Myocarditis

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Myocarditis
Classification and external resources
Viral myocarditis (1).JPG
Histopathological image of myocarditis at autopsy in a patient with acute onset of congestive heart failure
ICD-10 I09.0, I51.4
ICD-9 391.2, 422, 429.0
DiseasesDB 8716
MedlinePlus 000149
eMedicine article/156330 article/890740 article/1612533
Patient UK Myocarditis
MeSH D009205

Myocarditis or inflammatory cardiomyopathy is inflammation of heart muscle (myocardium).

Myocarditis is most often due to infection by common viruses, such as parvovirus B19, less commonly nonviral pathogens such as Borrelia burgdorferi (Lyme disease) or Trypanosoma cruzi, or as a hypersensitivity response to drugs.[1]

The definition of myocarditis varies, but the central feature is an infection of the heart, with an inflammatory infiltrate, and damage to the heart muscle, without the blockage of coronary arteries that define a heart attack (myocardial infarction) or other common noninfectious causes.[2] Myocarditis may or may not include death (necrosis) of heart tissue. It may include dilated cardiomyopathy.[1]

Myocarditis is often an autoimmune reaction. Streptococcal M protein and coxsackievirus B have regions (epitopes) that are immunologically similar to cardiac myosin. During and after the viral infection, the immune system may attack cardiac myosin.[1]

Because a definitive diagnosis requires a heart biopsy, which doctors are reluctant to do because they are invasive, statistics on the incidence of myocarditis vary widely.[1]

The consequences of myocarditis thus also vary widely. It can cause a mild disease without any symptoms that resolves itself, or it may cause chest pain, heart failure, or sudden death. An acute myocardial infarction-like syndrome with normal coronary arteries has a good prognosis. Heart failure, even with dilated left ventricle, may have a good prognosis. Ventricular arrhythmias and high-degree heart block have a poor prognosis. Loss of right ventricular function is a strong predictor of death.[1]

Signs and symptoms[edit]

The signs and symptoms associated with myocarditis are varied, and relate either to the actual inflammation of the myocardium or to the weakness of the heart muscle that is secondary to the inflammation. Signs and symptoms of myocarditis include:[3]

  • Chest pain (often described as "stabbing" in character)
  • Congestive heart failure (leading to edema, breathlessness and hepatic congestion)
  • Palpitations (due to arrhythmias)
  • Sudden death (in young adults, myocarditis causes up to 20% of all cases of sudden death)[4]
  • Fever (especially when infectious, e.g. in rheumatic fever)
  • Symptoms in infants and toddlers tend to be more nonspecific, with generalized malaise, poor appetite, abdominal pain, and/or chronic cough. Later stages of the illness will present with respiratory symptoms with increased work of breathing, and is often mistaken for asthma.

Since myocarditis is often due to a viral illness, many patients give a history of symptoms consistent with a recent viral infection, including fever, rash, diarrhea, joint pains, and easy fatigability.

Myocarditis is often associated with pericarditis, and many patients present with signs and symptoms that suggest concurrent myocarditis and pericarditis.

Causes[edit]

A large number of causes of myocarditis have been identified, but often a cause cannot be found. In Europe and North America, viruses are common culprits. Worldwide, however, the most common cause is Chagas' disease, an illness endemic to Central and South America that is due to infection by the protozoan Trypanosoma cruzi.[3]

Infections[edit]

Bacterial myocarditis is rare in patients without immunodeficiency.

Toxins[edit]

Immunologic[edit]

Physical agents[edit]

Diagnosis[edit]

Endomyocardial biopsy specimen with extensive eosinophilic infiltrate involving the endocardium and myocardium (hematoxylin and eosin stain)

Myocarditis refers to an underlying process that causes inflammation and injury of the heart. It does not refer to inflammation of the heart as a consequence of some other insult. Many secondary causes, such as a heart attack, can lead to inflammation of the myocardium and therefore the diagnosis of myocarditis cannot be made by evidence of inflammation of the myocardium alone.[6]

Myocardial inflammation can be suspected on the basis of electrocardiographic (ECG) results, elevated C-reactive protein (CRP) and/or Erythrocyte sedimentation rate (ESR), and increased IgM (serology) against viruses known to affect the myocardium. Markers of myocardial damage (troponin or creatine kinase cardiac isoenzymes) are elevated.[3]

The ECG findings most commonly seen in myocarditis are diffuse T wave inversions; saddle-shaped ST-segment elevations may be present (these are also seen in pericarditis).[3]

The gold standard is still biopsy of the myocardium, in general done in the setting of angiography. A small tissue sample of the endocardium and myocardium is taken, and investigated by a pathologist by light microscopy and—if necessary—immunochemistry and special staining methods. Histopathological features are myocardial interstitium with abundant edema and inflammatory infiltrate, rich in lymphocytes and macrophages. Focal destruction of myocytes explains the myocardial pump failure.[3]

Cardiac magnetic resonance imaging (cMRI or CMR) has been shown to be very useful in diagnosing myocarditis by visualizing markers for inflammation of the myocardium.[7] Recently, consensus criteria for the diagnosis of myocarditis by CMR have been published [8]

Treatment[edit]

As most viral infections cannot be treated with directed therapy, symptomatic treatment is the only form of therapy for those forms of myocarditis.[9] In the acute phase, supportive therapy, including bed rest, is indicated. For symptomatic patients, digoxin and diuretics provide clinical improvement. For patients with moderate to severe dysfunction, cardiac function can be supported by use of inotropes such as Milrinone in the acute phase, followed by oral therapy with ACE inhibitors (Captopril, Lisinopril) when tolerated. People who do not respond to conventional therapy are candidates for bridge therapy with left ventricular assist devices. Heart transplantation is reserved for patients who fail to improve with conventional therapy.[10]

