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MR Angiography for Detection of Pulmonary Arteriovenous Malformations in Patients with Hereditary Hemorrhagic Telangiectasia

Guenther Schneider1, Michael Uder2, Michael Koehler3, Miles A. Kirchin4, Alexander Massmann1, Arno Buecker1 and Urban Geisthoff5

1 Department of Diagnostic and Interventional Radiology, University Hospital of Saarland, 66421 Homburg/Saar, Germany.
2 Department for Radiology, Friedrich-Alexander-University, Erlangen-Nuremberg, Germany.
3 Department for Clinical Radiology, Westfaelische Wilhelms-University, Muenster, Germany.
4 Worldwide Medical & Regulatory Affairs, Bracco Imaging SpA, Milan, Italy.
5 Department of Otorhinolaryngology, University Hospital of Saarland, Homburg/Saar, Germany.


Figure 1
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  		Fig. 1 —Flowchart shows management of patients with proven hereditary hemorrhagic telangiectasia (HTT) (criteria described by Shovlin et al. [45]). CE-MRA = contrast-enhanced MR angiography, PAVM = pulmonary arteriovenous malformation, and first-degree relatives. PA = pulmonary angiography.

 

Figure 2
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  		Fig. 2A —56-year-old man with proven hemorrhagic telangiectasia (HTT) according to criteria described by Shovlin et al [45]. Maximum-intensity-projection reconstruction shows two pulmonary arteriovenous malformations (AVMs) (arrows) in right lung. Larger AVM shows one feeding artery, one draining vein, and septate aneurysm sac. Smaller AVM similarly has one feeding artery and one draining vein (no septations).

 

Figure 3
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  		Fig. 2B —56-year-old man with proven hemorrhagic telangiectasia (HTT) according to criteria described by Shovlin et al [45]. Volume rendering of same data set accurately depicts 3D orientation of feeding arteries to both pulmonary AVMs.

 

Figure 4
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  		Fig. 2C —56-year-old man with proven hemorrhagic telangiectasia (HTT) according to criteria described by Shovlin et al [45]. Selective pulmonary angiography of right lung (C) reveals both pulmonary AVMs (arrows, C), although detailed anatomy is seen only on superselective pulmonary angiography of feeding arteries (D and E). Note that in E larger pulmonary AVM (arrow) is already embolized.

 

Figure 5
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  		Fig. 2D —56-year-old man with proven hemorrhagic telangiectasia (HTT) according to criteria described by Shovlin et al [45]. Selective pulmonary angiography of right lung (C) reveals both pulmonary AVMs (arrows, C), although detailed anatomy is seen only on superselective pulmonary angiography of feeding arteries (D and E). Note that in E larger pulmonary AVM (arrow) is already embolized.

 

Figure 6
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  		Fig. 2E —56-year-old man with proven hemorrhagic telangiectasia (HTT) according to criteria described by Shovlin et al [45]. Selective pulmonary angiography of right lung (C) reveals both pulmonary AVMs (arrows, C), although detailed anatomy is seen only on superselective pulmonary angiography of feeding arteries (D and E). Note that in E larger pulmonary AVM (arrow) is already embolized.

 

Figure 7
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  		Fig. 3A —30-year-old woman with three pulmonary arteriovenous malformations (AVMs). Pulmonary AVMs are detected on maximum-intensity-projection reconstruction.

 

Figure 8
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  		Fig. 3B —30-year-old woman with three pulmonary arteriovenous malformations (AVMs). Multiplanar reconstructions reveal three feeding arteries (arrows). This additional information was considered of value for improved embolization planning.

 

Figure 9
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  		Fig. 3C —30-year-old woman with three pulmonary arteriovenous malformations (AVMs). Multiplanar reconstructions reveal three feeding arteries (arrows). This additional information was considered of value for improved embolization planning.

 

Figure 10
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  		Fig. 4A —56-year-old man with multiple pulmonary arteriovenous malformations (AVMs). Initial screening contrast-enhanced MR angiography (CE-MRA) reveals several small pulmonary AVMs in both lungs.

 

Figure 11
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  		Fig. 4B —56-year-old man with multiple pulmonary arteriovenous malformations (AVMs). Follow-up CE-MRA 6 years later reveals several new pulmonary AVMs (arrows) not seen on first screening CE-MRA and increased lesion size for several preexisting pulmonary AVMs (arrowheads).

 

Figure 12
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  		Fig. 5A —26-year-old woman (daughter of patient in Figs. 2A, 2B, 2C, 2D, and 2E). Maximum-intensity-projection contrast-enhanced MR angiography (CE-MRA) image shows a complex pulmonary arteriovenous malformation (AVM) in the right lung.

 

Figure 13
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  		Fig. 5B —26-year-old woman (daughter of patient in Figs. 2A, 2B, 2C, 2D, and 2E). Anatomy is much better depicted on volume-rendered CE-MRA image, which reveals two peripheral feeding arteries (arrows) and one central feeding artery that divides into two further feeding arteries (arrowheads) just before pulmonary AVM. In addition, two draining veins (asterisks) are nicely depicted.

 

Figure 14
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  		Fig. 5C —26-year-old woman (daughter of patient in Figs. 2A, 2B, 2C, 2D, and 2E). Superselective pulmonary angiography of central feeding artery confirms findings in B.

 

Figure 15
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  		Fig. 5D —26-year-old woman (daughter of patient in Figs. 2A, 2B, 2C, 2D, and 2E). Embolization of central feeding arteries leads to reflux of contrast medium into two peripheral feeding arteries.

 

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