Aortic Stenosis (AS) and Von Willebrand Disease (vWD)

Question 1

Symptoms of aortic stenosis?

Answer 1

Shortness of breath

Syncope

Question 2

Specific sign of aortic stenosis?

Answer 2

Ejection systolic murmur

Question 3

Causes of aortic stenosis?

Answer 3

Age-related calcification (commonest)
Congenital bicuspid aortic valves (vulnerable to calcification)
Rheumatic fever also increases calcification

Question 4

What can aortic stenosis lead to?

Answer 4

Heart failure
Angina
Heyde syndrome (colonic angiodysplasia, lower GI bleeding associated with aortic stenosis)
Von Willebrand Disease type 2A as large Von Willebrand factor molecule undergoes shear stress traversing the stenosed aortic valve (proteolysis component of 2A)

Question 5

What are the types of Von Willebrand Disease?

Answer 5

I - heterozygous for gene defect, reduced levels of vWF
II - normal vWF levels but molecule structurally abnormal
IIA - abnormality in synthesis of vWF or proteolysis of vWF (e.g. aortic stenosis)
IIB - increased function of vWF leading to spontaneous platelet binding
III - homozygous for gene defect resulting in severe bleeding tendencies