The incidence of acquired von Willebrand syndrome (AvWS) in patients with heart disease is commonly perceived as rare. However, its occurrence is underestimated and underdiagnosed, potentially leading to inadequate treatment resulting in increased morbidity and mortality.In patients with cardiac disease, AvWS frequently occurs in patients with structural heart disease and in those undergoing mechanical circulatory support (MCS).The clinical manifestation of an AvWS is usually characterized by apparent or occult gastrointestinal (GI) or mucocutaneous hemorrhage frequently accompanied by signs of anemia and/or increased bleeding during surgical procedures. The primary change is loss of high-molecular weight von Willebrand factor multimers (HMWM). Whereas the loss of HMWM in patients with structural heart disease is caused by increased HMWM cleavage by von Willebrand factor (vWF)-cleaving protease, ADAMTS13, AvWS in MCS patients is predominantly a result of a high shear stress coupled with mechanical destruction of vWF itself.This manuscript provides a comprehensive review of the evidence regarding both diagnosis and contemporary management of AVWS in patients with heart disease.
We present a patient with ruptured suprarenal aortic aneurysm, involving origins of visceral and renal arteries. Associated spondylodiscitis and left psoas muscle abscess were also diagnosed. The patient was initially treated with antibiotics. Diagnostic survey showed progression of the aneurysm diameter and enlargement of the psoas muscle abscess. Surgical treatment using a cryopreserved aortic homograft with debranching of visceral arteries was performed. Different modalities of surgical repair within the infected aortic segment and the rationale for usage of cryopreserved homografts are considered. The importance of optimal timing for surgery is emphasized as well.
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