Recommendations for defining giant cell arteritis fast-track clinics. English version.

A German expert committee recommends defining fast-track clinics (FTC) for the acute diagnosis of giant cell arteritis (GCA) as follows: easy and prompt reachability at least on weekdays, scheduling appointments ideally within 24 h, examination by a specialist with GCA expertise, ≥ 2 experts per FTC, ≥ 50 patients with suspected GCA per year, sonologists with ≥ 300 (≥ 50) temporal and axillary artery examinations, adherence to standard operating procedures, availability of an ≥ 18 (≥ 15) MHz and a lower frequency linear ultrasound probe, and collaboration with partners for neurology and ophthalmology consultations, magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT, possibly CT), and for temporal artery biopsy.

[Recommendations for defining giant cell arteritis fast-track clinics]. / Empfehlungen zur Definition von Riesenzellarteriitis-Fast-Track-Kliniken.

An expert committee recommends defining fast-track clinics (FTC) for the acute diagnostics of giant cell arteritis (GCA) as follows: low-threshold, easy and prompt reachability at least on weekdays, scheduling appointments ideally within 24 h, examination by a specialist with GCA expertise, ≥ 2 experts per FTC, ≥ 50 patients with suspected GCA per year, sonologists with ≥ 300 (≥ 50) temporal and axillary artery examinations, adherence to standard operating procedures, availability of an ≥ 18 (≥ 15) MHz and a lower frequency linear ultrasound probe and collaboration with partners for fast performance of neurological and ophthalmological examinations, magnetic resonance imaging (MRI), positron emission tomography-computed tomography (PET-CT, possibly CT) and for temporal artery biopsy.

Intravascular lithotripsy-assisted percutaneous deep vein arterialization for no-option chronic limb-threatening patients and heavily calcified tibial occlusive disease.

PURPOSE: To report the first chronic limb-threatening ischemia (CLTI) patients who underwent an intravascular lithotripsy (IVL)-assisted percutaneous deep vein arterialization (pDVA). CASE REPORT 1: An 81-year-old patient presented with CLTI and a heavily calcified lesion of the popliteal artery and tibioperoneal trunk (TPT), with a distal tibial and foot arch occlusion. The patient underwent IVL and drug-coated balloon angioplasty for the distal popliteal artery and of the TPT to improve the inflow prior to pDVA. The wound situation remained stable without secondary procedure until the patient`s deaths due to complications of urosepsis 3 months later. CASE REPORT 2: A 64-year-old patient with rest pain of the left limb with a single-vessel tibial run-off (peroneal artery) and occluded pedal arch was treated with 3.5 mm IVL followed by a successful pDVA as mentioned above. IVL performed in the proximal posterior tibial artery to optimize the inflow to the circuit and change the compliance of the crossing point from the arterial to the vein system. The patient underwent repeat angioplasty of the plantar vein arch 5 months after the index procedure and thereafter remained asymptomatic during 2 years of follow-up. CONCLUSION: The combined use of IVL and pDVA could improve the patency of the reconstruction with clinical benefits in no-option CTLI patients.

Early REperfusion therapy with intravenous alteplase for recovery of VISION in acute central retinal artery occlusion (REVISION): Study protocol of a phase III trial.

RATIONALE: Meta-analyses of case series of non-arteritic central retinal artery occlusion (CRAO) indicate beneficial effects of intravenous thrombolysis when initiated early after symptom onset. Randomized data are lacking to address this question. AIMS: The REperfusion therapy with intravenous alteplase for recovery of VISION in acute central retinal artery occlusion (REVISION) investigates intravenous alteplase within 4.5 h of monocular vision loss due to acute CRAO. METHODS: This study is the randomized (1:1), double-blind, placebo-controlled, multicenter adaptive phase III trial. STUDY OUTCOMES: Primary outcome is functional recovery to normal or mildly impaired vision in the affected eye defined as best-corrected visual acuity of the Logarithm of the Minimum Angle of Resolution of 0.5 or less at 30 days (intention-to-treat analysis). Secondary efficacy outcomes include modified Rankin Score at 90 days and quality of life. Safety outcomes include symptomatic intracranial hemorrhage, major bleeding (International Society on Thrombosis and Haemostasis definition) and mortality. Exploratory analyses of optical coherence tomography/angiography, ultrasound and magnetic resonance imaging (MRI) biomarkers will be conducted. SAMPLE SIZE: Using an adaptive design with interim analysis at 120 patients, up to 422 participants (211 per arm) would be needed for 80% power (one-sided alpha = 0.025) to detect a difference of 15%, assuming functional recovery rates of 10% in the placebo arm and 25% in the alteplase arm. DISCUSSION: By enrolling patients within 4.5 h of CRAO onset, REVISION uses insights from meta-analyses of CRAO case series and randomized thrombolysis trials in acute ischemic stroke. Increased rates of early reperfusion and good neurological outcomes in stroke may translate to CRAO with its similar pathophysiology. TRIAL REGISTRATION: ClinicalTrials.gov: NCT04965038; EU Trial Number: 2023-507388-21-00.

