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Encyclopedia > Cardiomyopathy
Cardiomyopathy
Classification & 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.e., the actual heart muscle) for any reason. People with cardiomyopathy are often at risk of arrhythmia or sudden cardiac death or both.[1] Image File history File links Idiopathic_cardiomyopathy,_gross_pathology_20G0018_lores. ... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10) is a coding of diseases and signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or diseases, as classified by the World Health Organization (WHO). ... // I00-I99 - Diseases of the circulatory system (I00-I02) Acute rheumatic fever (I00) Rheumatic fever without mention of heart involvement (I01) Rheumatic fever with heart involvement (I02) Rheumatic chorea (I05-I09) Chronic rheumatic heart diseases (I05) Rheumatic mitral valve diseases (I050) Mitral stenosis (I051) Rheumatic mitral insufficiency (I06) Rheumatic aortic... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The Disease Bold textDatabase 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 in the life sciences. ... Myocardium is the muscular tissue of the heart. ... A cardiac arrhythmia, also called cardiac dysrhythmia, is a disturbance in the regular rhythm of the heartbeat. ... A cardiac arrest is the cessation of normal circulation of the blood due to failure of the ventricles of the heart to contract effectively during systole. ...


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

Contents

Extrinsic cardiomyopathies

These are cardiomyopathies where the primary pathology is outside the myocardium itself. Most cardiomyopathies are extrinsic, because by far the most common cause of a cardiomyopathy is ischemia. The World Health Organization calls these specific cardiomyopathies:[2] A renal cell carcinoma (chromophobe type) viewed on a hematoxylin & eosin stained slide Pathologist redirects here. ... In medicine, ischemia (Greek ισχαιμία, isch- is restriction, hema or haema is blood) is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. ...

  • 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

Alcoholic cardiomyopathy is a disease in which alcohol use damages the heart muscle causing heart failure. ...

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. 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. 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). 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. Coronary heart disease (CHD), also called coronary artery disease (CAD) and atherosclerotic heart disease, is the end result of the accumulation of atheromatous plaques within the walls of the arteries that supply the myocardium (the muscle of the heart). ... Acute myocardial infarction (AMI or MI), more commonly known as a heart attack, is a disease state that occurs when the blood supply to a part of the heart is interrupted. ... Myocardium is the muscular tissue of the heart. ... Necrosis (in Greek Νεκρός = Dead) is the name given to unprogrammed death of cells/living tissue (compare with apoptosis - programmed cell death). ... 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 tissue as a normal constituent of an organ or tissue. ... Congestive heart failure (CHF), also called congestive cardiac failure (CCF) or just heart failure, is a condition that can result from any structural or functional cardiac disorder that impairs the ability of the heart to fill with or pump a sufficient amount of blood through the body. ...


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. The intrinsic cardiomyopathies are a mixed-bag of disease states, each with their own causes. This article is about the medical term. ...


Intrinsic cardiomyopathy has a number of causes including drug and alcohol toxicity, certain infections (including Hepatitis C), and various genetic and idiopathic (i.e., unknown) causes. This article does not cite any references or sources. ... Grain alcohol redirects here. ... An infection is the detrimental colonization of a host organism by a foreign species. ... This page is for the disease. ... This article is about the general scientific term. ... Idiopathic means arising spontaneously or from an obscure or unknown cause. ...


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. In DCM the heart (especially the left ventricle) is enlarged and the pumping function is diminished. Approximately 40% of cases are familial, but the genetics are poorly understood compared with HCM. In some cases it manifests as peripartum cardiomyopathy, and in other cases it may be associated with alcoholism.
  • Hypertrophic cardiomyopathy (HCM or HOCM), a genetic disorder caused by various mutations in genes encoding sarcomeric proteins. 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. The right ventricle is generally most affected.
  • Restrictive cardiomyopathy (RCM) is an uncommon 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. 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. 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.

