Immunadsorption therapy for end stage heart failure due to Duchenne muscular dystrophy

• 作者: Christian Willaschek Jochen Selbach Reiner Buchhorn Roland Jahns
• 来源期刊: 儿科学期刊(英文)
• 学科: 医学
• 关键词: DUCHENNE MUSCULAR DYSTROPHY IMMUNOADSORPTION Therapy Heart Failure Betablocker Cardiomyopathy
• 年，卷（期）: ekxqkyw_2013,（1）
• 页码范围: 17-19
• 页数: 3页
• 分类号: R5

摘要

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.