Occult Gastrointestinal Bleeding due to Acquired von Willebrand Syndrome in a Patient with Hypertrophic Obstructive Cardiomyopathy

Abstract

Abstract

Hypertrophic Obstructive Cardiomyopathy, Bleeding History, and Acquired von Willebrand Syndrome: Response to Septal Myectomy

“…An association between HOCM and gastrointestinal hemorrhage and angiodysplasia has been reported several times, [10][11][12][13][14][15][16] and resolution of the bleeding disorder has also been reported with definitive therapies to reduce the out- flow gradient, including β-blockade, alcohol septal ablation, and septal myectomy. 10,[15][16][17] Aside from one previous case report of a patient with HOCM and a history of bleeding who had confirmation of loss of HMWMs and some qualitative improvement after intensification of medical therapy, 10 no reports have been published regarding the response of HMWMs to pharmacolgocial therapy, surgery, or alcohol septal ablation. Our first and fifth cases appeared to have qualitatively less severe loss of HMWMs with increased β-blocker therapy, one preoperatively and the other postoperatively (Figures 2 and 5), suggesting dynamism in HMWMs paralleling changes in gradient.…”

Clinical and laboratory diagnosis of von Willebrand disease: A synopsis of the 2008 NHLBI/NIH guidelines

“…Although VWF:RCo values are typically decreased (with variably decreased VWF:Ag and/or FVIII) in AVWS, sometimes only VWF multimer analysis is abnormal, with mild reduction or loss of the highest molecular weight multimers. Evolving information [7][8][9][10][11] suggests this latter situation is more likely for AVWS associated with enhanced VWF proteolysis reflecting shear-induced VWF conformational changes leading to increased proteolysis of VWF, such as with aortic valvular stenosis and other conditions causing abnormally high shear blood flow (Table I).…”

Functional Impairment of Von Willebrand Factor in Hypertrophic Cardiomyopathy

“…The occurrence of severe angiodysplasia hemorrhage has been described in HCM, [25][26][27][28] and VWF impairment has been demonstrated recently in 1 patient. 22 In some cases, bleeding cessation was obtained after initiation of ␤-blocker or calcium antagonist therapy, 22,26 after septal myectomy, 27 or after percutaneous alcohol septal ablation. 28 Although an acquired VWF syndrome has been suspected previously in HCM, 19 the present study emphasizes the frequent occurrence of VWF impairment, which can predispose to bleeding syndrome in the presence of bleeding-prone lesions or after invasive intervention.…”