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ObjectiveTo examine the role of rheumatoid arthritis (RA) flare, remission and RA severity burden in cardiovascular disease (CVD).MethodsIn a population-based cohort of patients with RA without CVD (age ≥30 years; 1987 American College of Rheumatology criteria met in 1988–2007), we performed medical record review at each clinical visit to estimate flare/remission status. The previously validated RA medical Records-Based Index of Severity (RARBIS) and Claims-Based Index of RA Severity (CIRAS) were applied. Age- and sex-matched non-RA subjects without CVD comprised the comparison cohort. Cox models were used to assess the association of RA activity/severity with CVD, adjusting for age, sex, calendar year of RA, CVD risk factors and antirheumatic medications.ResultsStudy included 525 patients with RA and 524 non-RA subjects. There was a significant increase in CVD risk in RA per time spent in each acute flare versus remission (HR 1.07 per 6-week flare, 95% CI 1.01 to 1.15). The CVD risk for patients with RA in remission was similar to the non-RA subjects (HR 0.90, 95% CI 0.51 to 1.59). Increased cumulative moving average of daily RARBIS (HR 1.16, 95% CI 1.03 to 1.30) and CIRAS (HR 1.38, 95% CI 1.12 to 1.70) was associated with CVD. CVD risk was higher in patients with RA who spent more time in medium (HR 1.08, 95% CI 0.98 to 1.20) and high CIRAS tertiles (HR 1.18, 95% CI 1.06 to 1.31) versus lower tertile.ConclusionsOur findings show substantial detrimental role of exposure to RA flare and cumulative burden of RA disease severity in CVD risk in RA, suggesting important cardiovascular benefits associated with tight inflammation control and improved flare management in patients with RA.

ObjectiveTo describe the diagnostic features of intracranial dural arteriovenous fistulae (DAVF) presenting with cervical cord or brainstem swelling.MethodsRetrospective case note and neuroimaging review of patients with angiographically confirmed DAVF diagnosed during January 2015–June 2020 at a tertiary neuroscience centre (Walton Centre NHS Foundation Trust, Liverpool, UK).ResultsSix intracranial DAVF causing cervical cord or brainstem oedema (all males aged 60–69 years) and 27 spinal DAVF (88% thoracolumbar) were detected over a 5.5-year period. Significantly more patients with intracranial DAVF received steroids for presumed inflammatory myelitis than those with spinal DAVF (5/6 vs 1/27, p = 0.0001, Fisher’s exact test). Several factors misled the treating clinicians: atypical rostral location of cord oedema (6/6); acute clinical deterioration (4/6); absence (3/6) or failure to recognise (3/6) subtle dilated perimedullary veins on MRI; intramedullary gadolinium enhancement (2/6); and elevated CSF protein (4/5). Acute deterioration followed steroid treatment in 4/5 patients. The following features may suggest DAVF rather than myelitis: older male patients (6/6), symptomatic progression over 4 or more weeks (6/6) and acellular CSF (5/5).ConclusionIntracranial DAVF are uncommon but often misdiagnosed and treated as myelitis, which can cause life-threatening deterioration. Neurologists must recognise suggestive features and consider angiography, especially in older male patients. Dilated perimedullary veins are an important clue to underlying DAVF, but may be invisible or easily missed on routine MRI sequences.

Background Subarachnoid hemorrhage in the basal cisterns is usually identified on an unenhanced computed tomography scan of the head in patients presenting acutely with a characteristic sudden onset headache. Case presentation Using imaging examples from our tertiary neurosciences center, we present six cases demonstrating a variety of causes for subarachnoid hemorrhage in the basal cisterns, ranging from benign to sinister causes. These include a venous perimesencephalic hemorrhage (35 years, female), pontine perforator aneurysm (54 years, male), vertebral artery dissection (69 years, male), cervical dural arteriovenous fistula (65 years, male), posterior fossa arteriovenous malformation (45 years, male), and vertebral artery aneurysm (78 years, female). Ethnically, all these patients were white. Specific imaging features are described and demonstrated. Conclusion A balance between avoiding excessive investigation and overlooking what may be a mimic of a venous perimesencephalic hemorrhage is important. To refine and establish more definitive indications on when to perform computed tomography angiogram, digital subtraction angiography, delayed angiography and magnetic resonance imaging in this context requires future research to focus on large-scale prospective multicenter studies with robust data.

Anomalous aortic origin of a coronary artery (AAOCA) comprises a set of rare congenital abnormalities in the origin or path of the coronary arteries with highly variable clinical implications. This is a pilot feasibility study where we investigated the influence of the anomalous coronary artery inlet architecture on coronary perfusion using coronary blood flow computational simulations to help predict the risk for coronary ischemia in patients with anomalous aortic origin of the right coronary artery (AAORCA) with these types of anomalous coronary artery inlet architectures. We developed a protocol for generating 3D models of patient coronary artery anatomies from an IRB-approved dataset of cardiac CT images of patients with AAORCA at our institution. Coronary blood flow simulations and analysis were performed. Instantaneous flow reserve (iFR), a parameter used clinically in coronary CT analysis to determine risk for ischemia, was compared between models as a measure of ischemia. Comparing the median iFR of the coronary outlets between the four inlet variants and baseline architecture showed important differences. We observed a possible association between the proportion of the semi-minor axis and the semi-major axis of the elliptical AAORCA inlet and iFR. These observations suggest that the elliptical axis quotient may be a significant risk factor for evaluation of AAORCA severity.

