TITLE:
Immunadsorption therapy for end stage heart failure due to Duchenne muscular dystrophy
AUTHORS:
Reiner Buchhorn, Christian Willaschek, Jochen Selbach, Roland Jahns
KEYWORDS:
Duchenne Muscular Dystrophy, Immunoadsorption Therapy, Heart Failure, Betablocker, Cardiomyopathy
JOURNAL NAME:
Open Journal of Pediatrics,
Vol.3 No.1,
March
11,
2013
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.