Cardiomyopathy Classification and external resources
Opened left ventricle of heart shows a thickened, dilated left ventricle with subendocardial fibrosis manifested as increased whiteness of endocardium. Autopsy.
ICD-10	I42.0
ICD-9	425.4
DiseasesDB	2137
MeSH	D009202

Cardiomyopathy, which literally means "heart muscle disease", is the deterioration of the function of the myocardium (i. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings I00-I99 - Diseases of the Circulatory system (I00-I02 Acute rheumatic fever ( Rheumatic fever without mention of Heart The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books Myocardium is the muscular tissue of the Heart. Relationship to other layers The other tissues of the heart are the Endocardium e. , the actual heart muscle) for any reason. People with cardiomyopathy are often at risk of arrhythmia or sudden cardiac death or both. Dysrhythmia redirects here For the American band see Dysrhythmia (band. The term sudden cardiac death refers to Natural death from cardiac causes heralded by abrupt loss of Consciousness within one hour of the onset of acute symptoms ^[1]

Cardiomyopathies can generally be categorized into two groups, based on World Health Organization guidelines: extrinsic cardiomyopathies and intrinsic cardiomyopathies. ^[2]

Contents 1 Extrinsic cardiomyopathies 1.1 Ischemic cardiomyopathy 1.2 Cardiomyopathy due to systemic diseases 2 Intrinsic cardiomyopathies 3 Treatment 4 Genetic causes of cardiomyopathy 5 References 6 External links

Extrinsic cardiomyopathies

These are cardiomyopathies where the primary pathology is outside the myocardium itself. Pathology (from Greek grc πάθος pathos, "fate harm" and grc -λογία -logia) is the study and Most cardiomyopathies are extrinsic, because by far the most common cause of a cardiomyopathy is ischemia. In Medicine, ischemia ( Greek ισχαιμία, isch- is restriction hema or haema is Blood) is a restriction The World Health Organization calls these specific cardiomyopathies:^[2]

• Coronary artery disease
• Congenital heart disease
• Nutritional diseases
• Ischemic (or ischaemic) cardiomyopathy
• Hypertensive cardiomyopathy
• Valvular cardiomyopathy
• Inflammatory cardiomyopathy
• Cardiomyopathy secondary to a systemic metabolic disease
• Alcoholic cardiomyopathy

Ischemic cardiomyopathy

Ischemic cardiomyopathy is a weakness in the muscle of the heart due to inadequate oxygen delivery to the myocardium with coronary artery disease being the most common cause. Alcoholic cardiomyopathy is a disease in which the chronic long-term abuse of Alcohol leads to Heart failure. Coronary artery disease (CAD (or atherosclerotic Heart disease) is the end result of the accumulation of atheromatous plaques within the walls Anemia and sleep apnea are relatively common conditions that can contribute to ischemic myocardium and hyperthyroidism can cause a 'relative' ischemia secondary to high output heart failure. Individuals with ischemic cardiomyopathy typically have a history of myocardial infarction (heart attack), although longstanding ischemia can cause enough damage to the myocardium to precipitate a clinically significant cardiomyopathy even in the absence of myocardial infarction. Myocardial infarction ( MI or AMI for acute myocardial infarction) also known as a heart attack, occurs when the blood supply Myocardium is the muscular tissue of the Heart. Relationship to other layers The other tissues of the heart are the Endocardium In a typical presentation, the area of the heart affected by a myocardial infarction will initially become necrotic as it dies, and will then be replaced by scar tissue (fibrosis). Necrosis (in Greek Νεκρός = "dead" is the name given to unnatural Death of cells and living tissue. Fibrosis is the formation or development of excess fibrous Connective tissue in an organ or tissue as a reparative or reactive process as opposed to a formation of Fibrous This fibrotic tissue is akinetic; it is no longer muscle and cannot 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 into failure, and this failure is the functional result of an ischemic cardiomyopathy. Heart failure is a Cardiac condition that occurs when a problem with the structure or function of the Heart impairs its ability to supply

Cardiomyopathy due to systemic diseases

Many 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.

