Hypertrophic obstructive cardiomyopathy, bleeding history, and acquired von Willebrand syndrome: response to septal myectomy

Abstract

Bleeding with severe aortic stenosis is linked to acquired von Willebrand syndrome and loss of high-molecular-weight multimers of von Willebrand factor. Valve replacement resolves bleeding tendency and loss of high-molecular-weight multimers. We report outcomes in 5 patients with symptomatic obstructive hypertrophic cardiomyopathy and spontaneous gastrointestinal, mucosal, or excessive postsurgical bleeding in whom acquired von Willebrand syndrome was documented. All 5 patients underwent surgical septal myectomy with resolution of acquired von Willebrand syndrome.

FIGURE 1.

Apical 5-chamber 2-dimensional echocardiographic view for case 1 before (upper left) and after (upper right) septal myectomy eliminating systolic anterior motion. Intraoperative arterial pressure tracings demonstrate a high left ventricular to aortic gradient and a positive Brockenbrough-Braunwald-Morrow sign before surgery (lower left) and the absence of a gradient after surgery (lower right).

FIGURE 2.

Left, von Willebrand factor multimers and control plasma multimers for case 1 for each determination. The immediate preoperative sample was taken when the patient was taking a higher dose of β-blockers than on the previous 2 occasions. Loss of high-molecular-weight (HMW) multimers was reversed after surgery. Right, Densitometric scan of HMW multimer gels. LMW = low-molecular-weight.

FIGURE 3.

von Willebrand factor multimers and simultaneous control plasma multimers before and after surgery in cases 2 (left) and 4 (right).

FIGURE 4.

Echocardiographic images for case 3 before and after surgery and corresponding von Willebrand factor and control plasma multimers. Preoperative and postoperative echocardiographic results for this case are available at www.mayoclinicproceedings.com/content/86/3/219/suppl/DC1.

FIGURE 5.

Magnetic resonance imaging studies before and after septal myectomy in case 5. von Willebrand factor and simultaneous control plasma multimers before surgery on β-blocker therapy, after surgery off β-blocker therapy, and after surgery on β-blocker therapy. Preoperative and postoperative magnetic resonance imaging results for this case are available at www.mayoclinicproceedings.com/content/86/3/219/suppl/DC1.