Skip to main content

Advertisement

Log in

Plasma exchange for the patients with dilated cardiomyopathy in children is safe and effective in improving both cardiac function and daily activities

  • Original Article
  • Apheresis
  • Published:
Journal of Artificial Organs Aims and scope Submit manuscript

Abstract

Autoantibodies against cardiac proteins play an important role in the development of dilated cardiomyopathy (DCM). The efficacy and safety of apheresis such as immunoadsorption (IA) or plasma exchange (PE) to remove such antibodies have been reported in adult DCM patients. However, apheresis for pediatric DCM has not been performed because of technical difficulty due to relatively low blood volume and instability of hemodynamics. As we have experiences of preforming apheresis on hemodynamically unstable children, we have preformed ten courses of PE on seven child DCM patients including both patients in chronic and acute phase to assess the safety and efficacy to PE. Under general anesthesia, the patients were administered PE three times during 3 days as 1 course. Simultaneously, continuous hemodiafiltration (CHDF) was performed in series with the PE circuit to stabilize hemodynamic status and to minimize the adverse effects of PE. The changes in LVEF, CTR, mBP, the dosage of furosemide and NYHA were assessed before and after the procedure of PE. There were no severe adverse effects such as systemic bleeding or refractory hypotension due to apheresis. Echocardiography showed that mean baseline LVEF was 24.3 ± 7.8%. Mean LVEF significantly increased 1 week after PE to 30.5 ± 12.5%. CTR significantly decreased after PE. Mean BP significantly increased 1 month after PE (54.5 ± 10.7 to 60.7 ± 9.8 mmHg). NYHA improved after PE significantly (NYHA; 3.4 ± 1.1 to 2.5 ± 1.1). PE is safe and effective in improving both cardiac function and daily activities.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Lipshultz SE, Sleeper LA, Towbin JA, et al. The incidence of pediatric cardiomyopathy in two regions of the United States. N Engl J Med. 2003;348:1647–55.

    Article  PubMed  Google Scholar 

  2. Singh TP, Almond CS, Piercey G, Gauvreau K. Current outcomes in US children with cardiomyopathy listed for heart transplantation. Circ Heart Fail. 2012;5:594–601.

    Article  PubMed  Google Scholar 

  3. Alexander PM, Daubeney PE, Nugent AW, et al. Long-term outcomes of dilated cardiomyopathy diagnosed during childhood: results from a national population-based study of childhood cardiomyopathy. Circulation. 2013;128:2039–46.

    Article  PubMed  Google Scholar 

  4. Hollander SA, Bernstein D, Yeh J, Dao D, Sun HY, Rosenthal D. Outcomes of children following a first hospitalization for dilated cardiomyopathy. Circ Heart Fail. 2012;5:437–43.

    Article  PubMed  Google Scholar 

  5. Baba A, Yoshikawa T, Fukuda Y, et al. Autoantibodies against M2-muscarinic acetylcholine receptors: new upstream targets in atrial fibrillation in patients with dilated cardiomyopathy. Eur Heart J. 2004;25:1108–15.

    Article  CAS  PubMed  Google Scholar 

  6. Iwata M, Yoshikawa T, Baba A, Anzai T, Mitamura H, Ogawa S. Autoantibodies against the second extracellular loop of beta1-adrenergic receptors predict ventricular tachycardia and sudden death in patients with idiopathic dilated cardiomyopathy. J Am Coll Cardiol. 2001;37:418–24.

    Article  CAS  PubMed  Google Scholar 

  7. Staudt A, Staudt Y, Dorr M, et al. Potential role of humoral immunity in cardiac dysfunction of patients suffering from dilated cardiomyopathy. J Am Coll Cardiol. 2004;44:829–36.

