Remission of recurrent gastrointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy‐associated acquired von Willebrand syndrome

Summary Background Gastrointestinal hemorrhage is considered to be a severe complication of von Willebrand disease. The optimal therapy for acquired von Willebrand syndrome and severe gastrointestinal bleeding with hypertrophic cardiomyopathy is undefined. Patients/Methods Seventy‐seven patients (me...

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Bibliographic Details
Published in:	Journal of thrombosis and haemostasis Vol. 13; no. 2; pp. 191 - 196
Main Authors:	Blackshear, J. L., Stark, M. E., Agnew, R. C., Moussa, I. D., Safford, R. E., Shapiro, B. P., Waldo, O. A., Chen, D.
Format:	Journal Article
Language:	English
Published:	England Elsevier Limited 01-02-2015
Subjects:	Adult Aged Aged, 80 and over Blood Transfusion Cardiac Surgical Procedures cardiomyopathy hypertrophic Cardiomyopathy, Hypertrophic - complications Cardiomyopathy, Hypertrophic - diagnosis Cardiomyopathy, Hypertrophic - surgery endoscopy Female gastrointestinal Gastrointestinal Hemorrhage - blood Gastrointestinal Hemorrhage - diagnosis Gastrointestinal Hemorrhage - etiology Gastrointestinal Hemorrhage - therapy Heart Septum - diagnostic imaging Heart Septum - surgery hemorrhage Humans Male Middle Aged Remission Induction Risk Factors Severity of Illness Index Treatment Outcome Ultrasonography von Willebrand diseases von Willebrand Diseases - blood von Willebrand Diseases - diagnosis von Willebrand Diseases - etiology von Willebrand Diseases - therapy von Willebrand factor gastrointestinal endoscopy cardiomyopathy hypertrophic von Willebrand diseases von Willebrand factor hemorrhage
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Summary:	Summary Background Gastrointestinal hemorrhage is considered to be a severe complication of von Willebrand disease. The optimal therapy for acquired von Willebrand syndrome and severe gastrointestinal bleeding with hypertrophic cardiomyopathy is undefined. Patients/Methods Seventy‐seven patients (median age, 67 years; interquartile range [IQR], 56–75 years; 49% women) with hypertrophic cardiomyopathy underwent von Willebrand factor multimer testing and acquisition of bleeding history. Bleeding was detected in 27 (36%) (median age, 74 years; IQR 66–76 years; 74% women), 20 with gastrointestinal bleeding, including 11 women with transfusion dependence. In these 11 women, the median duration of transfusion dependency was 36 months (IQR 18–44 months), and the median number of transfusions required was 25 (IQR 20–38). Two patients had undergone bowel resection for bleeding, one of them twice. Seven patients showed angiodysplasia, and the remainder had no endoscopic lesion. Bleeding recurred after bowel surgery or endoscopic intervention and medical therapy for hypertrophic cardiomyopathy in 10 of 11 patients. Two patients had septal myectomy, and six patients underwent alcohol septal ablation. With the exception of one patient in whom a significant gradient persisted after septal ablation, after the periprocedural period, patients after septal reduction therapy remained free of recurrent bleeding and need for transfusions. Conclusion Acquired von Willebrand syndrome is common in hypertrophic cardiomyopathy. Gastrointestinal bleeding often recurs after endoscopic therapy, but may be relieved by structural cardiac repair.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	1538-7933 1538-7836 1538-7836
DOI:	10.1111/jth.12780