Cardiomyopathy Explained

Cardiomyopathy
Field:Cardiology
Treatment:Depends on type and symptoms
Frequency:2.5 million with myocarditis (2015)
Deaths:354,000 with myocarditis (2015)

Cardiomyopathy is a group of primary diseases of the heart muscle. Early on there may be few or no symptoms. As the disease worsens, shortness of breath, feeling tired, and swelling of the legs may occur, due to the onset of heart failure. An irregular heart beat and fainting may occur.[1] Those affected are at an increased risk of 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."[3] [4] Types of cardiomyopathy include hypertrophic cardiomyopathy, dilated cardiomyopathy, restrictive cardiomyopathy, arrhythmogenic right ventricular dysplasia, and Takotsubo cardiomyopathy (broken heart syndrome). In hypertrophic cardiomyopathy the heart muscle enlarges and thickens. In dilated cardiomyopathy the ventricles enlarge and weaken. In restrictive cardiomyopathy the ventricle stiffens.[5]

In many cases, the cause cannot be determined. Hypertrophic cardiomyopathy is usually inherited, whereas dilated cardiomyopathy is inherited in about one third of cases. Dilated cardiomyopathy may also result from alcohol, heavy metals, coronary artery disease, cocaine use, and viral infections. Restrictive cardiomyopathy may be caused by amyloidosis, hemochromatosis, and some cancer treatments.[6] Broken heart syndrome is caused by extreme emotional or physical stress.[5]

Treatment depends on the type of cardiomyopathy and the severity of symptoms. Treatments may include lifestyle changes, medications, or surgery. Surgery may include a ventricular assist device or heart transplant.[7] In 2015 cardiomyopathy and myocarditis affected 2.5 million people.[8] Hypertrophic cardiomyopathy affects about 1 in 500 people while dilated cardiomyopathy affects 1 in 2,500.[5] [9] They resulted in 354,000 deaths up from 294,000 in 1990.[10] [11] Arrhythmogenic right ventricular dysplasia is more common in young people.[2]

Signs and symptoms

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 coronary artery disease, hypertension, or abnormalities of the heart valves.[17] Often, the underlying cause remains unknown, but in many cases the cause may be identifiable.[18] Alcoholism, for example, has been identified as a cause of dilated cardiomyopathy, as has drug toxicity, and certain infections (including Hepatitis C).[19] [20] [21] Untreated 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.[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

thumb|175 px|Normal sinus rhythm on EKGAmong the diagnostic procedures done to determine a cardiomyopathy are:[29]

Classification

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 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.[29]