Patient Characteristics in the Recording Courses of Vascular Diseases (Reccord) Registry: Comparison with the Voyager Pad Endovascular Cohort.

BACKGROUND: To compare the characteristics of a "real world" population included in a prospective registry to patients enrolled in a randomized, controlled trial (RCT) after endovascular revascularization (EVR) for symptomatic peripheral artery disease (PAD). METHODS: The RECcording COurses of vasculaR Diseases (RECCORD) registry is an observational registry prospectively recruiting patients undergoing EVR for symptomatic PAD in Germany. VOYAGER PAD was an RCT which demonstrated the superiority of rivaroxaban and aspirin versus aspirin to reduce major cardiac and ischemic limb events following infrainguinal revascularization for symptomatic PAD. For this exploratory analysis, the clinical characteristics of 2.498 patients enrolled in RECCORD and of 4.293 patients from VOYAGER PAD who underwent EVR were compared. RESULTS: The rate of patients aged ≥ 75 years was considerably higher in the registry (37.7 vs. 22.5%). More patients in the registry had undergone previous EVR (50.7 vs. 38.7%) or suffered from critical limb threatening ischemia (24.3 vs. 19.5%). Registry patients were more commonly active smokers (51.8 vs. 33.6%), but less frequently suffered from diabetes mellitus (36.4 vs. 44.7%). While statins (70.5 vs. 81.7%) were less frequently used, antiproliferative catheter technologies (45.6 vs. 31.4%) and postinterventional dual antiplatelet therapy (64.5 vs. 53.6%) were more commonly applied in the registry. CONCLUSIONS: There were many similarities but some clinically meaningful differences in clinical characteristics between PAD patients who underwent EVR and were included in a nationwide registry and PAD patients from the VOYAGER PAD trial.

The Flashlight-Sign: A Novel B-Flow Based Ultrasound Finding for Detection of Intraluminal, Wall-Adherent, Floating Structures of the Abdominal Aorta and Peripheral Arteries.

This study aimed to evaluate the potential diagnostic value of a novel, sonographic, B-Flow (BFl)-based sign ("flashlight sign", FLS) for the detection of wall-adherent, floating arterial structures (WAFAS). The FLS, characterized by a fast moving, very bright, intraluminal signal, was detected in 28 patients with WAFAS. We divided this cohort into three subgroups according to the affected vascular segments: (1) peripheral arteries (n = 10); (2) native abdominal aorta (n = 8); and (3) abdominal aorta after endovascular aortic repair (EVAR; n = 10). Clinical characteristics were analyzed and BFl-findings were compared with contrast-enhanced ultrasound (CEUS) and computed tomography angiography (CTA). Seven patients (25%) suffered from arterial embolism downstream to the FLS (EVAR, n = 4; native abdominal aorta, n = 1; peripheral arteries, n = 2). WAFAS of the abdominal aorta (native or after EVAR), as indicated by the FLS, were visible by CEUS and CTA in 60% and 93.3%, respectively. Based on the largest cohort (to this point) of patients with WAFAS, we propose a clinically useful, BFl-based sonographic sign for the detection of these underrated arterial pathologies in the abdominal aorta and the peripheral arteries.

Diagnostic yield of axillary artery ultrasound in addition to temporal artery ultrasound for the diagnosis of giant cell arteritis.

