Heyde's Syndrome

HPI: The patient is a 69 yo Male with suttering chest pain for the last 8 or 9 days who woke up initially with severe stabbing 10/10 substernal chest pain with concurrent shortness of breath.  He did not seek immediate medical attention, but continued to have recurrent episodes of chest pain over the course of the next week which were both exertional and nonexertional.  Throughout this same time period he reports fatigue and SOB for which he sought evaluation at his PCP, who referred him to the emergency department.
 
Exam:  significant for a systolic ejection murmur 3/6 in intensity heard best over the left sternal border and radiating to the axilla.  Radiation is also noted to the carotids bilaterally.  The second heart sound is only mildly decreased. Hemoccult positive.
 
Labs: Notable for normocytic anemia with Hgb 7.5.  Initial troponin was 0.80 and peaked at 1.14.
 
EKG:

Interpretation: Normal Sinus rhythm.   Rate 89.  Normal axis.  Normal PR, QRS, and QT intervals.  T wave inversions noted in II, III, and aVF.  Poor R wave progression and cannot rule out remote anteroseptal MI evidenced by Q waves in V1,V2, V3.
 
Cath: The patient was subsequently cath’d and found to have 100% mid-distal RCA  occlusion which is consistent with inferior ischemia previously noted on EKG. LAD ostial 50% and mid 50% stenosis.
 
Echo: significant for mild aortic stenosis with mean gradient 22 mmHg.
 
Conclusion: Following catheterization, no stenting was performed and plans for follow-up with GI for colonoscopy to determine source of occult bleed. Cath did confirm a remote RCA infarct presumed to be exacerbated by the patient’s anemia.  Aortic stenosis was not considered severe enough to generate Heyde’s syndrome (mean gradient of <40mmHg)
 
Teaching Points:

Heyde's Syndrome

• gastrointestinal bleeding from angiodysplasia in the GI tract in the setting of aortic stenosis
• thought to be secondary to turbulent flow which disrupts von willibrand factor, resulting in spontaneous bleeding from angiodysplasia
• Aortic stenosis must be severe in order to generate enough turbulence to case VW dysfunction

 
Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F, Bauters A, Decoene C, Goudemand J, Prat A, Jude B.  Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med. 2003;349(4):343.
 
Blog by Dr. Jaron Raper