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.

Natural history

Myocarditis with myocardial infarction like ECG changes are usually self limiting with no overt sequelae[1]. Patients with ECG changes suggestive of bundle branch block, old infarct or high degree AV block are associated with poor long term prognosis and may progress to develop cardiac failure. Viral myocarditis are usually benign and 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, may progress to severe ventricular dysfunction and may lead to arrhythmias or even death[2]. Though only a small fraction of patients with lymphocytic myocarditis develop ventricular arrhythmias, idiopathic ventricular tachycardia is seen in upto 33% of these patients[3].

Complications

As mentioned above, myocarditis may present with ventricular dysfunction and may progress to cardiac failure due to presence of scar tissue. This can further lead to development of pulmonary edema, arrhythmias or cardiogenic shock.

Other complications include recurrent myositis, cardiac conduction abnormalities and may even lead to sudden death[3].

Prognosis

Endomyocardial biopsy which is the primary diagnostic tool, is usually used in patients presenting with advanced heart failure or arrhythmias and thus long term prognosis in mild cases is not known. Prognosis in myocarditis varies with the cause and the severity of the disease.

  • The prognosis in patients with new onset heart failure depends on degree of ventricular dysfunction[5]. Majority of these patients recover well with treatment. Approximately 25% of patients develop chronic ventricular dysfunction and 25% of patients continue to deteriorate[6]
  • In a series, among 15 patients who met the criteria for fulminant myocarditis, 14(93%) patients survived for 11 years without the need for heart transplant. This suggests that patients with fulminant myocarditis have a good long term prognosis if they survive the acute phase of the disease[7]. 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 transplant.
  • Serological markers such as Fas, Fas ligand, interleukin-10 or antimyosin autoantibodies are of prognostic value in myocarditis.
    • Fas and Fas ligand can lead to apoptotic death of myocytes and thus causing cardiac dysfunction. A study evaluating the role of gene expression for predicting myocardial recovery in recent-onset cardiomyopathy, reported that patients in the highest tertile of Fas expression had minimal improvement at six months when compared with the intermediate and lowest tertiles[8].
    • Antimyosin autoantibodies are associated with left ventricular systolic dysfunction and diastolic stiffness in patients with chronic myocarditis[9].
    • 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[10].
  • Idiopathic giant cell myocarditis (GCM) is a rare type of myocarditis which usually occurs in relatively young and predominantly healthy adults. It is frequently associated with poor prognosis. In a series of 63 patients with idiopathic giant-cell myocarditis, death and cardiac transplantation accounted for 89% and the median survival was 5.5 months from the onset of symptoms. Of the 34 patients who underwent cardiac transplantation, 9 patients (26%) died during an average follow-up of 3.7 years and 5 died within 30 days post surgery[13].

References

  1. 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.
  2. 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.
  3. 3.0 3.1 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.
  4. 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.
  5. 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. 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.
  7. 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.
  8. 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.
  9. 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.
  10. 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.
  11. 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.
  12. Cooper LT (2009). "Myocarditis". N Engl J Med. 360 (15): 1526–38. doi:10.1056/NEJMra0800028. PMID 19357408.
  13. 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.

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