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Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder leading to vascular malformations in different organ systems. Approximately 10% of patients with HHT have brain vascular malformations (BVMs). Due to the negative health consequences related to BVMs, screening with MRI is recommended. There are no health jurisdictional standards for medical imaging protocols in North America or elsewhere. The objective of this project is to adopt a provincial standardized operating protocol (SOP) to improve diagnostic accuracy and reduce inappropriate imaging in patients with and without HHT in Alberta.
Methods:
Multiple fora were held among the five Alberta Health Services zones with stakeholders from urban, suburban and rural radiology groups, neurology, pulmonology and hematology. The consensus process took five years to complete between 2015 and 2020. The content of the fora was approved by all participants.
Results:
The SOP was implemented in February 2020 and defines that screening for BVMs must include standard unenhanced brain MRI (sagittal T1, axial fluid-attenuated inversion recovery and axial T2) with susceptibility-weighted imaging (SWI), a 3T or 1.5T magnet strength and minimum imaging standards to include 3 mm contiguous slice thickness.
Discussion:
Incorporation of SWI allowed for the elimination of MR contrast to improve access to the local performance of studies within the province, facilitating virtual care.
Conclusion:
A provincial SOP for BVM screening in patients with suspected or confirmed HHT was successfully implemented in Alberta. Gadolinium was avoided, as it was felt to be unnecessary for screening purposes and might complicate imaging at more remote sites.
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