What is Hyde syndrome?
Table of Contents
What is Hyde syndrome?
Heyde syndrome is a multisystem disorder characterized by the triad of aortic stenosis (AS), gastrointestinal bleeding, and acquired von Willebrand syndrome. Heyde syndrome is an uncommon condition that can lead to severe morbidity and mortality if not recognized promptly.
How is Heyde syndrome treated?
Management of Heyde syndrome is challenging, with treatment options that include medical therapy, endoscopic interventions, colonic surgery and valve replacement. If valve replacement is performed, antiplatelet and anticoagulant agents should be used with caution, to minimize the risk of rebleeding.
Why does aortic stenosis cause bleeding?
A possible mechanism for the bleeding in Heyde’s syndrome is an acquired form of von Willebrand’s disease (type IIa) due to the aortic stenosis. High shear rates across the stenotic valve lead to increased consumption of high-molecular-weight multimers of von Willebrand factor (vWF).
Why does aortic stenosis cause AVM?
Aortic stenosis or sclerosis is associated with high shear stress, which elevates von Willebrand factorcleaving metalloprotease activity, leading to proteolysis of von Willebrand factor, and increases interactions between von Willebrand factor and platelets, leading to degradation or clearance of von Willebrand factor
What causes Heyde’s syndrome?
Heyde syndrome is a triad of aortic stenosis, an acquired coagulopathy and anaemia due to bleeding from intestinal angiodysplasia. The evidence that aortic stenosis is the root cause of this coagulopathy is compelling.
How does aortic stenosis cause Avms?
Management of Heyde syndrome is challenging, with treatment options that include medical therapy, endoscopic interventions, colonic surgery and valve replacement. If valve replacement is performed, antiplatelet and anticoagulant agents should be used with caution, to minimize the risk of rebleeding.
How common is Heyde syndrome?
Because of this, Heyde syndrome is likely underdiagnosed and underreported. Aortic stenosis is prevalent in approximately 7.5% of persons 75 years of age or older. [3] Furthermore, approximately 1.8% of persons over the age of 75 classify as having moderate or severe aortic stenosis.
Can aortic stenosis cause internal bleeding?
Heyde syndrome is a triad of aortic stenosis, an acquired coagulopathy and anaemia due to bleeding from intestinal angiodysplasia. The evidence that aortic stenosis is the root cause of this coagulopathy is compelling.
How does aortic stenosis affect coronary blood flow?
It has been hypothesized that the aortic stenosis causes an acquired von Willebrand factor deficiency that leads to GI bleeding. Aortic valve replacement, when possible, can prevent recurrent GI bleeding in these cases, but medical decisions in these cases are complex and difficult.
How does aortic stenosis cause von Willebrand disease?
Patients with severe aortic stenosis and no obstructed coronary arteries are reported to have reduced coronary flow. Doppler evaluation of proximal coronary flow is feasible using transesophageal echocardiography.
Why does aortic stenosis cause angiodysplasia?
Aortic-valve stenosis can be complicated by bleeding, particularly that due to gastrointestinal angiodysplasia (Heyde’s syndrome). This hemorrhagic syndrome is associated with acquired type 2A von Willebrand syndrome, which is characterized by the loss of the largest multimers of von Willebrand factor
Does aortic stenosis affect the brain?
This can lead to chest pain. As the pressure continues to rise, blood may back up into the lungs. Severe aortic stenosis can limit the amount of blood that reaches the brain and the rest of the body
How is Heyde’s syndrome diagnosed?
Initial evaluation of Heyde syndrome includes a CBC, coagulation studies, metabolic panel, and test for fecal occult blood. The Platelet Function Assay (PFA) is the preferred screening modality to test for acquired von Willebrand syndrome (AVWS), as seen in Heyde syndrome.
What is the cause of aortic stenosis?
In most elderly adults, aortic stenosis is caused by a build-up of calcium (a mineral found in your blood) on the valve leaflets. Over time, this causes the leaflets to become stiff, reducing their ability to fully open and close.
Can aortic stenosis cause digestive problems?
Management of Heyde syndrome is challenging, with treatment options that include medical therapy, endoscopic interventions, colonic surgery and valve replacement. If valve replacement is performed, antiplatelet and anticoagulant agents should be used with caution, to minimize the risk of rebleeding.
Can aortic stenosis cause blood clots?
Aortic-valve stenosis can be complicated by bleeding, particularly that due to gastrointestinal angiodysplasia (Heyde’s syndrome). This hemorrhagic syndrome is associated with acquired type 2A von Willebrand syndrome, which is characterized by the loss of the largest multimers of von Willebrand factor
Why does angiodysplasia cause aortic stenosis?
Management of Heyde syndrome is challenging, with treatment options that include medical therapy, endoscopic interventions, colonic surgery and valve replacement. If valve replacement is performed, antiplatelet and anticoagulant agents should be used with caution, to minimize the risk of rebleeding.
Can aortic valve stenosis cause bleeding?
High shear rates across the stenotic valve lead to increased consumption of high-molecular-weight multimers of von Willebrand factor (vWF). The subsequent functional deficit of vWF predisposes to bleeding. In a recent study, 21% of patients with severe aortic stenosis experienced skin or mucosal bleeding.
What are the end stages of aortic stenosis?
If left untreated, severe aortic stenosis can lead to heart failure. Intense fatigue, shortness of breath, and swelling of your ankles and feet are all signs of this. It can also lead to heart rhythm problems (arrhythmias) and even sudden cardiac death.
What are the complications of aortic stenosis?
Aortic valve stenosis can cause complications, including:
- Heart failure.
- Stroke.
- Blood clots.
- Bleeding.
- Heart rhythm problems (arrhythmias)
- Infections that affect the heart, such as endocarditis.
- Death.
26-Feb-2021
What organs are affected by aortic stenosis?
If you have aortic valve stenosis, more commonly known as aortic stenosis, the valve does not open fully. This abnormal narrowing of the valve (stenosis) makes it harder for blood to flow from the heart to the body and can weaken the heart. Blood can then back up into your lungs.
How does aortic stenosis affect blood flow?
Aortic valve stenosis causes a thickening and narrowing of the valve between the heart’s main pumping chamber (left ventricle) and the body’s main artery (aorta). The narrowing creates a smaller opening for blood to pass through, reducing or blocking blood flow from the heart to the rest of the body
How does aortic stenosis affect coronary arteries?
In patients with aortic valve stenosis, LV hypertrophy progression is associated with an increase in left coronary dimensions, while right coronary artery dimensions remain unchanged. Coronary artery size increase seems to be insufficient when LV hypertrophy severity increases.
What happens when stenosis occurs in the coronary arteries?
A buildup of plaque can narrow these arteries, decreasing blood flow to your heart. Eventually, the reduced blood flow may cause chest pain (angina), shortness of breath, or other coronary artery disease signs and symptoms. A complete blockage can cause a heart attack.
How does aortic pressure affect coronary blood flow?
Normal Cardiac Physiology and Ventricular Function A sudden change in aortic pressure is met by a rapid adjustment of coronary vascular resistance so that blood flow remains constant. This autoregulatory phenomenon protects the myocardium from inadequate blood flow owing to a decline in coronary perfusion pressure.