编辑“︁心肌病”︁

| name = 心肌病<br/>Cardiomyopathy

| caption = 切開的左[[心室]]，可以看到心内膜下的增厚，扩张和纤维化，伴隨著[[心内膜]]白色部份的增加

| causes = 不明、[[遗传学|遗传]]、[[酒精]]、[[有毒重金屬|重金属]]、[[淀粉样变]]、壓力<ref name=NIH2016Cau/><ref name=NIH2016Type/>

| frequency = 2015年有250萬人罹患心肌病和[[心肌炎]]<ref name=GBD2015Pre>{{cite journal|last1=GBD 2015 Disease and Injury Incidence and Prevalence|first1=Collaborators.|title=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.|journal=Lancet|date=2016-10-08|volume=388|issue=10053|pages=1545–1602|pmid=27733282|doi=10.1016/S0140-6736(16)31678-6|pmc=5055577}}</ref>

| deaths = 2015年有35萬4千人因為心肌炎而死亡<ref name=GBD2015De>{{cite journal|last1=GBD 2015 Mortality and Causes of Death|first1=Collaborators.|title=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.|journal=Lancet|date=2016-10-08|volume=388|issue=10053|pages=1459–1544|pmid=27733281|doi=10.1016/s0140-6736(16)31012-1|pmc=5388903}}</ref>

'''心肌病'''<ref>https://www.termonline.cn/word/1324163693122019330/1</ref>（{{lang-en|cardiomyopathy}}）也称'''心肌病變'''，是一组由不同病因引起的心脏机械和电活动异常的异质性[[心肌]]疾病的通称，以心肌形态、结构和功能异常为特点。

心肌病在[[组织病理]]上，以心肌细胞发生[[变性]]、[[坏死]]为主，相对于[[心肌炎]]较无炎症变化，即使[[炎症性心肌病]]（inflammatory cardiomyopathy）也较初发急性心肌炎时较少炎症变化<ref>Tschöpe C, Ammirati E, Bozkurt B, Caforio ALP, Cooper LT, Felix SB, Hare JM, Heidecker B, Heymans S, Hübner N, Kelle S, Klingel K, Maatz H, Parwani AS, Spillmann F, Starling RC, Tsutsui H, Seferovic P, Van Linthout S. Myocarditis and inflammatory cardiomyopathy: current evidence and future directions. Nat Rev Cardiol. 2021 Mar;18(3):169-193. doi: 10.1038/s41569-020-00435-x. Epub 2020 Oct 12. PMID 33046850; PMCID: PMC7548534.</ref>。心肌病概括分为：肥厚性心肌病、扩张型心肌病、限制型心肌病、致心律失常性心肌病，和未分类心肌病，约5种类型；但各国际学术单位的分类方法均不同<ref>林曼欣, 吴林, 盛琴慧. 心肌病的分类及进展回顾 [J] . 中国心血管杂志, 2018, 23(1) : 81-86. DOI: 10.3969/j.issn.1007-5410.2018.01.019.</ref>。

==體徵及症狀==

[[File:Ventricular fibrillation.png|thumb|upright=1.3|心電圖上可以看出的心律失常、[[心室颤动]]]]

心肌病早期的症狀可能很輕微，甚至沒有症狀<ref name=NIH2016Sym/>。有些會是因為[[心臟衰竭]]而有[[呼吸困难]]、容易[[疲倦]]或腳部[[水肿]]的情形<ref name=NIH2016Sym/>，可能會有[[心律不整]]或是[[昏厥]]的症狀<ref name=NIH2016Sym>{{cite web|title=What Are the Signs and Symptoms of Cardiomyopathy?|url=http://www.nhlbi.nih.gov/health/health-topics/topics/cm/signs|website=NHLBI|accessdate=2016-08-31|date=2016-06-22|deadurl=no|archiveurl=https://web.archive.org/web/20160915080931/http://www.nhlbi.nih.gov/health/health-topics/topics/cm/signs|archivedate=2016-09-15}}</ref>。心肌病變也會增加[[心搏停止|心因性猝死]]的風險<ref name=NIH2016Risk>{{cite web|title=Who Is at Risk for Cardiomyopathy?|url=http://www.nhlbi.nih.gov/health/health-topics/topics/cm/atrisk|website=NHLBI|accessdate=2016-08-31|date=2016-06-22|deadurl=no|archiveurl=https://web.archive.org/web/20160816115709/https://www.nhlbi.nih.gov/health/health-topics/topics/cm/atrisk|archivedate=2016-08-16}}</ref>。