Intrinsic cardiomyopathies

An intrinsic cardiomyopathy is weakness in the muscle of the heart that is not due to an identifiable external cause. 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 specific etiology of weakened heart muscle. Etiology (alternatively aetiology, aitiology) is the study of causation. The intrinsic cardiomyopathies are a mixed-bag of disease states, each with their own causes.

Intrinsic cardiomyopathy has a number of causes including drug and alcohol toxicity, certain infections (including Hepatitis C), and various genetic and idiopathic (i. Medication, also referred to as medicine, can be loosely defined as any substance intended for use in the diagnosis cure mitigation treatment or prevention of disease An infection is the detrimental Colonization of a host Organism by a foreign Species. Hepatitis C is a blood-borne infectious disease that is caused by the Hepatitis C virus ( HCV) affecting the Liver. Genetics (from Ancient Greek grc-Latn genetikos, “genitive” and that from grc-Latn genesis, “origin” a discipline of Biology, is Idiopathic is an Adjective used primarily in Medicine meaning arising spontaneously or from an obscure or unknown cause. e. , unknown) causes.

Intrinsic cardiomyopathies are generally classified into four types,^[2][3] but additional types are also recognized:

• Dilated cardiomyopathy (DCM), the most common form, and one of the leading indications for heart transplantation. Dilated cardiomyopathy or DCM, also known as congestive cardiomyopathy, is a condition in which the Heart becomes weakened and enlarged and cannot pump Heart transplantation or cardiac transplantation, is a surgical transplant procedure performed on patients with end-stage Heart failure or severe Coronary In DCM the heart (especially the left ventricle) is enlarged and the pumping function is diminished. The left ventricle is one of four chambers (two atria and two ventricles in the Human Heart. Approximately 40% of cases are familial, but the genetics are poorly understood compared with HCM. Genetics (from Ancient Greek grc-Latn genetikos, “genitive” and that from grc-Latn genesis, “origin” a discipline of Biology, is In some cases it manifests as peripartum cardiomyopathy, and in other cases it may be associated with alcoholism. Peripartum cardiomyopathy (PPCM is a form of Dilated cardiomyopathy that is defined as deterioration in cardiac function presenting typically between the last month of
• Hypertrophic cardiomyopathy (HCM or HOCM), a genetic disorder caused by various mutations in genes encoding sarcomeric proteins. Hypertrophic cardiomyopathy, or HCM, is a disease of the Myocardium (the Muscle of the Heart) in which a portion of the myocardium is A genetic disorder is a condition caused by abnormalities in Genes or Chromosomes While some diseases such as Cancer, are due to genetic abnormalities acquired In biology mutations are changes to the Nucleotide sequence of the Genetic material of an organism "A-band" redirects here For other uses of the term see A band. 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 electrical disturbance of the heart in which heart muscle is replaced by fibrous scar tissue. Arrhythmogenic right ventricular dysplasia ( ARVD, also known as arrhythmogenic right ventricular cardiomyopathy or ARVC) is a type of nonischemic Cardiomyopathy The right ventricle is generally most affected. The right ventricle is one of four chambers (two atria and two ventricles in the Human Heart.
• Restrictive cardiomyopathy (RCM) is an uncommon cardiomyopathy. Restrictive cardiomyopathy (RCM is the least common Cardiomyopathy. The walls of the ventricles are stiff, but may not be thickened, and resist the normal filling of the heart with blood. A rare form of restrictive cardiomyopathy is the obliterative cardiomyopathy, seen in the hypereosinophilic syndrome. The hypereosinophilic syndrome (HS is a disease process characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³ in the blood for at least six months without 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 cardiomyopathy has been recognized as a separate type since the 1980s. Non-compaction cardiomyopathy (NCCsometimes known as "spongiform cardiomyopathy" is a rare congenital Cardiomyopathy that affects both children and adults The term 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.

