Myocarditis natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-In-Chief: Varun Kumar M.B.B.S., Maliha Shakil, M.D. [2]

Overview

Myocarditis is usually self limiting and is associated with a good prognosis especially if it is secondary to a viral infection. Patients rarely develop cardiac failure, pulmonary edema, arrhythmias, or cardiogenic shock. In some instances, myocarditis may be associated with sudden death. Patients with fulminant myocarditis have a good long term prognosis if they survive the acute phase of the disease.[1] The prognosis of fulminant myocarditis is better than that of either acute myocarditis or giant cell myocarditis. The presence of left bundle branch block, q waves, AV block, syncope and a left ventricular ejection fraction < 40% are associated with sudden death and cardiac transplantation.[2]

Natural History

The course of viral myocarditis is usually benign and the majority of cases of Coxsackie B virus infection are subclinical. Patients presenting with mild ventricular dysfunction secondary to viral myocarditis typically improve within weeks to months and rarely progress to severe ventricular dysfunction, heart block, arrhythmias, or even sudden cardiac death.[3][4] Among patients who present with more advanced left ventricular dysfunction; 50% of patients develop chronic ventricular dysfunction and 25% have spontaneous improvement in ventricular function while the remaining 25% progress to transplantation or death.[5]

Complications

Complications of myocarditis include:[3][4][5]

Prognosis

Endomyocardial Biopsy

An endomyocardial biopsy is usually obtained in patients presenting with advanced heart failure or arrhythmias. Endomyocardial biopsy can shed light on the prognosis by ascertaining the underlying cause and the histopathologic severity of the disease.

Fas and Fas-ligand

Fas and Fas ligand (cell death receptors) are associated with apoptotic death of myocytes and are a marker of cardiac dysfunction.[6]

Antimyosin Autoantibodies

Antimyosin autoantibodies are associated with left ventricular systolic dysfunction and diastolic stiffness in patients with chronic myocarditis.[7]

Persistence of the Viral Genome

Persistence of the viral genome in the myocardium is associated with worsening of left ventricular ejection fraction.[8][9]

Giant Cell Myocarditis

Giant cell myocarditis (GCM) is a less common form of myocarditis which usually occurs in relatively young and healthy adults. It is associated with a poorer prognosis. [10]

Prognostic Implications of EKG Changes

Despite its worrisome appearance, ST segment elevation suggestive of myocardial infarction is usually self-limited with no overt sequelae.[11] In contrast, the presence of either left bundle branch block, q waves suggestive of old infarct or high degree AV block is associated with a poor long term prognosis, development of cardiac failure and the need for cardiac transplantation.[12]

Clinical Predictors of Prognosis

The development of syncope, bundle branch block, ejection fraction <40%, and pulmonary hypertension are known to be poor predictors of myocarditis and are associated with death or cardiac transplantation.

Prognosis Associated with Left Ventricular Dysfunction

The prognosis in patients with new onset heart failure depends on the degree of ventricular dysfunction.[5] The majority of myocarditis patients recover well with treatment. However, approximately 25% of patients develop chronic ventricular dysfunction and 25% of patients will continue to deteriorate.[13]

Prognosis Associated with Fulminant Myocarditis vs Acute Myocarditis

In a small series of 15 patients with fulminant myocarditis, 14(93%) survived for 11 years without the need for cardiac transplantation. This suggests that patients with fulminant myocarditis have a good long term prognosis if they survive the acute phase of the disease.[1] In the same series, 132 patients met the criteria for acute myocarditis and 60(45%) of these patients were alive at the end of 11 years without having received a cardiac transplant.