OBJECTIVES: There are limited data on the additional diagnostic yield of axillary artery ultrasound (axUS) in addition to temporal artery ultrasound (tempUS) for the diagnosis of giant cell arteritis (GCA). METHODS: Retrospective study of consecutive patients with suspected GCA who underwent a standardized axUS and tempUS between 01/2015 and 03/2017. The diagnostic yield of axUS in addition to ultrasound of the temporal arteries with respect to the final clinical diagnosis was assessed, with a positive axUS defined as circumferential, hypoechogenic thickening of the far wall axillary artery intima media thickness (axIMT) ≥1.3 mm. A subgroup of patients underwent PET-CT within one week before or after the sonographic study. Separate analyses were performed regarding certain subgroups according to clinical presentation and to clinical pre-test probability for cranial GCA. RESULTS: Out of 228 patients, 92 received a final diagnosis of GCA. From the 92 patients with a final diagnosis of GCA, 50 (54.3%), 13 (14.1%) and 15 (16.3%) had a positive tempUS, positive axUS, and combined positive tempUS and axUS, respectively. The sensitivity of sonographic imaging for the final diagnosis of GCA increased from 69.6% to 84.8%, when axUS results were considered in addition to tempUS, while the specificity remained high (no false positive axUS). The diagnostic yield of axUS was highest in patients with a low clinical probability of cranial GCA and lowest in patients with symptoms of ocular ischemia. We observed a substantial rate (42.1%) of discordant results between axUS and PET-CT in a subgroup of 38 patients. CONCLUSIONS: In conclusion, axUS offers a substantial diagnostic yield in addition to tempUS in subjects with suspected GCA, mainly in those subjects with low clinical probability for cranial GCA.

Zieve syndrome presenting with lipaemia and treated by plasmapheresis.

Zieve syndrome is a rare condition which occurs in patients with severe alcohol abuse. It is typically characterised by the triad of jaundice, haemolytic anaemia and transient hyperlipidaemia. In the following report, we present the case of a man in his 30s who was admitted to our emergency department with severe epigastric pain and signs of alcohol intoxication. Blood samples showed signs of severe hyperlipidaemia and jaundice. Due to massive hyperlipidaemia, laboratory measurements of triglycerides were impaired and the blood samples had a 'yellowish' and 'creamy' texture. In order to prevent pancreatitis, plasmapheresis was performed. Subsequently, triglyceride concentration dropped and the patient was discharged a few days later in significantly improved physical condition. In the following case report, we discuss plasmapheresis as a possible treatment for patients with severe Zieve syndrome in addition to conventional symptomatic therapy.

Imaging of peripheral vascular malformations - current concepts and future perspectives.

Vascular Malformations belong to the spectrum of orphan diseases and can involve all segments of the vascular tree: arteries, capillaries, and veins, and similarly the lymphatic vasculature. The classification according to the International Society for the Study of Vascular Anomalies (ISSVA) is of major importance to guide proper treatment. Imaging plays a crucial role to classify vascular malformations according to their dominant vessel type, anatomical extension, and flow pattern. Several imaging concepts including color-coded Duplex ultrasound/contrast-enhanced ultrasound (CDUS/CEUS), 4D computed tomography angiography (CTA), magnetic resonance imaging (MRI) including dynamic contrast-enhanced MR-angiography (DCE-MRA), and conventional arterial and venous angiography are established in the current clinical routine. Besides the very heterogenous phenotypes of vascular malformations, molecular and genetic profiling has recently offered an advanced understanding of the pathogenesis and progression of these lesions. As distinct molecular subtypes may be suitable for targeted therapies, capturing certain patterns by means of molecular imaging could enhance non-invasive diagnostics of vascular malformations. This review provides an overview of subtype-specific imaging and established imaging modalities, as well as future perspectives of novel functional and molecular imaging approaches. We highlight recent pioneering imaging studies including thermography, positron emission tomography (PET), and multispectral optoacoustic tomography (MSOT), which have successfully targeted specific biomarkers of vascular malformations.

[Large vessel vasculitis: update 2021]. / Update Großgefäßvaskulitis 2021.

In recent years, clinically significant advances have been made in the management of giant cell arteritis and Takayasu arteritis. This concise review article highlights important aspects of the diagnostic workup and imaging-based treatment surveillance of the large vessel vasculitides.

Hyperechogenic intimal lesions and wall thickness of the temporal and facial arteries in elderly patients with arterial occlusions of the eye.