    Article  PubMed  Google Scholar 

  8. Cihakova D, Rose NR. Pathogenesis of myocarditis and dilated cardiomyopathy. Adv Immunol. 2008;99:95–114.

    Article  CAS  PubMed  Google Scholar 

  9. Jahns R, Boivin V, Schwarzbach V, Ertl G, Lohse MJ. Pathological autoantibodies in cardiomyopathy. Autoimmunity. 2008;41:454–61.

    Article  CAS  PubMed  Google Scholar 

  10. Lappe JM, Pelfrey CM, Tang WH. Recent insights into the role of autoimmunity in idiopathic dilated cardiomyopathy. J Card Fail. 2008;14:521–30.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Okazaki T, Tanaka Y, Nishio R, et al. Autoantibodies against cardiac troponin I are responsible for dilated cardiomyopathy in PD-1-deficient mice. Nat Med. 2003;9:1477–83.

    Article  CAS  PubMed  Google Scholar 

  12. Yoshizawa A, Nagai S, Baba Y, et al. Autoimmunity against M(2)muscarinic acetylcholine receptor induces myocarditis and leads to a dilated cardiomyopathy-like phenotype. Eur J Immunol. 2012;42:1152–63.

    Article  CAS  PubMed  Google Scholar 

  13. Sugiyama H, Hoshiai M, Sugita K, Matsuda K. Plasma exchange for removal of antibeta1-adrenergic receptor antibody in a small child with dilated cardiomyopathy. Pediatr Cardiol. 2009;30:374–6.

    Article  PubMed  Google Scholar 

  14. Baba A. Autoantigen estimation and simple screening assay against cardiodepressant autoantibodies in patients with dilated cardiomyopathy. Ther Apher Dial Off Peer Rev J Int Soc Apher Jpn Soc Apher Jpn Soc Dial Ther. 2008;12:109–116.

    Google Scholar 

  15. Felix SB, Staudt A, Dorffel WV, et al. Hemodynamic effects of immunoadsorption and subsequent immunoglobulin substitution in dilated cardiomyopathy: three-month results from a randomized study. J Am Coll Cardiol. 2000;35:1590–8.

    Article  CAS  PubMed  Google Scholar 

  16. Muller J, Wallukat G, Dandel M, et al. Immunoglobulin adsorption in patients with idiopathic dilated cardiomyopathy. Circulation. 2000;101:385–91.

    Article  CAS  PubMed  Google Scholar 

  17. Christ T, Dobrev D, Wallukat G, Schuler S, Ravens U. Acute hemodynamic effects during immunoadsorption in patients with dilated cardiomyopathy positive for beta 1-adrenoceptor autoantibodies. Methods Find Exp Clin Pharmacol. 2001;23:141–4.

    Article  CAS  PubMed  Google Scholar 

  18. Schimke I, Muller J, Priem F, et al. Decreased oxidative stress in patients with idiopathic dilated cardiomyopathy one year after immunoglobulin adsorption. J Am Coll Cardiol. 2001;38:178–83.

    Article  CAS  PubMed  Google Scholar 

  19. Staudt A, Schaper F, Stangl V, et al. Immunohistological changes in dilated cardiomyopathy induced by immunoadsorption therapy and subsequent immunoglobulin substitution. Circulation. 2001;103:2681–6.

    Article  CAS  PubMed  Google Scholar 

  20. Konovalov GA, Belenkov Iu N, Zvezdkin PV, et al. Apheresis of immunoglobulins—new approach to the treatment of severe dilated cardiomyopathy. Kardiologiia. 2002;42:92–6.

    CAS  PubMed  Google Scholar 

  21. Staudt A, Dorr M, Staudt Y, et al. Role of immunoglobulin G3 subclass in dilated cardiomyopathy: results from protein A immunoadsorption. Am Heart J. 2005;150:729–36.

    Article  CAS  PubMed  Google Scholar 

  22. Felix SB, Staudt A. Non-specific immunoadsorption in patients with dilated cardiomyopathy: mechanisms and clinical effects. Int J Cardiol. 2006;112:30–3.