Signs and symptoms Cardiomyopathy is usually found incidentally - "case finding" - by healthcare professionals during a routine checkup. The only test for hypertension is a blood pressure measurement. Hypertension in isolation usually produces no symptoms although some people report headaches, fatigue, wanting to sleep more than usual, dizziness, blurred vision, facial flushing or tinnitus. [10]

Malignant Cardiomyopathy (or accelerated Cardiomyopathy) is distinct as a late phase in the condition, and may present with headaches, blurred vision and end-organ damage.

Cardiomyopathy is often confused with mental tension, stress and anxiety. While chronic anxiety and/or irritability is associated with poor outcomes in people with hypertension, it alone does not cause it. Accelerated hypertension is associated with somnolence, confusion, visual disturbances, and nausea and vomiting (hypertensive encephalopathy)

Treatment

Treatment depends on the type of cardiomyopathy, but may include medication, implanted pacemakers, defibrillators, or ventricular assist devices (LVADs), or ablation. For other uses see Pacemaker (disambiguation A pacemaker (or artificial pacemaker, so as not to be confused with the heart's natural pacemaker An implantable cardioverter-defibrillator ( ICD) is a small battery -powered electrical impulse generator which is implanted in patients who are at risk of Sudden A Ventricular assist device, or VAD, is a mechanical device that is used to partially or completely replace the function of a failing Heart. Ablation is defined as the removal of material from the surface of an object by Vaporization, Chipping, or other erosive processes The goal of treatment is often symptom relief, and some patients may eventually require a heart transplant. Heart transplantation or cardiac transplantation, is a surgical transplant procedure performed on patients with end-stage Heart failure or severe Coronary Treatment of cardiomyopathy (and other heart diseases) using alternative methods such as stem cell therapy is commercially available but is not supported by convincing evidence. Many Medical researchers believe that stem cell treatments have the potential to change the face of human disease and alleviate suffering

