Makindo Medical Notes

Heyde Syndrome is a rare medical condition characterized by the association of aortic valve stenosis (narrowing of the aortic valve) and gastrointestinal (GI) bleeding due to angiodysplasia. This syndrome is particularly significant because the bleeding tends to be refractory to typical treatments until the aortic valve stenosis is addressed.

Pathophysiology

• Aortic Valve Stenosis :
  • A narrowing of the aortic valve opening, which restricts blood flow from the left ventricle to the aorta and the rest of the body.
  • Leads to increased shear stress across the valve, which is thought to contribute to the formation of GI angiodysplasia.
• Gastrointestinal Angiodysplasia :
  • Abnormal blood vessels in the GI tract, often in the colon, which can become fragile and prone to bleeding.
  • In Heyde Syndrome, these lesions are more likely to bleed due to the mechanical stresses and changes in blood flow associated with aortic stenosis.
• Acquired von Willebrand Syndrome :
  • Occurs due to the high shear stress in the narrowed aortic valve, which leads to degradation of von Willebrand factor (vWF), a protein crucial for blood clotting.
  • Results in increased bleeding tendencies, particularly from areas like angiodysplastic lesions.

Clinical Presentation

• Symptoms of Aortic Stenosis :
  • Chest pain (angina)
  • Shortness of breath
  • Syncope (fainting)
  • Heart failure symptoms, such as fatigue and oedema
• Symptoms of GI Bleeding :
  • Melaena (black, tarry stools)
  • Hematochezia (bright red blood per rectum)
  • Iron deficiency anaemia
  • Fatigue and pallor due to chronic blood loss

Diagnosis

• Clinical Evaluation :
  • History and physical examination to identify symptoms of aortic stenosis and GI bleeding.
• Echocardiography :
  • Used to diagnose and assess the severity of aortic stenosis.
• Endoscopy :
  • Upper and lower GI endoscopy to identify sources of bleeding, such as angiodysplasia.
• Laboratory Tests :
  • Complete blood count (FBC) to check for anaemia.
  • Coagulation studies, including tests for von Willebrand factor.

Treatment

• Medical Management :
  • Initial management of GI bleeding with endoscopic interventions, blood transfusions, and iron supplementation.
• Surgical Intervention :
  • Aortic valve replacement (surgical or transcatheter) is often necessary to relieve aortic stenosis and reduce GI bleeding.
  • Successful valve replacement can lead to resolution of GI bleeding and improvement in von Willebrand factor levels.

Prognosis

• The prognosis of Heyde Syndrome improves significantly with appropriate treatment of aortic stenosis.
• Untreated, patients may suffer from recurrent GI bleeding and complications related to severe aortic stenosis, such as heart failure.

Summary

Heyde Syndrome is a condition characterized by the association of aortic valve stenosis and gastrointestinal bleeding due to angiodysplasia. The pathophysiology involves increased shear stress causing degradation of von Willebrand factor and subsequent bleeding. Diagnosis involves clinical evaluation, echocardiography, endoscopy, and laboratory tests. Treatment focuses on managing GI bleeding and addressing aortic stenosis, often through valve replacement surgery, which can lead to significant improvement in symptoms and prognosis.