See also

Further reading

Notes and References

  1. Web site: What Are the Signs and Symptoms of Cardiomyopathy?. NHLBI. 31 August 2016. 22 June 2016. live. https://web.archive.org/web/20160915080931/http://www.nhlbi.nih.gov/health/health-topics/topics/cm/signs. 15 September 2016.
  2. Web site: Who Is at Risk for Cardiomyopathy? . NHLBI. 31 August 2016. 22 June 2016 . live. https://web.archive.org/web/20160816115709/https://www.nhlbi.nih.gov/health/health-topics/topics/cm/atrisk. 16 August 2016.
  3. Book: Harrison's principles of internal medicine . 2022 . McGraw Hill . 978-1-264-26850-4 . 21st . New York . 1954.
  4. Arbustini . Eloisa . Narula . Navneet . Dec . G. William . Reddy . K. Srinath . Greenberg . Barry . Kushwaha . Sudhir . Marwick . Thomas . Pinney . Sean . Bellazzi . Riccardo . Favalli . Valentina . Kramer . Christopher . Roberts . Robert . Zoghbi . William A. . Bonow . Robert . Tavazzi . Luigi . 3 December 2013 . The MOGE(S) Classification for a Phenotype–Genotype Nomenclature of Cardiomyopathy . Journal of the American College of Cardiology . en . 62 . 22 . 2046–2072 . 10.1016/j.jacc.2013.08.1644. 24263073 . 43240625 .
  5. Web site: Types of Cardiomyopathy. NHLBI. 31 August 2016. 22 June 2016. live. https://web.archive.org/web/20160728023515/http://www.nhlbi.nih.gov/health/health-topics/topics/cm/types. 28 July 2016.
  6. Web site: What Causes Cardiomyopathy? . NHLBI. 31 August 2016. 22 June 2016. live. https://web.archive.org/web/20160915092001/https://www.nhlbi.nih.gov/health/health-topics/topics/cm/causes. 15 September 2016.
  7. Web site: How Is Cardiomyopathy Treated?. NHLBI. 31 August 2016. 22 June 2016 . live. https://web.archive.org/web/20160915082544/https://www.nhlbi.nih.gov/health/health-topics/topics/cm/treatment. 15 September 2016.
  8. ((GBD 2015 Disease and Injury Incidence and Prevalence Collaborators)) . 8 October 2016 . Global, regional, and national incidence, prevalence, and years lived with disability for 310 diseases and injuries, 1990-2015: a systematic analysis for the Global Burden of Disease Study 2015. . Lancet . 388 . 10053 . 1545–1602 . 10.1016/S0140-6736(16)31678-6 . 5055577 . 27733282.
  9. Book: Practical Cardiovascular Pathology . 2010 . Lippincott Williams & Wilkins . 978-1-60547-841-8 . 148 . live . https://web.archive.org/web/20160914000626/https://books.google.ca/books?id=ZjaOUqf9LEQC&pg=PA148 . 14 September 2016.
  10. ((GBD 2015 Mortality and Causes of Death Collaborators)) . 8 October 2016 . Global, regional, and national life expectancy, all-cause mortality, and cause-specific mortality for 249 causes of death, 1980-2015: a systematic analysis for the Global Burden of Disease Study 2015. . Lancet . 388 . 10053 . 1459–1544 . 10.1016/s0140-6736(16)31012-1 . 5388903 . 27733281.
  11. ((GBD 2013 Mortality and Causes of Death Collaborators)) . 17 December 2014 . Global, regional, and national age-sex specific all-cause and cause-specific mortality for 240 causes of death, 1990-2013: a systematic analysis for the Global Burden of Disease Study 2013 . Lancet . 385 . 9963 . 117–71 . 10.1016/S0140-6736(14)61682-2 . 4340604 . 25530442.
  12. Bakalakos . Athanasios . Ritsatos . Konstantinos . Anastasakis . Aris . 2018-09-01 . Current perspectives on the diagnosis and management of dilated cardiomyopathy Beyond heart failure: a Cardiomyopathy Clinic Doctor's point of view . Hellenic Journal of Cardiology . en . 59 . 5 . 254–261 . 10.1016/j.hjc.2018.05.008 . 29807197 . 44146977 . 1109-9666. free .
  13. Rath . Anika . Weintraub . Robert . 2021-07-23 . Overview of Cardiomyopathies in Childhood . Frontiers in Pediatrics . 9 . 708732 . 10.3389/fped.2021.708732 . 2296-2360 . 8342800 . 34368032 . free.
  14. Gorla . Sudheer . Raja . Kishore . Garg . Ashish . Barbouth . Deborah . Rusconi . Paolo . December 2018 . Infantile Onset Hypertrophic Cardiomyopathy Secondary to PRKAG2 Gene Mutation is Associated with Poor Prognosis . Journal of Pediatric Genetics . en . 07 . 4 . 180–184 . 10.1055/s-0038-1657763 . 2146-4596 . 6234042 . 30430036.
  15. Law . Michelle L. . Cohen . Houda . Martin . Ashley A. . Angulski . Addeli Bez Batti . Metzger . Joseph M. . February 2020 . Dysregulation of Calcium Handling in Duchenne Muscular Dystrophy-Associated Dilated Cardiomyopathy: Mechanisms and Experimental Therapeutic Strategies . Journal of Clinical Medicine . en . 9 . 2 . 520 . 10.3390/jcm9020520 . 32075145 . 7074327 . 2077-0383. free.
