Key result
Immunoadsorption therapy in a boy with end-stage heart failure due to Duchenne muscular dystrophy led to clinical improvement from NYHA class IV to II and an ejection fraction increase from 25% to 30%.
Why the study?
Does immunoadsorption therapy improve clinical status and cardiac function in a pediatric patient with Duchenne muscular dystrophy and end-stage heart failure?
Case Report (n=1)
Does immunoadsorption therapy improve clinical status and cardiac function in a pediatric patient with Duchenne muscular dystrophy and end-stage heart failure?
Immunoadsorption therapy may be a viable rescue strategy to enable beta-blocker up-titration and improve cardiac function in end-stage heart failure due to Duchenne muscular dystrophy.
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.
No takes yet. Share an insight, caveat, or question.
Buchhorn et al. (2013) conducted a case report in End stage heart failure due to Duchenne muscular dystrophy (n=1). Immunoadsorption therapy was evaluated on Clinical improvement (NYHA class, NT-Pro-BNP, heart rate, ejection fraction). Immunoadsorption therapy in a boy with end-stage heart failure due to Duchenne muscular dystrophy led to clinical improvement from NYHA class IV to II and an ejection fraction increase from 25% to 30%.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: