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E-292 Aviator: efficient and comprehensive advanced magnetic resonance imaging of hereditary hemorrhagic telangiectasia vascular malformations

Year: 2024
Type: conference-abstract
Abstract: Background and Rationale Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for vascular malformations in the brain, lungs, and liver. Current guidelines for AVM screening in HHT include contrast-enhanced brain MRI/MRA, chest CT or echocardiography with agitated saline, and liver ultrasound with Doppler. Given the complexity of vascular malformations and their clinical implications in patients with HHT, improvements are needed both in the efficiency of multi-organ screening and the sensitivity of organ-specific focused imaging examinations to key anatomic and physiologic features. We propose to improve HHT care through development of advanced MR techniques. Goals Goal 1. Develop an efficient multistation screening 3T MRI/MRA examination for new patients with possible or definite HHT. We will combine ferumoxytol-enhanced 4D flow and structural imaging from the head to liver. Results will be compared to standard clinical imaging in the same patients. Goal 2. Develop 7T brain MRI/MRA techniques to evaluate the detailed angioarchitecture of brain AVMs. A 7T exam including high-resolution 7T TOF noncontrast MRA, susceptibility weighted imaging (SWI), ferumoxytol-enhanced 4D flow MRA, and post-contrast structural images will be compared to current clinical state-of-the-art 3T MRI/MRA and DSA. Metrics for success: 7T noncontrast MRA will detect more brain AVMs ≥2 mm than 3T contrast-enhanced MRI, 7T SWI will detect more microhemorrhages than 3T SWI, and the combination of 7T images will detect venous outflow stenoses and nidus aneurysms with ≥80% sensitivity and specificity compared to DSA. Methods Volunteers with HHT were recruited and provided informed consent per an approved IRB protocol. They received intravenous fermoxytol infusion either in the nursing station prior to 3T head- through-abdomen screening scan or in the scanner during the 7T head-only high-resolution scan. Results The first 13 patients enrolled have demonstrated that AVMs as small as 2 mm in the peripheral lung and brain can be identified on the head-through-abdomen 3T screening study. The hepatic artery iseasily measured on this screening study as well. For the dose-ranging 7T study, a relatively low dose of ferumoxytol yields the greatest structural detail for the small vessels in brain AVM nidus (figure 1). Conclusion Early results indicate ferumoxytol enhanced MRI/MRA can provide a multi-station, multi-organ vascular screening examination that compares favorably to the multiple current clinical imaging studies that HHT patients undergo. High-resolution images at 7T may be able to discern imaging features currently only consistently identifiable on invasive digital subtraction angiography. Disclosures S. Hetts: 1; C; Department of Defense, National Institutes of Health, Siemens, Route 92. 2; C; Stryker, Imperative. 4; C; Filtro, ThrombX. 5; C; UCSF. 6; C; Penumbra. M. Ohliger: None. Y. Lee: None. D. Langston: None. T. Lomax Truong: None. A. Gill: None. Y. Park: None. J. Liu: None. J. Lupo: None. M. Conrad: None. D. Saloner: None.
Language: English
Subfield: Genetics
Field: Medicine
Open Access status: bronze
OpenAlex ID: W4400892459
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