Coronary Artery Aneurysms in
Hyper IgE Recurrent Infection
Syndrome
Alexandra Freeman MD
NIAID, NIH
November 5, 2006
CASE
• 43 year old man with HIES presented to NIH
after suffering a myocardial infarction
– Found in cardiac arrest next to his car,
resuscitated, and stabilized at a local hospital
– MI risk factors: smoker, mild hyperlipidemia,
HTN
• EKG consistent with anteroseptal MI in the
LAD distribution
CASE
• Past Medical history:
– Recurrent pneumonias: including Staph aureus, Strep
pneumoniae, H. influenza, Pseudomonas.
– LUL lobectomy secondary to aspergillus infection
– Eczema, boils
– Recurrent sinusitis and otitis media
– Oral candidiasis
– Scoliosis/leg length discrepancy
– Minimal trauma fractures (including left humerus x 2,
right humerus, ribs)
– T2 hyperintensities on brain MRI and left thalamic
lacunar infarct
– Wernicke’s encephalopathy
Case
• Cardiac Evaluation at NIH
– Coronary angiography revealed fusiform LAD
aneurysm and ectasia of the RCA
– CT angio/MRI
– Minimal intimal thickening, no calcifications on
intravascular ultrasound
• No history per patient or family of Kawasaki
disease
• Discharged on aspirin, clopidogrel
LAD
RCA
LAD with Filling Defect
HIES Features
• Characteristic face
• Hyperextensibility
• Retention of primary
teeth
• Scoliosis
• Osteopenia
• Minimal Trauma
Fractures
• Craniosynostosis
• Abnormalities on brain
MRI
• Degenerative joint
disease?
• Esophageal disease?
• Vasculopathy?
Immunologic
Eczema, recurrent pneumonias, boils
Non-immunologic
Aneurysms and HIES?
• Autopsy series of 6
women with HIES
• No coronary artery
aneurysms
• One patient with bilateral
berry cerebral aneurysms
• One patient with a
mycotic MCA aneurysm
Berry Aneurysm
Etiology of Coronary Artery
Aneurysms
• Kawasaki disease
• Atherosclerosis
• Congenital
• Infectious (mycotic)
• Autoimmune (SLE)
• Connective Tissue Disorders
– Ehler-Danlos syndrome Type IV
– Marfan syndrome
– Loeys-Dietz syndrome
Future Research
• Another patient identified (50 year old man)
with aneury