In several small case series and randomized control trials, systemic corticosteroids have shown to have beneficial effects in patients with proven myocarditis.[10] However, data on the usefulness of corticosteroids should be interpreted with caution, since 58% of adults recover spontaneously, while most studies on children and infants lack control groups.[9] Studies have shown no benefit for the use of herbal medicine on all cause mortality in viral myocarditis.[11]

Epidemiology[edit]

The exact incidence of myocarditis is unknown. However, in series of routine autopsies, 1–9% of all patients had evidence of myocardial inflammation. In young adults, up to 20% of all cases of sudden death are due to myocarditis.[3]

Among patients with HIV, myocarditis is the most common cardiac pathological finding at autopsy, with a prevalence of 50% or more.[1]

History[edit]

Cases of myocarditis have been documented as early as the 1600s,[12] but the term "myocarditis", implying an inflammatory process of the myocardium, was introduced by German physician Joseph Friedrich Sobernheim in 1837.[13] However, the term has been conflated with other cardiovascular conditions, such as hypertension and ischaemic heart disease;[14] Following admonition regarding the indiscriminate use of myocarditis as a diagnosis from authorities such as British cardiologist Sir Thomas Lewis and American cardiologist and a co-founder of the American Heart Association Paul White, myocarditis was under-diagnosed.[14]

Although myocarditis is clinically and pathologically clearly defined as "inflammation of the myocardium", its definition, classification, diagnosis, and treatment are subject to continued controversy, but endomyocardial biopsy has helped define the natural history of myocarditis and clarify clinicopathological correlations.[15]

References[edit]

  1. ^ a b c d e f Leslie T. Cooper, Jr., (April 9, 2009) Myocarditis, N. Engl. J. Med. 360(15) 1526-38
  2. ^ Kenneth L. Baughman, Special Report: Diagnosis of Myocarditis; Death of Dallas Criteria. Circulation. 2006;113:593-595 Free full text
  3. ^ a b c d e f Feldman AM, McNamara D (November 2000). "Myocarditis". N. Engl. J. Med. 343 (19): 1388–98. doi:10.1056/NEJM200011093431908. PMID 11070105. 
  4. ^ Eckart RE, Scoville SL, Campbell CL, et al. (December 2004). "Sudden death in young adults: a 25-year review of autopsies in military recruits". Ann. Intern. Med. 141 (11): 829–34. doi:10.7326/0003-4819-141-11-200412070-00005. PMID 15583223. 
  5. ^ Nicholson PJ, Quinn MJ, Dodd JD (December 2010). "Headshop heartache: acute mephedrone 'meow' myocarditis". Heart (British Cardiac Society) 96 (24): 2051–2. doi:10.1136/hrt.2010.209338. PMID 21062771. 
  6. ^ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; & Mitchell, Richard N. (2007). Robbins Basic Pathology (8th ed.). Saunders Elsevier. pp. 414-416 ISBN 978-1-4160-2973-1
  7. ^ Skouri HN, Dec GW, Friedrich MG, Cooper LT (2006). "Noninvasive imaging in myocarditis". J. Am. Coll. Cardiol. 48 (10): 2085–93. doi:10.1016/j.jacc.2006.08.017. PMID 17112998. 
  8. ^ Friedrich MG, Sechtem U, Schulz-Menger J, Holmvang G, Alakija P, Cooper LT, White JA, Abdel-Aty H, Gutberlet M, Prasad S, Aletras AH, Laissy JP, Paterson I, Filipchuk NG, Kumar A, Pauschinger M, Liu P (2009). "Cardiovascular Magnetic Resonance in Myocarditis: A JACC White Paper". J. Am. Coll. Cardiol. 53 (17): 1475–87. doi:10.1016/j.jacc.2009.02.007. PMC 2743893. PMID 19389557. 
  9. ^ a b Hia, CP; Yip, WC, Tai, BC, Quek, SC (June 2004). "Immunosuppressive therapy in acute myocarditis: an 18 year systematic review". Archives of Disease in Childhood 89 (6): 580–4. doi:10.1136/adc.2003.034686. PMC 1719952. PMID 15155409. 
  10. ^ a b Aziz, KU; Patel, N; Sadullah, T; Tasneem, H; Thawerani, H; Talpur, S (October 2010). "Acute viral myocarditis: role of immunosuppression: a prospective randomised study". Cardiology in the young 20 (5): 509–15. doi:10.1017/S1047951110000594. PMID 20584348. 
  11. ^ Liu, Z.L.; Liu, Z.J.; Liu, J.P.; Kwong, J.S. (28 August 2013). "Herbal medicines for viral myocarditis". Heart Group. Cochrane Database of Systematic Reviews (John Wiley & Sons) (8): Art. no. CD003711. doi:10.1002/14651858.CD003711.pub5. PMID 23986406. (subscription required (help)). 
  12. ^ P. Schölmerich. (1983.) "Myocarditis — Cardiomyopathy Historic Survey and Definition", International Boehringer Mannheim Symposia, 1:5.
  13. ^ Joseph Friedrich Sobernheim. (1837.) Praktische Diagnostik der inneren Krankheiten mit vorzueglicher Ruecksicht auf pathologische Anatomic. Hirschwald, Berlin, 117.
  14. ^ a b Eckhardt G. J. Olsen. (1985.) "What is myocarditis?", Heart and Vessels, 1(1):S1-3.
  15. ^ Jared W. Magnani; G. William Dec. (2006.) "Myocarditis", Circulation, 113:876-890.

External links[edit]