Genetic causes of cardiomyopathy

Phenotype	Inheritance pattern	Chromosomal locus	Gene	Protein	Skeletal myopathy
Dilated cardiomyopathy	X-linked	Xp21	dystrophin	Dystrophin	Duchenne / Becker muscular dystrophy
	X-linked	Xq28	G4. Dilated cardiomyopathy or DCM, also known as congestive cardiomyopathy, is a condition in which the Heart becomes weakened and enlarged and cannot pump Sex linkage is the phenotypic expression of an Allele that is related to the chromosomal sex of the individual Dystrophin is a rod-shaped Cytoplasmic Protein, and a vital part of a Protein complex that connects the Cytoskeleton of a Muscle fiber Dystrophin is a rod-shaped Cytoplasmic Protein, and a vital part of a Protein complex that connects the Cytoskeleton of a Muscle fiber Duchenne muscular dystrophy ( DMD) is a severe recessive x-linked form of Muscular dystrophy that is characterized by rapid progression of muscle degeneration eventually Becker's muscular dystrophy (also known as Benign pseudohypertrophic Muscular dystrophy) is an X-linked recessive inherited disorder characterized Sex linkage is the phenotypic expression of an Allele that is related to the chromosomal sex of the individual 5	Tafazzin	Barth syndrome
	Autosomal dominant	15q14	actin	Actin	Nemaline myopathy
		2q35	desmin	Desmin	Desmin myopathy
		5q33	δ-sarcoglycan	δ-sarcoglycan	Limb girdle muscular dystrophy 2F
		1q32	Troponin T	Troponin T
		14q11	β-myosin heavy chain	β-myosin heavy chain
		15q2	α-tropomyosin	α-tropomyosin	Nemaline myopathy
		Midna	Mitochondrial respiratory chain	Mitochondrial respiratory chain	Mitochondrial myopathy
Dilated cardiomyopathy with conduction disease	Autosomal dominant	1q21	lamin A/C	Lamin A/C	Emery-Dreifuss muscular dystrophy
Hypertrophic cardiomyopathy	Autosomal dominant	14q11	β-myosin heavy chain	β-myosin heavy chain
		14q11	β-myosin heavy chain	β-myosin heavey chain
		1q32	Troponin T	Troponin T
		12q23	Troponin T	Troponin T
		15q2	α-tropomyosin	α-tropomyosin	Nemaline myopathy
		11q11	myosin-binding protein C	myosin-binding protein C
		3p21	myosin essential light chain	myosin essential light chain
		3p21	myosin regulatory light chain	myosin regulatory light chain
		2p31	titin	Titin
Hypertrophic cardiomyopathy with Wolf-Parkinson-White syndrome		7q3	AMPK	AMPK
		MIDINA	Mitochondrial respiratory chain	Mitochondrial respiratory chain	Mitochondrial myopathy
Left ventricular noncompaction	X-linked	Xq28	G4. Tafazzin is a Protein highly expressed in cardiac and Skeletal muscle. Barth syndrome ( BTHS) also known as 3-Methylglutaconic aciduria type II and Cardiomyopathy-neutropenia syndrome is a rare Genetic disorder classified Actin is a globular roughly 42-kDa Protein found in all eukaryotic cells (except for Nematode sperm where it may be present at concentrations of Actin is a globular roughly 42-kDa Protein found in all eukaryotic cells (except for Nematode sperm where it may be present at concentrations of Nemaline myopathy (also called rod myopathy or nemaline rod myopathy) is a Congenital, Hereditary neuromuscular disorder that causes muscle Desmin is a type III Intermediate filament found near the Z line in Sarcomeres It was first purified in 1977 the Gene was characterized in 1989 and the The sarcoglcyans are a family of five transmembrane proteins (α β γ δ or ε involved in the Protein complex responsible for connecting the muscle fibre Cytoskeleton Limb-girdle muscular dystrophy or Erb's muscular dystrophy is an Autosomal class of Muscular dystrophy that is similar but distinct from Duchenne muscular Troponin T is a part of the Troponin complex It binds to Tropomyosin, interlocking them to form a troponin-tropomyosin complex Troponin T is a part of the Troponin complex It binds to Tropomyosin, interlocking them to form a troponin-tropomyosin complex Myosins are a large family of Motor proteins found in Eukaryotic tissues. Tropomyosin is an actin-binding protein that regulates actin mechanics Nemaline myopathy (also called rod myopathy or nemaline rod myopathy) is a Congenital, Hereditary neuromuscular disorder that causes muscle Dilated cardiomyopathy or DCM, also known as congestive cardiomyopathy, is a condition in which the Heart becomes weakened and enlarged and cannot pump Emery-Dreifuss muscular dystrophy is a condition that chiefly affects Muscles used for movement (skeletal muscles and heart ( Cardiac) muscle Hypertrophic cardiomyopathy, or HCM, is a disease of the Myocardium (the Muscle of the Heart) in which a portion of the myocardium is Hypertrophic cardiomyopathy, or HCM, is a disease of the Myocardium (the Muscle of the Heart) in which a portion of the myocardium is Non-compaction cardiomyopathy (NCCsometimes known as "spongiform cardiomyopathy" is a rare congenital Cardiomyopathy that affects both children and adults Sex linkage is the phenotypic expression of an Allele that is related to the chromosomal sex of the individual 5	Tafazzin	Barth syndrome
	Autosomal dominant	18q12	α-dystrobrevin	α-Dystrobrevin	Muscular dystrophy

Table from article *The Failing Heart. Tafazzin is a Protein highly expressed in cardiac and Skeletal muscle. Barth syndrome ( BTHS) also known as 3-Methylglutaconic aciduria type II and Cardiomyopathy-neutropenia syndrome is a rare Genetic disorder classified Nature. Retrieved on June 15, 2007. Events 763 BC - Assyrians record a Solar eclipse that will be used to fix the Chronology of Mesopotamian history Year 2007 ( MMVII) was a Common year starting on Monday of the Gregorian calendar in the 21st century.

References

External links

• The Cardiomyopathy Association A site is designed to provide you with information on the main forms of the heart muscle disease known as cardiomyopathy. Great information for adults and young.
• Cardiomyopathy information from Seattle Children's Hospital Heart Center
• Information from the Stanford Hypertrophic Cardiomyopathy Center
• Cardiomyopathy-related antibodies
• Treatment Cardiomyopathy (Alternative Medicine)

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