cardiomyopathy wiki
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CardiomyopathyCardiomyopathy, which literally means "heart muscle disease," is the deterioration of the function of
themyocardium(i.e., the actual heart muscle) for any reason. People with cardiomyopathy are often at
risk ofarrhythmiaorsudden cardiac deathor both.[1]
Cardiomyopathies can be categorized as extrinsic or intrinsic
Extrinsic cardiomyopathiesThese are cardiomyopathies where the primarypathologyis outside the myocardium itself. Most
cardiomyopathies are extrinsic, because by far the most common cause of a cardiomyopathy isischemia.The World Health Organization calls thesespecific cardiomyopathies:
[2]
Congenital heart disease Nutritional diseases Ischemic (or non-ischaemic) cardiomyopathy Hypertensive cardiomyopathy Valvular cardiomyopathy Inflammatory cardiomyopathy (includingChagas disease,which is an important cause of
cardiomyopathy in Central and South America.[3][4]
)
Cardiomyopathy secondary to a systemic metabolic disease Alcoholic cardiomyopathy Diabetic cardiomyopathy Restrictive cardiomyopathy
[editIschemic cardiomyopathyCommonly used term "ischemic cardiomyopathy," referring to myocardial ischemia andinfarction,is
not supported by current cardiomyopathies classification schemes[5][6]
.
Ischemic cardiomyopathy is a weakness in the muscle of the heart due to inadequate oxygen delivery to
the myocardium withcoronary artery diseasebeing the most common cause.Anemiaandsleep apnea
are relatively common conditions that can contribute to ischemic myocardium andhyperthyroidismcan
cause a 'relative' ischemia secondary to high output heart failure. Individuals with ischemic
cardiomyopathy typically have a history ofmyocardial infarction(heart attack), although longstanding
ischemia can cause enough damage to themyocardiumto precipitate a clinically significant
cardiomyopathy even in the absence of myocardial infarction. In a typical presentation, the area of theheart affected by a myocardial infarction will initially becomenecroticas it dies, and will then be
replaced bymyocardial scarring(fibrosis). This fibrotic tissue is akinetic; it is no longer muscle andcannot contribute to the heart's function as a pump. If the akinetic region of the heart is substantial
enough, the affected side of the heart (i.e. the left or right side) will go intofailure,and this failure is
the functional result of an ischemic cardiomyopathy.
In some individuals, severe emotional stress may lead to "takotsubo cardiomyopathy", a specific
cardiomyopathy which has a particularaetiology.
[edit]Cardiomyopathy due to systemic diseasesMany diseases can result in cardiomyopathy. These include diseases like hemochromatosis, (an
abnormal accumulation of iron in the liver and other organs), amyloidosis (an abnormal accumulation
of the amyloid protein), diabetes, hyperthyroidism, lysosomal storage diseases and the muscular
dystrophies.
Some current chemotheraphy drugs can also be the cause. However, this is only likely in 2-3% ofpatients.
[edit] Intrinsic cardiomyopathiesSee also:Myocarditis
An intrinsic cardiomyopathy is defined as weakness in the muscle of the heart that is not due to an
identifiable external cause. This definition was used to categorize previously idiopathic
cardiomyopathies although specific external causes have since been identified for many. For example,alcoholism has been identified as a cause for some forms of dilated cardiomyopathy.
To make a diagnosis of an intrinsic cardiomyopathy, significant coronary artery disease should be ruled
out (amongst other things). The term intrinsic cardiomyopathy does not describe the specificetiology
of weakened heart muscle. The intrinsic cardiomyopathies consist of a variety of disease states, each
with their own causes.
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Many intrinsic cardiomyopathies now have identifiable external causes includingdrugandalcohol
toxicity, certaininfections(includingHepatitis C), and variousgeneticandidiopathic(i.e., unknown)
causes.
