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

• 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
• DOI: 10.1111/jth.12780
• EISSN: 1538-7836
• ISSN: 1538-7933; 1538-7836

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.