  16. Cimiotti . Diana . Budde . Heidi . Hassoun . Roua . Jaquet . Kornelia . 2021-01-08 . Genetic Restrictive Cardiomyopathy: Causes and Consequences—An Integrative Approach . International Journal of Molecular Sciences . en . 22 . 2 . 558 . 10.3390/ijms22020558 . 1422-0067 . 7827163 . 33429969. free .
  17. Book: Harrison's Principles of Internal Medicine . McGraw-Hill . Kasper . DL . Fauci . AS . Hauser . SL . Longo . DL . Jameson . JL . Loscalzo . J . Lakdawala . NK . Stevenson . LW . Loscalzo . J . 2015 . Chapter 287 . 1553 . 19th . 978-0-07-180215-4.
  18. Book: Pathophysiology of heart disease: a collaborative project of medical students and faculty . 2011 . Wolters Kluwer/Lippincott Williams & Wilkins . Lilly, Leonard S. . 978-1-60547-723-7 . 5th . Baltimore, MD . 649701807.
  19. Adam A, Nicholson C, Owens L . Alcoholic dilated cardiomyopathy. . Nurs Stand . 2008 . 22 . 38 . 42–7 . 18578120 . Review . 10.7748/ns2008.05.22.38.42.c6565.
  20. Westphal JG, Rigopoulos AG, Bakogiannis C, Ludwig SE, Mavrogeni S, Bigalke B et al. . The MOGE(S) classification for cardiomyopathies: current status and future outlook. . Heart Fail Rev . 2017 . 22 . 6 . 743–752 . 28721466 . 10.1007/s10741-017-9641-4 . 36117047 . Review.
  21. Domont F, Cacoub P . Chronic hepatitis C virus infection, a new cardiovascular risk factor? . Liver Int . 2016 . 36 . 5 . 621–7 . 26763484 . 10.1111/liv.13064 . Review . free.
  22. Ciaccio EJ, Lewis SK, Biviano AB, Iyer V, Garan H, Green PH . Cardiovascular involvement in celiac disease. . World J Cardiol . 2017 . 9 . 8 . 652–666 . 28932354 . 10.4330/wjc.v9.i8.652 . 5583538 . Review . free .
  23. Simpson S, Rutland P, Rutland CS . Genomic Insights into Cardiomyopathies: A Comparative Cross-Species Review. . Vet Sci . 2017 . 4 . 1 . 19 . 29056678 . 10.3390/vetsci4010019 . 5606618 . Review . free.
  24. Book: Valentin Fuster . John Willis Hurst . Hurst's the heart . 11 November 2010 . 2004 . McGraw-Hill Professional . 978-0-07-143225-2 . 1884 . live . https://web.archive.org/web/20130527075031/http://books.google.com/books?id=eWQAJDrVV7gC&pg=PA1884 . 27 May 2013.
  25. Llamas-Esperón GA, Llamas-Delgado G . Hypertrophic cardiomyopathy. Proposal for a new classification . Arch Cardiol Mex . 92 . 3 . 377–389 . 2022 . 35772124 . 9262289 . 10.24875/ACM.21000301 .
  26. Cardiomyopathy Classification: Ongoing Debate in the Genomics Era . McCartan C, Maso R, Jayasinghe SR, Griffiths LR . 2012 . Biochem Res Int . 2012 . 796926 . 22924131 . 10.1155/2012/796926 . 3423823 . free.
  27. Cellular mechanisms of cardiomyopathy . The Journal of Cell Biology . 2011-08-08 . 0021-9525 . 3153638 . 21825071 . 355–365 . 194 . 3 . 10.1083/jcb.201101100 . Pamela A. . Harvey . Leslie A. . Leinwand.
  28. Braunwald . Eugene . 2017-09-15 . Cardiomyopathies: An Overview . Circulation Research . 121 . 7 . 711–721 . 10.1161/CIRCRESAHA.117.311812 . 1524-4571 . 28912178 . 36384619. free .
  29. Web site: What Are the Signs and Symptoms of Cardiomyopathy? - NHLBI, NIH . live . https://web.archive.org/web/20160728023343/http://www.nhlbi.nih.gov/health/health-topics/topics/cm/signs . 28 July 2016 . 25 July 2016 . nhlbi.nih.gov.
  30. Book: Vinay, Kumar . Robbins Basic Pathology . Elsevier . 2013 . 978-1-4377-1781-5 . 396.
  31. Maron. Barry J.. Towbin. Jeffrey A. . Thiene . Gaetano . Antzelevitch . Charles . Corrado . Domenico . Arnett . Donna . Moss . Arthur J. . Seidman . Christine E. . Young . James B. . 11 April 2006 . Contemporary Definitions and Classification of the Cardiomyopathies . live . Circulation . en . 113 . 14 . 1807–1816 . 10.1161/CIRCULATIONAHA.106.174287 . 0009-7322 . 16567565 . 6660623 . https://web.archive.org/web/20160820130306/http://circ.ahajournals.org/content/113/14/1807 . 20 August 2016 . 1 August 2016 .
  32. Séguéla PE, Iriart X, Acar P, Montaudon M, Roudaut R, Thambo JB . Eosinophilic cardiac disease: Molecular, clinical and imaging aspects . Archives of Cardiovascular Diseases . 108 . 4 . 258–68 . 2015 . 25858537 . 10.1016/j.acvd.2015.01.006 . free.
  33. Rose NR . Viral myocarditis . Current Opinion in Rheumatology . 28 . 4 . 383–9 . 2016 . 27166925 . 4948180 . 10.1097/BOR.0000000000000303.
  34. Book: Pediatric Metabolic Syndrome: Comprehensive Clinical Review and Related Health Issues . Lipshultz . Steven E. . Messiah . Sarah E. . Miller . Tracie L. . 2012-04-05 . Springer Science & Business Media . 978-1-4471-2365-1 . 200 . https://web.archive.org/web/20160529024647/https://books.google.com/books?id=9GZkyP5NYPwC . 29 May 2016 . live.

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