Circulation: Cardiovascular Imaging. 2008;1:e11-e12
doi: 10.1161/CIRCIMAGING.108.788570
Paradoxical Systemic Embolization in Hereditary Hemorrhagic Telangiectasia
Nishith K. Singh, MD
and
Raghu Kolluri, MD
From the Department of Internal Medicine (N.K.S., R.K.), Division of Cardiology, Southern Illinois University School of Medicine, Springfield, IL; Prairie Vascular Institute (R.K.), St John’s Hospital, Prairie Vascular Institute, Springfield, IL; and Prairie Education & Research Cooperative (R.K.), Springfield, IL.
Correspondence to Raghu Kolluri, MD, 401 East Carpenter St, Springfield, IL 62702. E-mail rkolluri@prairieheart.com
An extract of the first 250 words of the full text is provided, because this article has no abstract.
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A 53-year-old man presented with sudden onset left-sided weakness. His medical history included hereditary hemorrhagic telangiectasia with recurrent epistaxis. On examination, he was hypoxemic at rest and had left-sided flaccid hemiparesis. He was noted to have multiple lower lip telangiectasias (Figure 1). Computed tomography of chest revealed bilateral segmental and subsegmental pulmonary emboli (Figure 2, arrows) and a left lower lobe arteriovenous malformation (AVM) (Figure 2, arrowhead). MRI of brain and computed tomography of abdomen showed bilateral cerebral (Figure 3), splenic, and renal segmental infarcts. A venous duplex revealed a calf vein deep venous thrombosis that was thought to be secondary to a recent preceding knee surgery. We concluded that the patient had pulmonary embolization followed by paradoxical systemic embolization through the AVM. Patient eventually underwent transcatheter coil embolization of the malformation (pulmonary angiography, Figure 4a and b, arrows).
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Figure 1. Lower lip of the patient showing multiple telangiectasias (arrows).
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Figure 2. Contrast enhanced computed tomography of chest showing thrombi (filling defects, arrows) in segmental right pulmonary arteries and tortuous draining vessels (arrowhead) from the left AVM.
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Figure 3. T2-weighted MRI of brain showing multiple bilateral infarcts.
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Figure 4. (a) Pulmonary angiogram showing left AVM. (b) Pulmonary angiogram after catheter embolization of left AVM showing coil in situ.
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Pulmonary AVMs are aneurysmal, direct, low-pressure, artery-to-vein
connections that result in a right-to-left shunt and are seen
in 15% to 35% of patients with hemorrhagic telangiectasia.
1 As illustrated in the vignette, most of
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