Dilated cardiomyopathy or DCM (also known as congestive cardiomyopathy), is a disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is dilated, often without any obvious cause. ... In the heart, a ventricle is a chamber which collects blood from an atrium (another heart chamber) and pumps it out of the heart. ... This article is about the general scientific term. ... Peripartum cardiomyopathy (PPCM) is a form of dilated cardiomyopathy that is defined as a deterioration in cardiac function presenting between the last month of gestation and up to five months post-partum. ... Hypertrophic cardiomyopathy, or HCM, is a disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is hypertrophied (thickened) without any obvious cause. ... A genetic disorder is a disease caused by abnormalities in genes or chromosomes. ... It has been suggested that mutant be merged into this article or section. ... Image of sarcomere A sarcomere is the basic unit of a cross striated muscles myofibril. ... Arrhythmogenic right ventricular dysplasia (ARVD, also known as arrhythmogenic right ventricular cardiomyopathy or ARVC) is a type of nonischemic cardiomyopathy that involves primarily the right ventricle. ... The right ventricle is one of four chambers (two atria and two ventricles) in the human heart. ... Restrictive cardiomyopathy (RCM) is the least common cardiomyopathy. ... The hypereosinophilic syndrome is a disease process characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm³) in the blood for at least six months without any recognizable cause after a careful workup, with evidence of involvement of either the heart, nervous system, or bone marrow. ... Non-compaction cardiomyopathy (NCC) is a rare congenital cardiomyopathy that affects both children and adults. ...

Treatment

Treatment depends on the type of cardiomyopathy, but may include medication, implanted pacemakers, defribillators, or ventricular assist devices (LVADs), or ablation. The goal of treatment is often symptom relief, and some patients may eventually require a heart transplant. 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. A pacemaker, scale in centimeters A pacemaker (or artificial pacemaker, so as not to be confused with the hearts natural pacemaker) is a medical device which uses electrical impulses, delivered by electrodes contacting the heart muscles, to regulate the beating of the heart. ... ICD An implantable cardioverter-defibrillator (ICD), also known as an automated implantable cardioverter-defibrillator (AICD), is a small battery powered electrical impulse generator which is implanted in patients who are at risk of sudden cardiac death due to ventricular fibrillation. ... A Ventricular assist device, or VAD, is 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. ... Mouse embryonic stem cells. ...


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 G4.5 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 G4.5 Tafazzin Barth syndrome
Autosomal dominant 18q12 α-dystrobrevin α-Dystrobrevin Muscular dystrophy

