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Cardiomyopathy
1. โข Cardiomyopathies
โข Definition
โA primary disorder of the heart muscle that causes abnormal myocardial performance and is not
the result of disease or dysfunction of other cardiac structures โฆ myocardial infarction, systemic
hypertension, valvular stenosis or regurgitationโ
Causes
๏ฎ Ischemic
๏ฎ Valvular
๏ฎ Hypertensive
๏ฎ Inflammatory
๏ฎ Metabolic
๏ฎ Inherited
๏ฎ Toxic reactions
๏ฎ Peripartum
Dilated Cardiomyopathy
The cause of dilated cardiomyopathy often isn't known. As many as one-third of the people who have
dilated cardiomyopathy inherit it from their parents.
Certain diseases, conditions, and substances also can cause the disease, such as:
๏ท Coronary heart disease, heart attack, high blood pressure, diabetes, thyroid disease, viral hepatitis,
and HIV
๏ท Infections, especially viral infections that inflame the heart muscle
๏ท Alcohol, especially if you also have a poor diet
๏ท Complications during the last month of pregnancy or within 5 months of birth
๏ท Certain toxins, such as cobalt
๏ท Certain drugs (such as cocaine and amphetamines) and two medicines used to treat cancer
(doxorubicin and daunorubicin)
Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy (HCM) usually is inherited. It's caused by a mutation (change) in some of
the genes in heart muscle proteins. HCM also can develop over time because of high blood pressure or
aging.
Other diseases, such as diabetes or thyroid disease, also can cause HCM. Sometimes the cause of the
disease isn't known.
2. Restrictive Cardiomyopathy
Certain diseases, conditions, and factors can cause restrictive cardiomyopathy, including:
๏ท Hemochromatosis . This is a disease in which too much iron builds up in your body. The extra
iron is toxic to the body and can damage the organs, including the heart.
๏ท Sarcoidosis . This disease causes inflammation and can affect various organs in the body. an
abnormal immune response may cause sarcoidosis.
๏ท Amyloidosis. This is a disease in which abnormal proteins build up in the body's organs,
including the heart.
๏ท Connective tissue disorders.
๏ท Some cancer treatments, such as radiation and chemotherapy.
Major Risk Factors
Certain diseases, conditions, or factors can raise your risk for cardiomyopathy. Major risk factors include:
๏ท A family history of cardiomyopathy, heart failure, or sudden cardiac arrest (SCA)
๏ท A disease or condition that can lead to cardiomyopathy, such as coronary heart disease, heart
attack, or a viral infection that inflames the heart muscle
๏ท Diabetes or other metabolic diseases, or severe obesity
๏ท Diseases that can damage the heart, such as hemochromatosis, sarcoidosis, or amyloidosis
๏ท Long-term alcoholism
๏ท Long-term high blood pressure
โข WHO Classification
โข Unknown cause
(primary)
โ Dilated
โ Hypertrophic
โ Restrictive
โ unclassified
โข Specific heart muscle disease (secondary)
โ Infective
โ Metabolic
โ Systemic disease
โ Heredofamilial
โ Sensitivity
3. โ Toxic
โข Functional Classification
โข Dilatated (congestive, DCM, IDC)
โ ventricular enlargement and syst dysfunction
โข Hypertrophic (IHSS, HCM, HOCM)
โ inappropriate myocardial hypertrophy
in the absence of HTN or aortic stenosis
โข Restrictive (infiltrative)
โ abnormal filling and diastolic function
โ Idiopathic Dilated Cardiomyopathy
โข IDC - Definition
โข a disease of unknown etiology that principally affects the myocardium
โข LV dilatation and systolic dysfunction
โข pathology
โ increased heart size and weight
โ ventricular dilatation, normal wall thickness
โ heart dysfunction out of portion to fibrosis
โข Incidence and Prognosis
โข Highest incidence in middle age
โ blacks 2x more frequent than whites
โ men 3x more frequent than women
โข 3-10 cases per 100,000
โข 20,000 new cases per year in the U.S.A.
