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{at}prairieheart.com
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 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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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 these pulmonary AVMs
may remain asymptomatic and a neurological catastrophe may be
the first manifestation.
1 Cerebral cortical infarctions attributable
to paradoxical thromboembolism are seen in 14% of such patients
with single AVM and is higher with multiple AVMs.
2 It is recommended
that hemorrhagic telangiectasia patients and their family members
undergo periodic screening contrast echocardiography and chest
radiograph, followed by chest computed tomography for AVMs.
3 All patients with AVMs should receive prophylactic antibiotics
before dental and other surgical procedures to minimize risk
of cerebral embolic abscesses and those with symptomatic large
AVMs should be considered for treatment with transcatheter coil
embolization.
3,4
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Acknowledgments
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None.
Disclosures
Dr R. Kolluri, Speaker for GSK, Sanofi-Aventis and The Medicines Company. None for Dr N.K. Singh.
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References
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1. Gossage JR, and Kanj GH. Pulmonary arteriovenous malformations a state of the art review.
Am J Respir Crit Care Med. 1998; 158: 643–661.
[Free Full Text]2. Moussouttas M, Fayad P, Rosenblatt M, Hashimoto M, Pollak J, Henderson K, Ma TY, White RI. Pulmonary arteriovenous malformations: cerebral ischemia and neurologic manifestations. Neurology. 2000; 55: 959–964.[Abstract/Free Full Text]
3. Cottin V, Plauchu H, Bayle JY, Barthelet M, Revel D, Cordier JF. Pulmonary arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia. Am J Respir Crit Care Med. 2004; 169: 994–1000.[Abstract/Free Full Text]
4. Lee DW, White RI Jr, Egglin TK, Pollak JS, Fayad PB, Wirth JA, Rosenblatt MM, Dickey KW, Burdge CM. Embolotherapy of large pulmonary arteriovenous malformations: long-term results. Ann Thorac Surg. 1997; 64: 930–939.[Abstract/Free Full Text]
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