心肌病的症狀會包括疲倦、下肢腫脹以及[[呼吸困难]]<ref name="ref2">{{cite web|title=What Are the Signs and Symptoms of Cardiomyopathy? - NHLBI, NIH|url=https://www.nhlbi.nih.gov/health/health-topics/topics/cm/signs|website=www.nhlbi.nih.gov|accessdate=2016-07-25|deadurl=no|archiveurl=https://web.archive.org/web/20160728023343/http://www.nhlbi.nih.gov/health/health-topics/topics/cm/signs|archivedate=2016-07-28}}</ref>。進一步的症狀包括<ref name=ref2/>：

* [[心律失常]]

* [[昏厥]]

* [[頭暈]]

==病因==

心肌病變通常沒有明確的病因<ref name=NIH2016Cau/>。增生性心肌病變通常是[[遺傳]]自父母，擴張性心肌病變則有三分之一是遺傳所致<ref name=NIH2016Cau/>。擴張性心肌病變也可能是由[[酒精]]、[[重金屬]]、[[冠心症]]、吸食[[古柯鹼]]或[[病毒感染]]造成<ref name=NIH2016Cau/>。限制性心肌病變可能和[[澱粉樣變性]]、[[血色素沉著病]]或某些{{le|癌症治療|Treatment of cancer}}有關<ref name=NIH2016Cau>{{cite web|title=What Causes Cardiomyopathy?|url=http://www.nhlbi.nih.gov/health/health-topics/topics/cm/causes|website=NHLBI|accessdate=2016-08-31|date=2016-06-22|deadurl=no|archiveurl=https://web.archive.org/web/20160915092001/https://www.nhlbi.nih.gov/health/health-topics/topics/cm/causes|archivedate=2016-09-15}}</ref>。心碎症候群是由非常極端的情緒或生理壓力所造成<ref name=NIH2016Type/>。

心肌病可分為只和心臟有關的，或是整體系統性失調的一部份，但兩者都會造成心血管死亡，或是漸進性的，和心臟衰竭相關的病症。若是因為其他病症導致心肌功能的障礙（例如[[冠狀動脈疾病]]、[[高血壓]]或是[[心瓣膜]]的異常），則不列在心肌病中<ref name="Harrison">{{Cite book | title=Harrison's Principles of Internal Medicine | url=https://archive.org/details/isbn_9780071842327 | publisher=McGraw-Hill | editor-last1=Kasper | editor-first1=DL | editor-last2=Fauci | editor-first2=AS | editor-last3=Hauser | editor-first3=SL | editor-last4=Longo | editor-first4=DL | editor-last5=Jameson | editor-first5=JL | editor-last6=Loscalzo | editor-first6=J | last1=Lakdawala | first1=NK | last2=Stevenson | first2=LW | last3=Loscalzo | first3=J | year=2015 | chapter=Chapter 287 | page=[https://archive.org/details/isbn_9780071842327/page/1553 1553] | edition=19th | isbn=978-0-07-180215-4 }}</ref>。有些心肌病的病因仍不明，不過有些已識別出病因<ref>{{Cite book|url=https://www.worldcat.org/oclc/649701807|title=Pathophysiology of heart disease : a collaborative project of medical students and faculty|date=2011|publisher=Wolters Kluwer/Lippincott Williams & Wilkins|others=Lilly, Leonard S., Harvard Medical School.|isbn=1605477230|edition=5th|location=Baltimore, MD|oclc=649701807}}</ref>：例如酒精就是造成扩张型心肌病的病因，而藥物毒性及特定感染（例如[[丙型肝炎]]）也是心肌病的病因<ref name="AdamNicholson2008">{{cite journal| vauthors=Adam A, Nicholson C, Owens L| title=Alcoholic dilated cardiomyopathy. | journal=Nurs Stand | year= 2008 | volume= 22 | issue= 38 | pages= 42-7 | pmid=18578120 | type=Review }} </ref><ref name="WestphalRigopoulos2017">{{cite journal| vauthors=Westphal JG, Rigopoulos AG, Bakogiannis C, Ludwig SE, Mavrogeni S, Bigalke B et al.| title=The MOGE(S) classification for cardiomyopathies: current status and future outlook. | journal=Heart Fail Rev | year= 2017 | volume= 22 | issue= 6 | pages= 743-752 | pmid=28721466 | doi=10.1007/s10741-017-9641-4 | | type=Review }} </ref><ref name="DomontCacoub2016">{{cite journal | author=Domont F, Cacoub P | title=Chronic hepatitis C virus infection, a new cardiovascular risk factor? | journal=Liver Int | year=2016 | volume=36 | issue=5 | pages=621-7 | pmid=26763484 | doi=10.1111/liv.13064 | type=Review | url=http://hal.upmc.fr/hal-01274679/document | access-date=2018-06-19 | archive-date=2017-09-23 | archive-url=https://web.archive.org/web/20170923010210/http://hal.upmc.fr/hal-01274679/document | dead-url=no }}</ref>。 沒有治療的[[乳糜泻]]也會導致心肌病，在長時間的診斷後結果可能會完全不同<ref name="CiaccioLewis2017">{{cite journal| vauthors=Ciaccio EJ, Lewis SK, Biviano AB, Iyer V, Garan H, Green PH| title=Cardiovascular involvement in celiac disease. | journal=World J Cardiol | year= 2017 | volume= 9 | issue= 8 | pages= 652-666 | pmid=28932354 | doi=10.4330/wjc.v9.i8.652 | pmc=5583538 |type= Review }}</ref>。除了病因以外，目前也使用分子生物學以及基因學來識別不同的基因相關病因<ref name="WestphalRigopoulos2017" /><ref name="SimpsonRutland2017">{{cite journal| vauthors=Simpson S, Rutland P, Rutland CS| title=Genomic Insights into Cardiomyopathies: A Comparative Cross-Species Review. | journal=Vet Sci | year= 2017 | volume= 4 | issue= 1 | pmid=29056678 | doi=10.3390/vetsci4010019 | pmc=5606618 | type=Review }} </ref>。

