Resolution of Acquired von Willebrand Disease Secondary to Hypertrophic Obstructive Cardiomyopathy Following Septal Myectomy.

Johnathan Hoggarth, Harry Rakowski, Erik Yeo, Anthony Ralph-Edwards


We report the case of a patient with hypertrophic obstructive cardiomyopathy, severe gastrointestinal bleeding and acquired von Willebrand syndrome. Pre-operative blood tests showed normal von Willebrand factor activity with decrease of high molecular weight multimers. Septal myectomy was performed in treatment of his hypertrophic disease. Follow-up blood tests indicated normal von Willebrand factor activity and high molecular weight multimers levels. Gastrointestinal bleeding has not recurred following surgery. In conclusion, septal myectomy resolves von Willebrand syndrome secondary to hypertrophic obstructive cardiomyopathy. von Willebrand factor Multimer testing can be used for the diagnosis of acquired von Willebrand syndrome which should be considered in patients who have gastrointestinal bleeding coinciding with hypertrophic obstructive cardiomyopathy.

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