โข complete recovery is rare
โข Clinical Manifestations
โข symptoms may be gradual in onset
โข Symptoms of heart failure
4. โ pulmonary congestion (left HF)
dyspnea (rest, exertional, nocturnal), orthpnea
โ systemic congestion (right HF)
edema, nausea, abdominal pain, nocturia
โ low cardiac output
fatigue and weakness
โข hypotension, tachycardia, tachypnea, JVD
โข diagnosis
โข History and Physical Examination
โข Cardiac Imaging
โข Chest radiogram
โข Electrocardiogram
โข 24-hour ambulatory ECG (Holter)
โ lightheadedness, palpitation, syncope
โข Two-dimensional echocardiogram
โข Radionuclide ventriculography
โข Cardiac catheterization
โ age >40, ischemic history, high risk profile, abnormal ECG
โข Management of DCM
โข Limit activity based on functional status
โข salt restriction of a 2-g Na+
(5g NaCl) diet
โข fluid restriction for significant low Na+
โข initiate medical therapy
โ ACE inhibitors, diuretics
โ digoxin, carvedilol
โ hydralazine / nitrate combination
โข Management of DCM
โข consider adding ร-blocking agents if symptoms persists
5. โข anticoagulation for EF <30%, history of thromboemoli, presence of mural thrombi
โข intravenous dopamine, dobutamine and/or phosphodiesterase inhibitors
โข cardiac transplantation
โข Hypertrophic Cardiomyopathy
โข Hypertrophic Cardiomyopathy
โข Hypertrophic cardiomyopathy (HCM) is very common and can affect people of any age. About 1
out of every 500 people has HCM. It affects men and women equally.
โข HCM is a common cause of sudden cardiac arrest (SCA) in young people, including young
athletes.
โข HCM occurs if heart muscle cells enlarge and cause the walls of the ventricles (usually the left
ventricle) to thicken. Despite this thickening, the ventricle size often remains normal. However,
the thickening may block blood flow out of the ventricle. If this happens, the condition is called
obstructive hypertrophic cardiomyopathy.
โข Sometimes, the septum thickens and bulges into the left ventricle. This also can block blood flow
out of the left ventricle. (The septum is the wall that divides the left and right sides of the heart.)
โข If a blockage occurs, the ventricle must work hard to pump blood to the body. Symptoms can
include chest pain, dizziness, shortness of breath, or fainting.
โข HCM also can affect the heart's mitral (MI-trul) valve, causing blood to leak backward through
the valve.
โข Sometimes the thickened heart muscle doesn't block blood flow out of the left ventricle. This is
called nonobstructive hypertrophic cardiomyopathy. The entire ventricle may thicken, or the
thickening may happen only at the bottom of the heart. The right ventricle also may be affected.
โข In both types of HCM (obstructive and nonobstructive), the thickened muscle makes the inside of
the left ventricle smaller, so it holds less blood. The walls of the ventricle also may stiffen. As a
result, the ventricle is less able to relax and fill with blood.
โข These changes can raise blood pressure in the ventricles and the blood vessels of the lungs.
Changes also occur to the cells in the damaged heart muscle. This may disrupt the heart's
electrical signals and lead to arrhythmias.
โข Some people who have HCM have no signs or symptoms. The disease doesn't affect their lives.
Others have severe symptoms and complications. For example, they may have shortness of
breath, serious arrhythmias, or an inability to exercise.
โข Rarely, people who have HCM can have SCA during very vigorous physical activity. The
physical activity can trigger dangerous arrhythmias. If you have HCM, ask your doctor what
types and amounts of physical activity are safe for you.
โข Restrictive Cardiomyopathy
โข Restrictive cardiomyopathy tends to mostly affect older adults. With this disease, the ventricles
become stiff and rigid. This happens because abnormal tissue, such as scar tissue, replaces the
normal heart muscle.
โข As a result, the ventricles can't relax normally and fill with blood, and the atria become enlarged.
Over time, blood flow in the heart is reduced. This can lead to problems such as heart failure or
arrhythmias.