心肌病較臨床上的分類，如「肥厚型」、「擴張型」、「限制型」等<ref name="FusterHurst2004">{{cite book|author1=Valentin Fuster|author2=John Willis Hurst|title=Hurst's the heart|url=https://books.google.com/books?id=eWQAJDrVV7gC&pg=PA1884|accessdate=2010-11-11|year=2004|publisher=McGraw-Hill Professional|isbn=978-0-07-143225-2|pages=1884–|deadurl=no|archiveurl=https://web.archive.org/web/20130527075031/http://books.google.com/books?id=eWQAJDrVV7gC&pg=PA1884|archivedate=2013-05-27}}</ref>，已經難以續用，因為有些病症在發展的過程中，可能同時會符合二種類型的條件。{{le|美國心臟協會|American Heart Association}}目前的定義中，將心肌病分為一級和二級，前者只影響心臟，後者則是由其他身體部位的疾病所造成。此二類再依新的基因及分子生物學，進一步細分為不同的次分類<ref>{{cite journal | title=Cardiomyopathy Classification: Ongoing Debate in the Genomics Era |vauthors=McCartan C, Maso R, Jayasinghe SR, Griffiths LR | date=2012 | journal=Biochem Res Int |volume=2012 | pages= 796926| pmid=22924131 | doi=10.1155/2012/796926 | pmc=3423823}}</ref>。

==機制==

心肌病的症狀可能包括體力消耗後的[[呼吸短促]]、疲勞，足部、腿部或腹部腫脹，另也可能有[[心律不整]]和胸痛的症狀<ref name=ref2/>。

隨著分子技術進步，心肌病的病理生理學在細胞的層面上已有較多的了解。突變的蛋白質會擾亂收縮器（或機械力敏感的複合體）中的心臟功能。心肌的變化以及其細胞層面上的持續反應其造成的變化與突發性心因性死亡及其他心臟問題相關<ref>{{Cite journal|title = Cellular mechanisms of cardiomyopathy|url = http://jcb.rupress.org/content/194/3/355|journal = The Journal of Cell Biology|date = 2011-08-08|issn = 0021-9525|pmc = 3153638|pmid = 21825071|pages = 355–365|volume = 194|issue = 3|doi = 10.1083/jcb.201101100|first = Pamela A.|last = Harvey|first2 = Leslie A.|last2 = Leinwand|deadurl = no|archiveurl = https://web.archive.org/web/20160928221709/http://jcb.rupress.org/content/194/3/355|archivedate = 2016-09-28}}</ref>。

==診斷==

[[file:SinusRhythmLabels.svg|thumb|175 px|正常[[竇性心律]]/心電圖]]

在診斷過程中，會用以下的程序來確診是否有心肌病<ref name=ref2/>：

* 身體檢查

* 家族病史

* [[血液檢查]]

* [[心電圖]]

* [[超声心动图|心臟超音波]]

* 心臟壓力測試

* [[基因檢測]]

==分型==

[[File:Tipet e kardiomiopative.png|300px|thumb|心肌病的結構性分類]]

