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

Abstract

trointestinal bleeding after septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy-associated acquired von Willebrand syndrome. J Thromb Haemost 2015; 13: 191-6.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.

Acquired bleeding disorders in the elderly

“…A study evaluating patients with hypertrophic cardiomyopathy showed that VWF levels were not predictive of bleeding, but older age, female gender, and higher peak outflow velocity measurements were. 35 A smaller study in mitral regurgitation also showed more bleeding with older age. This study actually showed higher VWF:Ag or VWF:Act levels in the bleeding patients but showed a significantly lower VWF:Act/VWF:Ag ratio and that this ratio decreased with MR severity.…”

Von Willebrand factor multimer quantitation for assessment of cardiac lesion severity and bleeding risk

“…We identified patients with cardiac lesions from our multispecialty practice between 2010 and 2016 as previously described 2, 3, 4, 7. The study protocol was approved by the Mayo Foundation Institutional Review Board is registered at clinicaltrials.gov (NCT01334801).…”

“…Subjects with LVADs were assumed to have severe lesions. Assessment of the severity of AS, AR, MR, prosthetic valvular dysfunction, and HOCM have been described previously 2, 3, 4, 7. All echocardiograms were reviewed for accuracy (J.L.B.…”

“…We identified patients with cardiac lesions from our multispecialty practice between 2010 and 2016 as previously described. [2][3][4]7 The study protocol was approved by the Mayo Foundation Institutional Review Board is registered at clinicaltrials.gov (NCT01334801). The protocol allowed inclusion of data from patients who had valve disorders and had VWF laboratory testing performed for the clinical indication of acquired bleeding and was further expanded to include patients with valvular or obstructive cardiac lesions that did not have bleeding.…”

“…1 Though loss of HMW VWF multimers and associated bleeding was first described in AS, recent descriptions of a similar constellation of findings has been associated with a variety of cardiac lesions including aortic regurgitation, mitral insufficiency and hypertrophic cardiomyopathy. [2][3][4] Additionally, VWF abnormalities have been described after valve replacement and repair and have been attributed to prosthetic valve dysfunction, perivalvular leak, patient prosthesis mismatch, [5][6][7] and in the setting of mechanical support with left ventricular assist devices. 8 More recently, abnormalities of VWF multimers have been identified in the absence of clinical bleeding and studies suggest that VWF multimers may provide utility as a biological assay for cardiac lesions in real-time and chronic settings.…”