OBJECTIVE: To determine the association of arteriosclerosis, characterised by hyperechogenic intimal lesions (HIL), with wall thickness of the temporal and facial arteries in elderly patients with ocular arterial occlusions. METHODS: Patients suffering from non-arteritic ocular perfusion disorders were included. High-resolution compression sonography (18 MHz) images of the temporal arteries (frontal and parietal branch at the upper margin of the auricle) and facial arteries (at the crossing point of the artery over the mandible) were analysed for the presence of HIL (grade 0: absent; grade 1: moderate; grade 2: severe). Characteristics of patients with and without evidence of HIL >grade 1 were compared. RESULTS: In total, 330 cranial artery segments of 55 patients were analysed. HIL ≥grade 1 was present in 13.0% of all artery segments and in 38.1% of all patients. Patients with HIL ≥grade 1 in at least one arterial segment displayed significantly increased maximum wall thickness of the temporal arteries (0.62±0.23 mm vs 0.50±0.13 mm; p<0.01) and facial arteries (0.71±0.20 mm vs 0.54±0.19 mm; p=0.01). Patients with at least one temporal or facial artery segment with HIL were older, more often male and more frequently suffered from diabetes mellitus. CONCLUSION: The presence of HIL goes along with a significantly increased wall thickness of the temporal and facial arteries. These findings should be considered when interpreting the results of sonography of the cranial arteries in the diagnostic workup of suspected giant cell arteritis.

A Diagnostic Algorithm Based on a Simple Clinical Prediction Rule for the Diagnosis of Cranial Giant Cell Arteritis.

BACKGROUND: Risk stratification based on pre-test probability may improve the diagnostic accuracy of temporal artery high-resolution compression sonography (hrTCS) in the diagnostic workup of cranial giant cell arteritis (cGCA). METHODS: A logistic regression model with candidate items was derived from a cohort of patients with suspected cGCA (n = 87). The diagnostic accuracy of the model was tested in the derivation cohort and in an independent validation cohort (n = 114) by receiver operator characteristics (ROC) analysis. The clinical items were composed of a clinical prediction rule, integrated into a stepwise diagnostic algorithm together with C-reactive protein (CRP) values and hrTCS values. RESULTS: The model consisted of four clinical variables (age > 70, headache, jaw claudication, and anterior ischemic optic neuropathy). The diagnostic accuracy of the model for discrimination of patients with and without a final clinical diagnosis of cGCA was excellent in both cohorts (area under the curve (AUC) 0.96 and AUC 0.92, respectively). The diagnostic algorithm improved the positive predictive value of hrCTS substantially. Within the algorithm, 32.8% of patients (derivation cohort) and 49.1% (validation cohort) would not have been tested by hrTCS. None of these patients had a final diagnosis of cGCA. CONCLUSION: A diagnostic algorithm based on a clinical prediction rule improves the diagnostic accuracy of hrTCS.

Transocular sonography in acute arterial occlusions of the eye in elderly patients: Diagnostic value of the spot sign.

PURPOSE: To characterize the diagnostic yield of the spot sign in the diagnostic workup of acute arterial occlusions of the eye in elderly patients. METHODS: Clinical characteristics of consecutive patients aged ≥ 50 years with acute central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO) or anterior ischemic optic neuropathy (AION) were recorded. Videos of transocular sonography were assessed for the presence of the spot sign by two blinded readers. Group comparisons were made between CRAO-patients with and without the spot sign. Two experienced cardiovascular physicians allocated CRAO-cases to a presumed aetiology, without and with knowledge on the presence/absence of the spot sign. RESULTS: One-hundred-twenty-three patients were included, 46 of whom suffered from CRAO. A spot sign was seen in 32 of 46 of patients with CRAO and in 7 of 23 patients with BRAO. Interobserver agreement was excellent (Cohen`s kappa 0.98). CRAO-patients with the spot sign significantly more frequently had a medical history of cardiovascular disease (62.8 vs. 21.4%, p = 0.03) and left heart valve pathologies (51.9 vs. 10%, p = 0.03). The spot sign was not found in any of the three patients with CRAO secondary to cranial giant cell arteritis. The assumed CRAO aetiology differed in 37% of cases between two cardiovascular physicians, regardless whether transocular sonography findings were known or not. CONCLUSION: The spot sign is a simple sonographic finding with excellent interobserver agreement, which proofs the embolic nature of CRAO, but does not allow exact attribution of the underlying aetiology.