Table from article *The Failing Heart. Nature. Retrieved on June 15, 2007. Dilated cardiomyopathy or DCM (also known as congestive cardiomyopathy), is a disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is dilated, often without any obvious cause. ... It has been suggested that sex chromosome be merged into this article or section. ... Dystrophin is a protein found in membranes surrounding individual muscle fibers, and its deficiency is one of the root causes of muscular dystrophy. ... Dystrophin is a protein found in membranes surrounding individual muscle fibers, and its deficiency is one of the root causes of muscular dystrophy. ... Duchenne muscular dystrophy (DMD) (also known as muscular dystrophy - Duchenne type) is an eventually fatal disorder that is characterized by rapidly progressive muscle weakness and atrophy of muscle tissue starting in the legs and pelvis and later affecting the whole body. ... Beckers muscular dystrophy (also known as Benign pseudohypertrophic muscular dystrophy) is an X-linked recessive inherited disorder characterized by slowly progressive muscle weakness of the legs and pelvis. ... It has been suggested that sex chromosome be merged into this article or section. ... Tafazzin is a protein highly expressed in cardiac and skeletal muscle. ... Barth syndrome is a rare genetic disorder classified by many signs and symptoms, including metabolism distortion, delayed motor skills, stamina deficiency, hypotonia, chronic fatigue, delayed growth, cardiomyopathy, and compromised immune system. ... It has been suggested that dominant allele be merged into this article or section. ... G-Actin (PDB code: 1j6z). ... G-Actin (PDB code: 1j6z). ... Nemaline myopathy (NM; also called rod myopathy or nemaline rod myopathy) is a group of congenital, hereditary neuromuscular disorders that cause muscle weakness, generally nonprogressive, of varying severity. ... Intermediate filaments are one component of the cytoskeleton - important structural components of living cells. ... 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 to the extracellular matrix, preventing damage the muscle fibre sarcolemma through shearing forces. ... Limb-girdle muscular distrophy or Erbs muscular dystrophy is a type of muscular dystrophy that includes Duchenne muscular dystrophy, Beckers muscular dystrophy, and a large number of rarer disorders. ... Troponin Troponin T is a part of the troponin complex. ... Troponin Troponin T is a part of the troponin complex. ... Myosin is a motor protein filament found in muscle tissue. ... Troponin Tropomyosin, along with the troponin, regulate the shortening of the muscle protein filaments actin and myosin. ... Nemaline myopathy (NM; also called rod myopathy or nemaline rod myopathy) is a group of congenital, hereditary neuromuscular disorders that cause muscle weakness, generally nonprogressive, of varying severity. ... Dilated cardiomyopathy or DCM (also known as congestive cardiomyopathy), is a disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is dilated, often without any obvious cause. ... It has been suggested that dominant allele be merged into this article or section. ... 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 hypertrophied (thickened) without any obvious cause. ... It has been suggested that dominant allele be merged into this article or section. ... Hypertrophic cardiomyopathy, or HCM, is a disease of the myocardium (the muscle of the heart) in which a portion of the myocardium is hypertrophied (thickened) without any obvious cause. ... Non-compaction cardiomyopathy (NCC) is a rare congenital cardiomyopathy that affects both children and adults. ... It has been suggested that sex chromosome be merged into this article or section. ... Tafazzin is a protein highly expressed in cardiac and skeletal muscle. ... Barth syndrome is a rare genetic disorder classified by many signs and symptoms, including metabolism distortion, delayed motor skills, stamina deficiency, hypotonia, chronic fatigue, delayed growth, cardiomyopathy, and compromised immune system. ... It has been suggested that dominant allele be merged into this article or section. ... is the 166th day of the year (167th in leap years) in the Gregorian calendar. ... Year 2007 (MMVII) is the current year, a common year starting on Monday of the Gregorian calendar and the AD/CE era in the 21st century. ...


References

  1. ^ Kasper, Denis L. et al (2005). Harrison's Principles of Internal Medicine, 16th edn. McGraw-Hill. ISBN 0-07-139140-1. 
  2. ^ a b c Richardson, P. et al (1996). "Report of the 1995 World Health Organization/International Society and Federation of Cardiology Task Force on the Definition and Classification of cardiomyopathies". Circulation 93 (5): 841-2. PMID 8598070.  (Full text)
  3. ^ Cardiomyopathy Association. About cardiomyopathy. Retrieved on 2006-09-28.

Year 2006 (MMVI) was a common year starting on Sunday of the Gregorian calendar. ... is the 271st day of the year (272nd in leap years) in the Gregorian calendar. ...

External links


  Results from FactBites:
 
MedlinePlus Medical Encyclopedia: Cardiomyopathy (1060 words)
Cardiomyopathy can be caused by viral infections, heart attacks, alcoholism, long-term, severe high blood pressure, nutritional deficiences (particularly selenium, thiamine, and L-carnitine), systemic lupus erythematosus, celiac disease, and end-stage kidney disease.
Dilated cardiomyopathy: This is a global, usually idiopathic myocardial disorder characterized by a marked enlargement and inadequate function of the left ventricle.
Ischemic cardiomyopathy: This is caused by heart attacks, which leave scars in the heart muscle (myocardium).
cardiomyopathy: Definition and Much More from Answers.com (2211 words)
Among the causes of cardiomyopathy in cancer patients is radiation therapy to the chest, which is oftenemployed for breast cancer, cancer of the esophagus or lymphoma.
Cardiomyopathy of any cause typically leads to signs and symptoms of heart failure as the pumping efficiency of the heart is diminished.
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.
  More results at FactBites »


 

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