Intrinsic cardiomyopathies are generally classified into four types,[2][7]
but additional types are also
recognized:
Dilated cardiomyopathy(DCM), the most common form, and one of the leading indicationsforheart transplantation.In DCM the heart (especially theleft ventricle)is enlarged and the
pumping function is diminished. Approximately 40% of cases are familial, but thegeneticsare
poorly understood compared with HCM. In some cases it manifests asperipartum
cardiomyopathy,and in other cases it may be associated with alcoholism.
Hypertrophic cardiomyopathy(HCM or HOCM), agenetic disordercaused by variousmutationsin genes encodingsarcomericproteins. Approximately 45% of mutations occur inB-myosin heavy chain gene while 35% occur in cardiac myosin binding protein C gene. In
HCM the heart muscle is thickened, which can obstruct blood flow and prevent the heart from
functioning properly.
Arrhythmogenic right ventricular cardiomyopathy(ARVC) arises from an electricaldisturbance of the heart in which heart muscle is replaced by fibrous scar tissue. Theright
ventricleis generally most affected.
Restrictive cardiomyopathy(RCM) is an uncommon cardiomyopathy. The walls of theventricles are stiff, but may not be thickened, and resist the normal filling of the heart with
blood. A rare form of restrictive cardiomyopathy is theobliterative cardiomyopathy,seen inthehypereosinophilic syndrome.In this type of cardiomyopathy, the myocardium in the
apices of the left and right ventricles becomes thickened and fibrotic, causing a decrease in the
volumes of the ventricles and a type of restrictive cardiomyopathy.
Noncompaction cardiomyopathyhas been recognized as a separate type since the 1980s. Theterm refers to a cardiomyopathy where the left ventricle wall has failed to grow properly from
birth and has a spongy appearance when viewed during an echocardiogram.
[edit] Signs and symptomsSymptoms and signs may mimic those of almost any form of heart disease. Chest pain is common.
Mild myocarditis or cardiomyopathy is frequently asymptomatic; severe cases are associated with heart
failure, arrhythmias, and systemic embolization. Manifestations of the underlying disease (e.g., Chagas'
disease) may be prominent. Most patients with biopsy-proven myocarditis report a recent viralprodrome preceding cardiovascular symptoms.
ECG abnormalities are often present, although the changes are frequently nonspecific. A pattern
characteristic of left ventricular hypertrophy may be present. Flat or invertedT wavesare most
common, often with low-voltage QRS complexes. Intraventricular conduction defects and bundle
branch block, especially left bundle branch block, are also common. An echocardiogram is useful to
detect wall motion abnormalities or a pericardial effusion. Chest radiographs can be normal or can
show evidence of congestive heart failure with pulmonary edema or cardiomegaly.
[edit] TreatmentTreatment depends on the type of cardiomyopathy, but may include medication, implantedpacemakers,
defibrillators,orventricular assist devices(LVADs), orablation.The goal of treatment is often
symptom relief, and some patients may eventually require aheart transplant.Treatment of
cardiomyopathy (and other heart diseases) using alternative methods such asstem cell therapyiscommercially available but is not supported by convincing evidence.
ESC algorithmTheESCalgorithm weights the following parameters in establishing the diagnosis of heart failure:
[35]
Influence
Parameter
Supports if presentOpposes if
normal or absent
+ - to some degree
++ - to intermediate degree
+++ - to high degree
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Compatible symptoms ++ ++
Compatible signs ++ +
Cardiac dysfunction
on echocardiography+++ +++
Response of symptoms
or signs to therapy+++ ++
ECG
Normal ++
Abnormal ++ +
Dysrhythmia +++ +
Laboratory
BNP > 400 pg/mL and/or
NT-proBNP > 2000 pg/mL+++ +
BNP < 100 pg/mL and
NT-proBNP < 400 pg/mL+ +++
Hyponatraemia + +
Renal dysfunction + +
Mild elevations of troponin + +
Chest X-ray
Pulmonary congestion +++ +
Reduced exercise capacity +++ ++
Abnormal pulmonary function tests+ +
Abnormal haemodynamics at rest +++ ++