominant feature is involvement of the heart muscle itself.
  3. 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. 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
  5. 5. 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).
  6. 6. 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.
  7. 7. 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
  8. 8. Pathophysiology, clinical features and examination
  9. 9. 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.
  10. 10. 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).
  11. 11. 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.
  12. 12. • 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
  13. 13. 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.
  14. 14. • 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.
  15. 15. 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.
  16. 16. 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.
  17. 17. • 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.
  18. 18. 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.
  19. 19. Clinical features • Exercise intolerance • Dyspnea • Dependent edema, ascites • Enlarged, tender and often pulsatile liver
  20. 20. 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.
  21. 21. • 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.
  22. 22. 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.
  23. 23. • 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.
  24. 24. • 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.
  25. 25. • 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.
  26. 26. • 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.
  27. 27. • 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
  28. 28. 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.
  29. 29. • 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.
  30. 30. 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.
  31. 31. • 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.
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short description of cardiomyopathy, cardiomyopathy means the disease of the muscles of heart.

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