[[File:HCM＿HE.jpg|thumb|肥厚型心肌病之伊紅染色]]

心肌病變包括{{le|增生性心肌病變|Hypertrophic cardiomyopathy}}、[[擴張性心肌病變]]、{{le|限制性心肌病變|Restrictive cardiomyopathy}}、{{le|導致心律不整的右心室發育不全|Arrhythmogenic right ventricular dysplasia}}及{{le|心碎症候群|Takotsubo cardiomyopathy}}<ref name=NIH2016Type/>增生性心肌病變是心臟肌肉變大且變厚<ref name=NIH2016Type/>，擴張性心肌病變是心室的腔室變大且無力<ref name=NIH2016Type/>。限制性心肌病變是心室壁硬化<ref name=NIH2016Type>{{cite web|title=Types of Cardiomyopathy|url=http://www.nhlbi.nih.gov/health/health-topics/topics/cm/types|website=NHLBI|accessdate=2016-08-31|date=2016-06-22|deadurl=no|archiveurl=https://web.archive.org/web/20160728023515/http://www.nhlbi.nih.gov/health/health-topics/topics/cm/types|archivedate=2016-07-28}}</ref>。

心肌病有幾種不同的分類標準<ref>{{Cite book|title=Robbins Basic Pathology|last=Vinay|first=Kumar|publisher=Elsevier|year=2013|isbn=978-1-4377-1781-5|location=|pages=396}}</ref>：

* 原發性/內源性心肌病<ref name="AMA">{{cite journal|last1=Maron|first1=Barry J.|last2=Towbin|first2=Jeffrey A.|last3=Thiene|first3=Gaetano|last4=Antzelevitch|first4=Charles|last5=Corrado|first5=Domenico|last6=Arnett|first6=Donna|last7=Moss|first7=Arthur J.|last8=Seidman|first8=Christine E.|last9=Young|first9=James B.|date=2006-04-11|title=Contemporary Definitions and Classification of the Cardiomyopathies|url=http://circ.ahajournals.org/content/113/14/1807|deadurl=no|journal=Circulation|language=en|volume=113|issue=14|pages=1807–1816|doi=10.1161/CIRCULATIONAHA.106.174287|issn=0009-7322|pmid=16567565|archiveurl=https://web.archive.org/web/20160820130306/http://circ.ahajournals.org/content/113/14/1807|archivedate=2016-08-20|accessdate=2016-08-01}}</ref>

** 遺傳性

*** {{le|肥厚型心肌病|Hypertrophic cardiomyopathy}}

*** {{le|致心律失常型右心室心肌病|Arrhythmogenic right ventricular cardiomyopathy}}（ARVC）

*** 孤立性左心室心肌緻密化不全

*** 離子通道病

*** {{le|擴張型心肌病|Dilated cardiomyopathy}}（DCM）

*** {{le|限制型心肌病|Restrictive cardiomyopathy}}（RCM）

** 後天性

*** {{le|壓力性心肌病|Stress cardiomyopathy}}

*** [[心肌炎]]：因為[[淋巴细胞]]及[[单核细胞]]滲入而導致的心臟組織損傷或發炎<ref name="pmid25858537">{{cite journal | vauthors = Séguéla PE, Iriart X, Acar P, Montaudon M, Roudaut R, Thambo JB | title = Eosinophilic cardiac disease: Molecular, clinical and imaging aspects | journal = Archives of Cardiovascular Diseases | volume = 108 | issue = 4 | pages = 258–68 | year = 2015 | pmid = 25858537 | doi = 10.1016/j.acvd.2015.01.006 | url = }}</ref><ref name="pmid27166925">{{cite journal | vauthors = Rose NR | title = Viral myocarditis | journal = Current Opinion in Rheumatology | volume = 28 | issue = 4 | pages = 383–9 | year = 2016 | pmid = 27166925 | pmc = 4948180 | doi = 10.1097/BOR.0000000000000303 | url = }}</ref>

*** {{le|嗜酸性粒細胞性心肌炎|Eosinophilic myocarditis}}：因為[[嗜酸性粒細胞]]滲入而導致的心臟組織損傷或發炎<ref name="pmid25858537"/>

*** {{le|缺血性心肌病|Ischemic cardiomyopathy}}<!-- (not formally included in the classification as a direct result of another cardiac problem)--><ref name="AMA"/>