Prognostic Biomarkers

High levels of interleukin-10 in fulminant myocarditis patients at admission may be predictive of subsequent development of cardiogenic shock (requiring mechanical cardiopulmonary support system) and mortality.[14]

References

  1. 1.0 1.1 McCarthy RE, Boehmer JP, Hruban RH, Hutchins GM, Kasper EK, Hare JM; et al. (2000). "Long-term outcome of fulminant myocarditis as compared with acute (nonfulminant) myocarditis". N Engl J Med. 342 (10): 690–5. doi:10.1056/NEJM200003093421003. PMID 10706898.
  2. Scartazzini R, Schneider P, Bickel H (1975). "[New beta-lactam antibiotics. Functionalisation of the cephem 3-position with sulfur or nitrogen bearing substituents (author's transl)]". Helv Chim Acta. 58 (8): 2437–50. doi:10.1002/hlca.19750580824. PMID 1194054.
  3. 3.0 3.1 Wentworth P, Jentz LA, Croal AE (1979). "Analysis of sudden unexpected death in southern Ontario, with emphasis on myocarditis". Can Med Assoc J. 120 (6): 676–80, 706. PMC 1819176. PMID 436050.
  4. 4.0 4.1 Hosenpud JD, McAnulty JH, Niles NR (1986). "Unexpected myocardial disease in patients with life threatening arrhythmias". Br Heart J. 56 (1): 55–61. PMC 1277385. PMID 3730208.
  5. 5.0 5.1 5.2 Magnani JW, Dec GW (2006). "Myocarditis: current trends in diagnosis and treatment". Circulation. 113 (6): 876–90. doi:10.1161/CIRCULATIONAHA.105.584532. PMID 16476862.
  6. Sheppard R, Bedi M, Kubota T, Semigran MJ, Dec W, Holubkov R; et al. (2005). "Myocardial expression of fas and recovery of left ventricular function in patients with recent-onset cardiomyopathy". J Am Coll Cardiol. 46 (6): 1036–42. doi:10.1016/j.jacc.2005.05.067. PMID 16168288. Unknown parameter |http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom= ignored (help)
  7. Lauer B, Schannwell M, Kühl U, Strauer BE, Schultheiss HP (2000). "Antimyosin autoantibodies are associated with deterioration of systolic and diastolic left ventricular function in patients with chronic myocarditis". J Am Coll Cardiol. 35 (1): 11–8. PMID 10636253. Unknown parameter |http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom= ignored (help)
  8. Kühl U, Pauschinger M, Seeberg B, Lassner D, Noutsias M, Poller W; et al. (2005). "Viral persistence in the myocardium is associated with progressive cardiac dysfunction". Circulation. 112 (13): 1965–70. doi:10.1161/CIRCULATIONAHA.105.548156. PMID 16172268. Unknown parameter |http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom= ignored (help)
  9. Cooper LT (2009). "Myocarditis". N Engl J Med. 360 (15): 1526–38. doi:10.1056/NEJMra0800028. PMID 19357408. Unknown parameter |http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom= ignored (help)
  10. Cooper LT, Berry GJ, Shabetai R (1997). "Idiopathic giant-cell myocarditis--natural history and treatment. Multicenter Giant Cell Myocarditis Study Group Investigators". N Engl J Med. 336 (26): 1860–6. doi:10.1056/NEJM199706263362603. PMID 9197214.
  11. Dec GW, Waldman H, Southern J, Fallon JT, Hutter AM, Palacios I (1992). "Viral myocarditis mimicking acute myocardial infarction". J Am Coll Cardiol. 20 (1): 85–9. PMID 1607543. Unknown parameter |http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom= ignored (help)
  12. Nakashima H, Katayama T, Ishizaki M, Takeno M, Honda Y, Yano K (1998). "Q wave and non-Q wave myocarditis with special reference to clinical significance". Jpn Heart J. 39 (6): 763–74. PMID 10089938.
  13. Magnani JW, Danik HJ, Dec GW, DiSalvo TG (2006). "Survival in biopsy-proven myocarditis: a long-term retrospective analysis of the histopathologic, clinical, and hemodynamic predictors". Am Heart J. 151 (2): 463–70. doi:10.1016/j.ahj.2005.03.037. PMID 16442915.
  14. Nishii M, Inomata T, Takehana H, Takeuchi I, Nakano H, Koitabashi T; et al. (2004). "Serum levels of interleukin-10 on admission as a prognostic predictor of human fulminant myocarditis". J Am Coll Cardiol. 44 (6): 1292–7. doi:10.1016/j.jacc.2004.01.055. PMID 15364334. Unknown parameter |http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom= ignored (help)

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