Cardiomyopathy

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Cardiomyopathy
Arrhythmogenic right ventricular dysplasia
  • Takotsubo cardiomyopathy[3]
  • Causes
    TreatmentDepends on type and symptoms[5]
    Frequency2.5 million with myocarditis (2015)[6]
    Deaths354,000 with myocarditis (2015)[7]

    Cardiomyopathy is a group of primary diseases of the

    sudden cardiac death.[2]

    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."

    arrhythmogenic right ventricular dysplasia, and Takotsubo cardiomyopathy (broken heart syndrome).[3] In hypertrophic cardiomyopathy the heart muscle enlarges and thickens.[3] In dilated cardiomyopathy the ventricles enlarge and weaken.[3] In restrictive cardiomyopathy the ventricle stiffens.[3]

    In many cases, the cause cannot be determined.

    hemochromatosis, and some cancer treatments.[4] Broken heart syndrome is caused by extreme emotional or physical stress.[3]

    Treatment depends on the type of cardiomyopathy and the severity of symptoms.

    heart transplant.[5] In 2015 cardiomyopathy and myocarditis affected 2.5 million people.[6] Hypertrophic cardiomyopathy affects about 1 in 500 people while dilated cardiomyopathy affects 1 in 2,500.[3][10] They resulted in 354,000 deaths up from 294,000 in 1990.[7][11] Arrhythmogenic right ventricular dysplasia is more common in young people.[2]

    Signs and symptoms

    The arrhythmia, ventricular fibrillation, seen on an ECG

    Signs and symptoms of cardiomyopathy include:

    • 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)

    Causes

    Cardiomyopathies can be of genetic (familial) or non-genetic (acquired) origin.[12] Genetic cardiomyopathies usually are caused by sarcomere or cytoskeletal diseases, neuromuscular disorders, inborn errors of metabolism, malformation syndromes and sometimes are unidentified.[13][14] Non-genetic cardiomyopathies can have definitive causes such as viral infections, myocarditis and others.[15][16]

    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

    celiac disease can cause cardiomyopathies, which can completely reverse with a timely diagnosis.[22] In addition to acquired causes, molecular biology and genetics have given rise to the recognition of various genetic causes.[20][23]

    A more clinical categorization of cardiomyopathy as 'hypertrophied', 'dilated', or 'restrictive',[24] 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.[25]

    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.[26]

    Mechanism

    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.[27]

    Cardiomyopathies are generally varied individually. Different factors can cause Cardiomyopathies in adults as well as children. To exemplify, Dilated Cardiomyopathy in adults is associated with Ischemic Cardiomyopathy, Hypertension, Valvular diseases, and Genetics. While in Children, Neuromuscular diseases such as Becker muscular dystrophy, including X-linked genetic disorder, are directly linked with their Cardiomyopathies.[28]

    Diagnosis

    Normal sinus rhythm on EKG

    Among the diagnostic procedures done to determine a cardiomyopathy are:[29]

    Classification

    Structural categories of cardiomyopathy
    Stained microscopic section of heart muscle in hypertrophic cardiomyopathy

    Cardiomyopathies can be classified using different criteria:[30]

    Treatment

    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

    heart transplant.[29]

    See also

    References

    1. ^ a b c d e f "What Are the Signs and Symptoms of Cardiomyopathy?". NHLBI. 22 June 2016. Archived from the original on 15 September 2016. Retrieved 31 August 2016.
    2. ^ a b c "Who Is at Risk for Cardiomyopathy?". NHLBI. 22 June 2016. Archived from the original on 16 August 2016. Retrieved 31 August 2016.
    3. ^ a b c d e f g h "Types of Cardiomyopathy". NHLBI. 22 June 2016. Archived from the original on 28 July 2016. Retrieved 31 August 2016.
    4. ^ a b c d e "What Causes Cardiomyopathy?". NHLBI. 22 June 2016. Archived from the original on 15 September 2016. Retrieved 31 August 2016.
    5. ^ a b c d "How Is Cardiomyopathy Treated?". NHLBI. 22 June 2016. Archived from the original on 15 September 2016. Retrieved 31 August 2016.
    6. ^
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    24. from the original on 27 May 2013. Retrieved 11 November 2010.
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    29. ^ a b "What Are the Signs and Symptoms of Cardiomyopathy? - NHLBI, NIH". nhlbi.nih.gov. Archived from the original on 28 July 2016. Retrieved 25 July 2016.
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    31. ^ from the original on 20 August 2016. Retrieved 1 August 2016.
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    34. from the original on 29 May 2016.

    Further reading

    External links