    Article  PubMed  Google Scholar 

  23. Nagatomo Y, Baba A, Ito H, et al. Specific immunoadsorption therapy using a tryptophan column in patients with refractory heart failure due to dilated cardiomyopathy. J Clin Apher. 2011;26:1–8.

    Article  PubMed  Google Scholar 

  24. Staudt A, Staudt Y, Hummel A, et al. Effects of immunoadsorption on the nt-BNP and nt-ANP plasma levels of patients suffering from dilated cardiomyopathy. Ther Apher Dial Off Peer Rev J Int Soc Apher Jpn Soc Apher Jpn Soc Dial Ther. 2006;10:42–48.

    CAS  Google Scholar 

  25. Trimpert C, Herda LR, Eckerle LG, et al. Immunoadsorption in dilated cardiomyopathy: long-term reduction of cardiodepressant antibodies. Eur J Clin Invest. 2010;40:685–91.

    Article  CAS  PubMed  Google Scholar 

  26. Ing RJ, Ames WA, Chambers NA. Paediatric cardiomyopathy and anaesthesia. Br J Anaesth. 2012;108:4–12.

    Article  CAS  PubMed  Google Scholar 

  27. Winters JL. Apheresis in the treatment of idiopathic dilated cardiomyopathy. J Clin Apher. 2012;27:312–9.

    Article  PubMed  Google Scholar 

  28. Sadahiro T, Hirasawa H, Oda S, et al. Usefulness of plasma exchange plus continuous hemodiafiltration to reduce adverse effects associated with plasma exchange in patients with acute liver failure. Crit Care Med. 2001;29:1386–92.

    Article  CAS  PubMed  Google Scholar 

  29. Hsia TY, Ringewald JM, Stroud RE, et al. Plasma profiling determinants of matrix homeostasis in paediatric dilated cardiomyopathy. Cardiol Young. 2011;21:52–61.

    Article  PubMed  Google Scholar 

  30. Lipshultz SE, Orav EJ, Wilkinson JD, et al. Risk stratification at diagnosis for children with hypertrophic cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy Registry. Lancet. 2013;382:1889–97.

    Article  PubMed  PubMed Central  Google Scholar 

  31. Jahns R, Boivin V, Hein L, et al. Direct evidence for a beta 1-adrenergic receptor-directed autoimmune attack as a cause of idiopathic dilated cardiomyopathy. J Clin Invest. 2004;113:1419–29.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Baba A, Akaishi M, Shimada M, et al. Complete elimination of cardiodepressant IgG3 autoantibodies by immunoadsorption in patients with severe heart failure. Circ J Off J Jpn Circ Soc. 2010;74:1372–8.

    CAS  Google Scholar 

  33. Torre-Amione G, Orrego CM, Khalil N, et al. Therapeutic plasma exchange a potential strategy for patients with advanced heart failure. J Clin Apher. 2010;25:323–30.

    Article  PubMed  Google Scholar 

  34. Ameling S, Herda LR, Hammer E, et al. Myocardial gene expression profiles and cardiodepressant autoantibodies predict response of patients with dilated cardiomyopathy to immunoadsorption therapy. Eur Heart J. 2013;34:666–75.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

I appreciate prof. Kanji Sugita, M.D., Ph.D., Takako Toda MD (University of Yamanashi School of Medicine, Department of Pediatrics) for conducting the present study. I appreciate Brittany Davis and Andrew Taylor for an act of proofreading. This study is supported by Yamanashi University research endowment program.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Takeshi Moriguchi.

Ethics declarations

Conflict of interest

There are no conflicts of interest, including related consultancies, shareholdings and funding grants.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Moriguchi, T., Koizumi, K., Matsuda, K. et al. Plasma exchange for the patients with dilated cardiomyopathy in children is safe and effective in improving both cardiac function and daily activities. J Artif Organs 20, 236–243 (2017). https://doi.org/10.1007/s10047-017-0956-7

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10047-017-0956-7

Keywords

Navigation