Template:Short description Template:Cs1 config Template:Use dmy dates Template:Infobox medical condition (new) Cardiomyopathy is a group of primary diseases of the heart muscle.<ref name=NIH2016Sym/> Early on there may be few or no symptoms.<ref name=NIH2016Sym/> As the disease worsens, shortness of breath, feeling tired, and swelling of the legs may occur, due to the onset of heart failure.<ref name=NIH2016Sym/> An irregular heart beat and fainting may occur.<ref name="NIH2016Sym">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> Those affected are at an increased risk of sudden cardiac death.<ref name="NIH2016Risk">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref>
As of 2013, cardiomyopathies are defined as "disorders characterized by morphologically and functionally abnormal myocardium in the absence of any other disease that is sufficient, by itself, to cause the observed phenotype."<ref>Template:Cite book</ref><ref>Template:Cite journal</ref> Types of cardiomyopathy include hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular dysplasia, and Takotsubo cardiomyopathy (broken heart syndrome).<ref name=NIH2016Type/> In hypertrophic cardiomyopathy the heart muscle enlarges and thickens.<ref name=NIH2016Type/> In dilated cardiomyopathy the ventricles enlarge and weaken.<ref name=NIH2016Type/> In restrictive cardiomyopathy the ventricle stiffens.<ref name="NIH2016Type">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref>
In many cases, the cause cannot be determined.<ref name=NIH2016Cau/> Hypertrophic cardiomyopathy is usually inherited, whereas dilated cardiomyopathy is inherited in about one third of cases.<ref name=NIH2016Cau/> Dilated cardiomyopathy may also result from alcohol, heavy metals, coronary artery disease, cocaine use, and viral infections.<ref name=NIH2016Cau/> Restrictive cardiomyopathy may be caused by amyloidosis, hemochromatosis, and some cancer treatments.<ref name="NIH2016Cau">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> Broken heart syndrome is caused by extreme emotional or physical stress.<ref name=NIH2016Type/>
Treatment depends on the type of cardiomyopathy and the severity of symptoms.<ref name=NIH2016Tx/> Treatments may include lifestyle changes, medications, or surgery.<ref name=NIH2016Tx/> Surgery may include a ventricular assist device or heart transplant.<ref name="NIH2016Tx">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref> In 2015 cardiomyopathy and myocarditis affected 2.5 million people.<ref name="GBD2015Pre">Template:Cite journal</ref> Hypertrophic cardiomyopathy affects about 1 in 500 people while dilated cardiomyopathy affects 1 in 2,500.<ref name=NIH2016Type/><ref>Template:Cite book</ref> They resulted in 354,000 deaths up from 294,000 in 1990.<ref name="GBD2015De">Template:Cite journal</ref><ref name="GDB2013">Template:Cite journal</ref> Arrhythmogenic right ventricular dysplasia is more common in young people.<ref name=NIH2016Risk/>
Signs and symptomsEdit
The presentation of cardiomyopathy is:Template:Citation needed
- Shortness of breath or trouble breathing, especially with physical exertion
- Fatigue
- Swelling in the ankles, feet, legs, abdomen and veins in the neck
- Dizziness
- Lightheadedness
- Fainting during physical activity
- Arrhythmias (abnormal heartbeats)
- Chest pain, especially after physical exertion or heavy meals
- Heart murmurs (unusual sounds associated with heartbeats)
CausesEdit
Cardiomyopathies can be of genetic (familial) or non-genetic (acquired) origin.<ref>Template:Cite journal</ref> Genetic cardiomyopathies usually are caused by sarcomere or cytoskeletal diseases, neuromuscular disorders, inborn errors of metabolism, malformation syndromes and sometimes are unidentified.<ref>Template:Cite journal</ref><ref>Template:Cite journal</ref> Non-genetic cardiomyopathies can have definitive causes such as viral infections, myocarditis and others.<ref>Template:Cite journal</ref><ref>Template:Cite journal</ref>
Cardiomyopathies are either confined to the heart or are part of a generalized systemic disorder, both often leading to cardiovascular death or progressive heart failure-related disability. Other diseases that cause heart muscle dysfunction are excluded, such as coronary artery disease, hypertension, or abnormalities of the heart valves.<ref name="Harrison">Template:Cite book</ref> Often, the underlying cause remains unknown, but in many cases the cause may be identifiable.<ref>Template:Cite book</ref> Alcoholism, for example, has been identified as a cause of dilated cardiomyopathy, as has drug toxicity, and certain infections (including Hepatitis C).<ref name="AdamNicholson2008">Template:Cite journal</ref><ref name="WestphalRigopoulos2017">Template:Cite journal</ref><ref name="DomontCacoub2016">Template:Cite journal</ref> Untreated celiac disease can cause cardiomyopathies, which can completely reverse with a timely diagnosis.<ref name="CiaccioLewis2017">Template:Cite journal</ref> In addition to acquired causes, molecular biology and genetics have given rise to the recognition of various genetic causes.<ref name="WestphalRigopoulos2017" /><ref name="SimpsonRutland2017">Template:Cite journal</ref>