โข
โข First described by the French and Germans around 1900
โข uncommon with occurrence of 0.02 to 0.2%
6. โข a hypertrophied and non-dilated left ventricle in the absence of another disease
โข small LV cavity, asymmetrical septal hypertrophy (ASH), systolic anterior motion of the mitral
valve leaflet (SAM)
โข Familial HCM
โข occurs as autosomal dominant in 50%
โข Pathophysiology
โข Systole
โ dynamic outflow tract gradient
โข Diastole
โ impaired diastolic filling, ๏ญ filling pressure
โข Myocardial ischemia
โ ๏ญ muscle mass, filling pressure, O2 demand
โ ๏ฏ vasodilator reserve, capillary density
โ abnormal intramural coronary arteries
โ systolic compression of arteries
โข Clinical Manifestation
โข Asymptomatic, echocardiographic finding
โข Symptomatic
โ dyspnea in 90%
โ angina pectoris in 75%
โ fatigue, pre-syncope, syncope
๏ญ risk of SCD in children and adolescents
โ palpitation, PND, CHF, dizziness less frequent
โข Increase in Gradient and Murmur
Management
โข beta-adrenergic blockers
โข calcium antagonist
7. โข disopyramide
โข amiodarone, sotolol
โข DDD pacing
โข myotomy-myectomy
โข plication of the anterior mitral leaflet
โข Other Causes of Hypertrophy
โข
โข Restrictive Cardiomyopathy
โข Hallmark: abnormal diastolic function
โข rigid ventricular wall with impaired ventricular filling
โข bear some functional resemblance to constrictive pericarditis
โข importance lies in its differentiation from operable constrictive pericarditis
โข Classification
โข Idiopathic
โข Myocardial
1. Noninfiltrative
โ Idiopathic
โ Scleroderma
2. Infiltrative
โ Amyloid
โ Sarcoid
โ Gaucher disease
โ Hurler disease
3. Storage Disease
โ Hemochromatosis
โ Fabry disease
8. โ Glycogen storage
โข Endomyocardial
โ endomyocardial fibrosis
โ Hyperesinophilic synd
โ Carcinoid
โ metastatic malignancies
โ radiation, anthracycline
โข Clinical Manifestations
โข Symptoms of right and left heart failure
โข Jugular Venous Pulse
โ prominent x and y descents
โข Echo-Doppler
โข Treatment
โข No satisfactory medical therapy
โข Drug therapy must be used with caution
โ diuretics for extremely high filling prssures
โ vasodilators may decrease filling pressure
โ ? Calcium channel blockers to improve diastolic compliance
โ digitalis and other inotropic agents are not indicated
Septal myectomy. In a septal myectomy, your surgeon removes part of the overgrown heart muscle
wall (septum) dividing the two lower heart chambers (ventricles) to improve blood flow and reduce your
symptoms.
Septal ablation. In septal ablation, your doctor injects an alcohol solution into an artery supplying
blood to the thickened heart muscle to destroy part of the heart muscle. This procedure may improve
blood flow and reduce your symptoms.
Implantable cardioverter-defibrillator (ICD). Your doctor may place an ICD under the skin in your
chest to prevent sudden cardiac arrest due to irregular heart rhythms (arrhythmias). An ICD sends
electronic signals to your heart when your heart rate reaches a certain limit or goes very high.
โ Pacemaker. Your doctor may place a pacemaker under the skin near your collarbone
to control and monitor your heart rhythm.
Lifestyle Changes
9. Your doctor may suggest lifestyle changes to manage a condition that's causing your cardiomyopathy.
These changes can help reduce symptoms.
Healthy Diet and Physical Activity
A healthy diet and physical activity are part of a healthy lifestyle. A healthy diet includes a variety of
fruits, vegetables, and grains; half of your grains should come from whole-grain products.
Choose foods that are low in saturated fat, trans fat, and cholesterol. Healthy choices include lean meats,
poultry without skin, fish, beans, and fat-free or low-fat milk and milk products.
Choose and prepare foods with little sodium (salt). Too much salt can raise your risk of high blood
pressure. Recent studies show that following the Dietary Approaches to Stop Hypertension (DASH)
eating plan can lower blood pressure.
Choose foods and beverages that are low in added sugar. If you drink alcoholic beverages, do so in
moderation.
Aim for a healthy weight by staying within your daily calorie needs. Balance the calories you take in with
the calories you use during physical activity. Be as physically active as you can.
Some people should get medical advice before starting or increasing physical activity. For example, talk
with your doctor if you have a chronic (ongoing) health problem, are on medicine, or have symptoms
such as chest pain, shortness of breath, or dizziness. Your doctor can suggest types and amounts of
physical activity that are safe for you
๏ท Quitting smoking
๏ท Losing excess weight
๏ท Avoiding the use of alcohol and illegal drugs
๏ท Getting enough sleep and rest
๏ท Reducing stress
๏ท Treating underlying conditions, such as diabetes and high blood pressure