* 繼發性/外在性心肌病<ref name="AMA" />

** 代謝/儲積

*** 法布瑞氏症

*** {{le|血色沉著症|hemochromatosis}}

** 心內膜

*** {{le|心內膜心肌纖維化|Endomyocardial fibrosis}}

*** [[嗜酸性粒细胞增多症]]

** 內分泌

*** [[糖尿病]]

*** [[甲狀腺功能亢進]]

*** {{le|末端肥大症|acromegaly}}

** 顎-心-臉症狀群

*** [[努南氏症候群]]

** 神經肌肉性

*** {{le|肌肉營養不良|muscular dystrophy}}

*** {{le|遺傳性共濟失調症|Friedreich's ataxia}}

** 其他因素

*** 肥胖相關之心肌病<ref>{{Cite book|url=https://books.google.com/books?id=9GZkyP5NYPwC|title=Pediatric Metabolic Syndrome: Comprehensive Clinical Review and Related Health Issues|last=Lipshultz|first=Steven E.|last2=Messiah|first2=Sarah E.|last3=Miller|first3=Tracie L.|date=2012-04-05|publisher=Springer Science & Business Media|isbn=9781447123651|page=200|archiveurl=https://web.archive.org/web/20160529024647/https://books.google.com/books?id=9GZkyP5NYPwC|archivedate=2016-05-29|deadurl=no}}</ref>

==治療==

治療是依照心肌病變的型式和嚴重度而定<ref name=NIH2016Tx/>，治療方式包括改變生活型態、藥物或手術<ref name=NIH2016Tx>{{cite web|title=How Is Cardiomyopathy Treated?|url=http://www.nhlbi.nih.gov/health/health-topics/topics/cm/treatment|website=NHLBI|accessdate=2016-08-31|date=2016-06-22|deadurl=no|archiveurl=https://web.archive.org/web/20160915082544/https://www.nhlbi.nih.gov/health/health-topics/topics/cm/treatment|archivedate=2016-09-15}}</ref>。在2013年時，罹患心肌病變和[[心肌炎]]的人數達到250萬人<ref name=GBD2015Pre/>，其中每500人有一人是罹患增生性心肌病變，而擴張性心肌病變則是在2500人中有一人<ref name=NIH2016Type/><ref>{{cite book|title=Practical Cardiovascular Pathology|date=2010|publisher=Lippincott Williams & Wilkins|isbn=9781605478418|page=148|url=https://books.google.ca/books?id=ZjaOUqf9LEQC&pg=PA148|deadurl=no|archiveurl=https://web.archive.org/web/20160914000626/https://books.google.ca/books?id=ZjaOUqf9LEQC&pg=PA148|archivedate=2016-09-14}}</ref>。同時，上述兩種心肌病變所造成的死亡人數，從1990年的294,000人上升到443,000人<ref name=GDB2013>{{cite journal|last1=GBD 2013 Mortality and Causes of Death|first1=Collaborators|title=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|journal=Lancet|date=2014-12-17|pmid=25530442|doi=10.1016/S0140-6736(14)61682-2|pmc=4340604|volume=385|issue=9963|pages=117–71}}</ref><ref name=GBD2015De/>。導致心律不整的右心房發育不全則較常見於年輕族群<ref name=NIH2016Risk/>。

心肌病的治療包括建議調整生活型態，以加強控制病症，另外治療也依心肌病的分類與病症的情況而定，但多數會包括投藥（保守的療法），心律過低者植入[[心律調節器]]，心律情況可能致死者使用[[去顫器]]，若有嚴重的心臟衰竭則採用{{le|心室輔助器|Ventricular assist device}}（VAD），若是有複發性心律失常，無法透過藥物或是機械性心臟復律復原的，會採用[[燒蝕]]治療。治療的目的多半是在症狀的緩解，有些病患會需要[[心脏移植]]。<ref name=ref2/>

{{Reflist|2}}

{{refbegin}}

* {{cite journal|last1=Boudina|first1=Sihem|last2=Abel|first2=Evan Dale|title=Diabetic cardiomyopathy, causes and effects|journal=Reviews in endocrine & metabolic disorders|date=2010-03-01|volume=11|issue=1|pages=31–39|doi=10.1007/s11154-010-9131-7|pmc=2914514|issn=1389-9155|pmid=20180026}}

* {{cite journal|last1=Marian|first1=A. J.|last2=Roberts|first2=Robert|title=The Molecular Genetic Basis for Hypertrophic Cardiomyopathy|journal=Journal of molecular and cellular cardiology|date=2001-04-01|volume=33|issue=4|pages=655–670|doi=10.1006/jmcc.2001.1340|pmc=2901497|issn=0022-2828|pmid=11273720}}

{{refend}}

==參見==

*[[體外反搏]] (External counterpulsation therapy (ECP))