A more clinical categorization of cardiomyopathy as 'hypertrophied', 'dilated', or 'restrictive',<ref name="FusterHurst2004">Template:Cite book</ref> has become difficult to maintain because some of the conditions could fulfill more than one of those three categories at any particular stage of their development.<ref name="pmid35772124">Template:Cite journal</ref>
The current American Heart Association (AHA) definition divides cardiomyopathies into primary, which affect the heart alone, and secondary, which are the result of illness affecting other parts of the body. These categories are further broken down into subgroups which incorporate new genetic and molecular biology knowledge.<ref>Template:Cite journal</ref>
MechanismEdit
The pathophysiology of cardiomyopathies is better understood at the cellular level with advances in molecular techniques. Mutant proteins can disturb cardiac function in the contractile apparatus (or mechanosensitive complexes). Cardiomyocyte alterations and their persistent responses at the cellular level cause changes that are correlated with sudden cardiac death and other cardiac problems.<ref>Template:Cite journal</ref>
Cardiomyopathies are generally varied individually. Different factors can cause cardiomyopathies in adults as well as children. For example, dilated cardiomyopathy in adults is associated with ischemic cardiomyopathy, hypertension, valvular diseases, and genetics. In children, neuromuscular diseases such as Becker muscular dystrophy or X-linked genetic disorder, are directly linked with cardiomyopathies.<ref>Template:Cite journal</ref>
DiagnosisEdit
Among the diagnostic procedures done to determine a cardiomyopathy are:<ref name="ref2">{{#invoke:citation/CS1|citation |CitationClass=web }}</ref>
- Physical exam
- Family history
- Blood test
- ECG
- Echocardiogram
- Stress test
- Genetic testing
ClassificationEdit
Cardiomyopathies can be classified using different criteria:<ref>Template:Cite book</ref>
- Primary/intrinsic cardiomyopathies<ref name="AMA">Template:Cite journal</ref>
- Congenital
- Mixed
- Acquired
- Stress cardiomyopathy
- Myocarditis, inflammation of and injury to heart tissue due in part to its infiltration by lymphocytes and monocytes<ref name="pmid25858537">Template:Cite journal</ref><ref name="pmid27166925">Template:Cite journal</ref>
- Eosinophilic myocarditis, inflammation of and injury to heart tissue due in part to its infiltration by eosinophils<ref name="pmid25858537" />
- Ischemic cardiomyopathy (not formally included in the classification, due to ischemic cardiomyopathy being a direct result of another cardiac problem)<ref name="AMA" />
- Secondary/extrinsic cardiomyopathies<ref name="AMA" />
- Metabolic/storage
- Endomyocardial
- Endocrine
- Cardiofacial
- Neuromuscular
- Other
- Obesity-associated cardiomyopathy<ref>Template:Cite book</ref>
TreatmentEdit
Treatment may include suggestion of lifestyle changes to better manage the condition. Treatment depends on the type of cardiomyopathy and condition of disease, but may include medication (conservative treatment) or iatrogenic/implanted pacemakers for slow heart rates, defibrillators for those prone to fatal heart rhythms, ventricular assist devices (VADs) for severe heart failure, or catheter ablation for recurring dysrhythmias that cannot be eliminated by medication or mechanical cardioversion. The goal of treatment is often symptom relief, and some patients may eventually require a heart transplant.<ref name=ref2/>
Acoramidis (Attruby) was approved for medical use in the United States in November 2024, to treat adults with cardiomyopathy of wild-type or variant (hereditary) transthyretin-mediated amyloidosis (ATTR-CM) to reduce death and hospitalization related to heart problems.<ref name="FDA 20241125">{{#invoke:citation/CS1|citation |CitationClass=web }} Template:PD-notice</ref>
See alsoEdit
- Basic Research in Cardiology (journal)
- Fibrosing cardiomyopathy (disease in great apes)
- Myopathy
ReferencesEdit
Further readingEdit
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External linksEdit
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