OJPed  Vol.3 No.1 , March 2013
Immunadsorption therapy for end stage heart failure due to Duchenne muscular dystrophy
Abstract: We report about a successful immunadsorption therapy of a boy with end stage heart failure due to Duchenne muscular dystrophy who has little chance to get cardiac transplantation. Prior to this therapy a medical therapy with an angiotensin converting enzyme inhibitor, a low dose betablocker, an aldosterone antagonist, and diuretics failed. In consent with the patient and his parents immunoadsorption therapy employing a protein A column was performed. Due to clinical improvement the betablocker carvedilol could be titrated from 6.25 mg up to 30 mg. In the following 4 month he improves from NYHA class IV to NYHA class II and NT-Pro-BNP levels fell from 5180 pg/ml to 402 pg/ml. The mean heart rate in Holter ECG decreases from 102/min to 68/min and ejection fraction improved from 25% to 30%. The boy began to walk without any support and was able to visit school. This clinical improvement now holds on for 2 years.
Cite this paper: Buchhorn, R. , Willaschek, C. , Selbach, J. and Jahns, R. (2013) Immunadsorption therapy for end stage heart failure due to Duchenne muscular dystrophy. Open Journal of Pediatrics, 3, 17-19. doi: 10.4236/ojped.2013.31003.

[1]   Mavrogeni, S., Papavasiliou, A., Spargias, C., Constandouklakis, P., Papadopoulos, G., Karanasios, E., et al. (2010) Myocardial inflammation in Duchenne muscular dystrophy as a precipitating factor of heart failure: A prospective study. BMC Neurology, 10, 33-40. doi:10.1186/1471-2377-10-33

[2]   Rosenthal, D., Chrisant, M.R., Edens, E., Mahony, L., Canter, C., Colan, S., et al. (2004) International society for heart and lung transplantation: Practice guidelines for management of heart failure in children. Journal of Heart Lung Transplant, 23, 1313-1333. doi:10.1016/j.healun.2004.03.018

[3]   Nikolaev, V.O., Boivin, V., Stork, S., Angermann, C.E., Ertl, G. and Lohse, M.J. (2007) A novel fluorescence method for rapid detection of functional β1-adrenergic receptor autoantibodies in heart failure. Journal of the American College of Cardiology, 50, 423-431. doi:10.1016/j.jacc.2007.03.051?

[4]   Ikeda, U., Kasai, H., Izawa, A., Koyama, J., Yazaki, Y., Takahashi, M., et al. (2008) Immunoadsorption therapy for patients with dilated cardiomyopathy and heart failure. Current Cardiology Reviews, 4, 219-222. doi:10.2174/157340308785160534

[5]   Dandel, M., Wallukat, G., Englert, A., Lehmkuhl, H.B., Knosalla, C. and Hetzer, R. (2012) Long-term benefits of immunadsorption in β(1)-adrenoceptor-autoantibody-positive transplant candidates with dilated cardiomyopathy. European Journal of Heart Failure, 14, 1374-1388. doi:10.1093/eurjhf/ hfs123

[6]   Kajimoto, H., Ishigaki, K., Okumura, K., Tomimatsu, H., Nakazawa, M, Saito, K., et al. (2006) Beta-blocker therapy for cardiac dysfunction in patients with muscular dystrophy. Circulation Journal, 70, 991-994. doi:10.1253/circj.70.991

[7]   Nishizawa, T. and Yasuma, F. (2007-2012) The preventive efficacy of carvedilol on cardiac dysfunction in duchenne muscular dystrophy. Indentifier. Current Study NCT00606775.

[8]   Hor, K.N., Wansapura, J.P., Al Khalidi, H.R., Gottliebson, Taylor, M.D., Czosek, R., et al. (2011) Presence of mechanical dyssynchrony in Duchenne muscular dystrophy. Journal of Cardiovascular Magnetic Resonance, 13, 12. doi:10.1186/1532-429X-13-12

[9]   Stork, S., Boivin, V., Horf, R., et al. (2006) Stimulating autoantibodies directed against the cardiac beta1-adrenergic receptor predict increased mortality in idiopathic cardiomyopathy. American Heart Journal, 152, 697-704. doi:10.1016/j.ahj.2006.05.004