BackgroundCervico-medullary spinal cord lesions are usually inflammatory (or rarely neoplastic). Dural arteriovenous fistula (DAVF) is considered very unlikely in the cervical cord, particularly if the history is of an acute myelopathy.MethodCase note review of five patients with skull base DAVF.ResultsAll were men aged 60–69 years. Four were symptomatic for a month or less, followed by acute deterioration over hours-7days, mimicking myelitis. MRIs showed extensive T2-hyperintensity and swelling in the cervico-medullary region. Worsening after corticosteroids occurred in 3/4 patients and one needed ventilation. Conspicuous and unambiguous cord-surface vessels were present in only one patient. Clinical suspicion prompted digital subtraction angiography (DSA) in the other four patients. Median time from initial MRI to diagnostic DSA and embolization was 28 days (7–91 days). All patients survived with good clinical recovery.Systematic retrospective review of MRIs showed lack of gadolinium enhancement in 4/5. CSF examination (n=4) showed normal leucocyte count in all cases and elevated protein concentration in 3/4 (0.6–1.2 g/L).ConclusionSkull base DAVF can mimic acute cervical myelitis. Steroids may prompt life-threatening clinical deterioration mistaken for ascending myelitis. A low threshold for angiography in the context of non-enhancing lesions and acellular CSF will facilitate earlier diagnosis and improve outcomes.

We present our initial patient experience with an innovative temporary bridging device, the Comaneci (Rapid Medical, Israel), to assist in the coiling of cerebral aneurysms. The Comaneci device confers the same benefits as balloon remodeling but without the risks of parent artery occlusion. This alleviates time pressure on the clinician, and could reduce the risk of parent artery thrombosis. Three patients were treated with the Comaneci device. Two patients had acute ruptured posterior communicating aneurysms and one patient was treated electively for a carotico-ophthalmic aneurysm. Excellent occlusion of all three aneurysms was obtained. One patient developed a distal middle cerebral artery clot, that was treated with intravenous aspirin, with minor neurological consequences. These early results show that the Comaneci device can be used to achieve good cerebral aneurysm occlusion. Vessel patency is maintained throughout the procedure with potential advantages over conventional balloon assisted coiling.

Ruptured arteriovenous malformation (AVM) is a frequent cause of intracranial hemorrhage. The presence of associated aneurysms, especially intranidal aneurysms, is considered to increase the risk of re-hemorrhage. We present two cases where an intranidal aneurysm was demonstrated on four-dimensional CT angiography (time-resolved CT angiography) (4D-CTA). These features were confirmed by digital subtraction angiography (catheter arterial angiogram). This is the first report of an intranidal aneurysm demonstrated by 4D-CTA. 4D-CTA can offer a comprehensive evaluation of the angioarchitecture and flow dynamics of an AVM for appropriate classification and management.

Purpose Intracranial arteriovenous malformations (AVMs) treated at our institution with modern techniques of endovascular intervention were analysed for the rate of complete occlusion, associated morbidity, and mortality. To our knowledge, this is the first series from the UK evaluating the effectiveness of endovascular embolisation as a primary treatment for selected cases. Methods All newly referred AVMs between January 2017 and June 2022 were reviewed and those treated with primary endovascular intervention were identified. Details of the endovascular procedures were retrospectively reviewed. Results In 5½ years, 41.1% of AVMs referred to our institution have been triaged for primary endovascular intervention. Sixty-eight AVMs were embolised and followed-up: 44 ruptured and 24 unruptured. Spetzler-Martin grading varied from I to III, and a single AVM was grade IV. The approach was arterial in 73.5%, solely venous in 7.4%, and combined in 19.1%. The mean follow-up was 18 months for imaging and 26 months for clinical assessment. Complete obliteration was achieved in 95.6%. Ruptured AVM cohort: The rate of functional deterioration was 13.6%. Unruptured AVM cohort: The rate of functional deterioration secondary to complications from embolisation was 4.2%. Conclusions Endovascular embolisation may be a favourable option for primary AVM treatment in carefully selected patients. However, selection criteria need to be better delineated for more specialists to consider this as a primary therapy.

We present an infant with incidentally found retro-aortic left brachiocephalic vein draining directly into azygous vein. This rare vascular variant is asymptomatic but does have clinical implications with surgical and interventional procedures.

Purpose Despite the potentially devastating and permanently disabling effects of paediatric arteriovenous malformations (pAVMs), there is a paucity of studies reporting long-term quality-of-life (QoL) outcomes in AVM patients. We aim to evaluate the management strategies for paediatric intracranial pAVMs in the UK and long-term QoL outcomes using a validated paediatric quality-of-life outcome measure. Methods In this single-centre case-series, we retrospectively reviewed a prospectively maintained database of all paediatric patients (i.e. 0–18 years old) with intracranial AVMs, who were managed at Alder Hey Children’s Hospital from July 2007 to December 2021. We also collected the PedsQL 4.0 score for these patients as a measure of QoL. Results Fifty-two AVMs were included in our analysis. Forty (80%) were ruptured, 8 (16%) required emergency intervention, 17 (35%) required elective surgery, 15 (30%) underwent endovascular embolisation, and 15 (30%) patients underwent stereotactic radiosurgery. There was an 88% overall obliteration rate. Two (4%) pAVMs rebled, and there were no mortalities. Overall, the mean time from diagnosis to definitive treatment was 144 days (median 119; range 0–586). QoL outcomes were collected for 26 (51%) patients. Ruptured pAVM presentation was associated with worse QoL ( p  = 0.0008). Location impacted psychosocial scores significantly (71.4, 56.9, and 46.6 for right supratentorial, left supratentorial, and infratentorial, respectively; p  = 0.04). Conclusion This study shows a staged multi-modality treatment approach to pAVMs is safe and effective, with superior obliteration rates with surgery alone. QoL scores are impacted by AVM presentation and location regardless of treatment modality.