Atherosclerotic Burden and Arterial Stiffness are Not Increased in Patients with Milder Forms of Primary Aldosteronism Compared to Patients with Essential Hypertension.

Patients with primary aldosteronism (PA) are at increased cardiovascular risk, compared to patients with essential hypertension (EH). Cardiovascular damage could depend on PA phenotype, potentially being lower in milder forms of PA. Our aim was to assess atherosclerotic burden and arterial stiffness in 88 prospectively recruited patients, including 44 patients with mild PA and EH respectively. All patients underwent a structured study program, including measurements of ankle-brachial index, oscillometric measurement of central pulse wave velocity (cPWV) and vascular ultrasound examination of the supraaortic arteries, the abdominal aorta, and the femoropopliteal arteries. A plaque score was calculated to estimate atherosclerotic burden for each patient. This is a prospective case-control study set at a tertiary care hospital. Patients with PA and EH matched well for age, gender, blood pressure, BMI, and cardiovascular risk factors such as diabetes mellitus and smoking status. Common carotid intima-media thickness (0.77 vs. 0.75 mm; p=0.997) and cPWV (7.2 vs. 7.1 m/s; p=0.372) were comparable between patients with PA and EH. The atherosclerotic burden, as expressed by the plaque score, did not differ between the two groups (p=0.159). However, after initiation of treatment cPWV was significantly decreased in patients with PA (p=0.017). This study shows that subclinical atherosclerotic burden and arterial stiffness in patients with milder forms of PA is comparable to patients with EH. Nevertheless, specific treatment for PA significantly improved cPWV, which argues for a more liberal use of mineralocorticoid receptor antagonists in patients with arterial hypertension.

Retrobulbar Spot Sign in Metachronous Bilateral Central Retinal Artery Occlusion of Cardioembolic Origin.

ABSTRACT: A 78-year-old man suffered sudden visual loss of his right eye. Five years earlier, he had experienced vision loss of his left eye due to central retinal artery occlusion (CRAO); back then, the etiology for the CRAO was not established. Current ocular ultrasound depicted a hyperechoic spot within the optic nerve in both eyes. Echocardiography identified a calcified mass adherent to the mitral valve as the embolic source of the CRAO. This case shows the value of ocular B-mode ultrasound in demonstration and proof of the etiology for CRAO.

Survival and quality of life after early discharge in low-risk pulmonary embolism.

INTRODUCTION: Early discharge of patients with acute low-risk pulmonary embolism requires validation by prospective trials with clinical and quality-of-life outcomes. METHODS: The multinational Home Treatment of Patients with Low-Risk Pulmonary Embolism with the Oral Factor Xa Inhibitor Rivaroxaban (HoT-PE) single-arm management trial investigated early discharge followed by ambulatory treatment with rivaroxaban. The study was stopped for efficacy after the positive results of the predefined interim analysis at 50% of the planned population. The present analysis includes the entire trial population (576 patients). In addition to 3-month recurrence (primary outcome) and 1-year overall mortality, we analysed self-reported disease-specific (Pulmonary Embolism Quality of Life (PEmb-QoL) questionnaire) and generic (five-level five-dimension EuroQoL (EQ-5D-5L) scale) quality of life as well as treatment satisfaction (Anti-Clot Treatment Scale (ACTS)) after pulmonary embolism. RESULTS: The primary efficacy outcome occurred in three (0.5%, one-sided upper 95% CI 1.3%) patients. The 1-year mortality was 2.4%. The mean±sd PEmb-QoL decreased from 28.9±20.6% at 3 weeks to 19.9±15.4% at 3 months, a mean change (improvement) of -9.1% (p<0.0001). Improvement was consistent across all PEmb-QoL dimensions. The EQ-5D-5L was 0.89±0.12 at 3 weeks after enrolment and improved to 0.91±0.12 at 3 months (p<0.0001). Female sex and cardiopulmonary disease were associated with poorer disease-specific and generic quality of life; older age was associated with faster worsening of generic quality of life. The ACTS burden score improved from 40.5±6.6 points at 3 weeks to 42.5±5.9 points at 3 months (p<0.0001). CONCLUSIONS: Our results further support early discharge and ambulatory oral anticoagulation for selected patients with low-risk pulmonary embolism. Targeted strategies may be necessary to further improve quality of life in specific patient subgroups.