[Functional assessment of internal carotid artery tortuosity in patients with multifocal atherosclerosis]. / Vazhnost' dinamicheskoi funktsional'noi otsenki patologicheskoi izvitosti vnutrennikh sonnykh arterii u bol'nykh s mul'tifokal'nym aterosklerozom.

The review is devoted to diagnosis and treatment of internal carotid artery tortuosity. The authors consider modern classification, epidemiology and diagnostic options using neuroimaging or ultrasound-assisted functional stress tests depending on medical history and complaints. In addition to standard Doppler ultrasound, rotational and orthostatic tests are advisable due to possible changes of local shape and hemodynamic parameters following body position changes, especially in patients with concomitant atherosclerotic stenosis. Thus, a personalized approach is especially important for treatment and diagnostics of internal carotid artery tortuosity.

[Dynamic functional assessment of internal carotid artery tortuosity in patients with multifocal atherosclerosis]. / Dinamicheskaya funktsional'naya otsenka patologicheskikh izvitostei vnutrennikh sonnykh arterii u bol'nykh s mul'tifokal'nym aterosklerozom. Klinicheskii sluchai.

A personalized approach with attention to anamnesis and specific symptoms is necessary in patients with internal carotid artery tortuosity. Neuroimaging (especially before elective surgery) or functional stress tests following ultrasound of supra-aortic vessels may be necessary depending on medical history and complaints. In addition to standard Doppler ultrasound, these patients should undergo rotational and orthostatic transformation tests. We analyze changes in shape and hemodynamic parameters within the tortuosity area in various body positions. This is especially valuable for patients with concomitant carotid artery stenosis. The article presents a clinical case illustrating the importance of such approach.

Clinical characteristics associated with pain in patients with peripheral vascular malformations.

OBJECTIVE: Vascular malformations (VM) can negatively impact the patient's quality of life (QoL). Pain is a common problem in these patients. The aim of this study was to investigate risk factors associated with pain and to assess how pain affects QoL. METHODS: This prospective cross-sectional study was conducted in a tertiary vascular anomaly expertise center. Between June and December 2020, all patients from our local database (334 adults and 189 children) with peripheral VMs were invited to complete the Outcome Measures for VAscular MAlformations questionnaire to evaluate the presence, frequency, and intensity of pain. Additionally, patients were asked to complete several Patient-Reported Outcome Measurement Information System scales to evaluate their QoL. Risk factors associated with pain were identified in bivariate analysis and multivariable logistic regression. QoL domains were compared between patients who experienced pain and patients who did not. RESULTS: A total of 164 patients completed the questionnaire about pain and 133 patients completed all QoL questionnaires. Approximately one-half of the patients (52%) reported pain in the past four weeks and 57% of these patients reported pain daily or several times a week. Female sex (P = .009), lesions located in the upper extremity (P < .001) or lower extremity (P < .001), and intramuscular/intraosseous lesions (P = .004) were independently associated with the presence of pain. The following QoL domains were diminished in patients who experienced pain in comparison with patients who did not: pain interference (P < .001), physical functioning (P < .001), and social participation (P < .001) in adults, and pain interference (P = .001), mobility (P = .001), and anxiety (P = .024) in children. CONCLUSIONS: Pain is a frequently reported complaint in patients with VMs and is present in approximately one-half of the patients. Patients with lesions located in the upper or lower extremity, intramuscular/intraosseous lesions, and female patients are more likely to experience pain. The presence of pain negatively impacted patients' QoL. Although VM are a benign condition and expectative management is frequently applied, our study shows that pain is a serious concern and needs to be actively assessed. Pain is a sign of various etiologies and should be examined to properly treat the pain.

Poor concordance between definitions of type III arch and implications for risk prediction and assessment for carotid artery stenting.

OBJECTIVE: The type III arch configuration has been inconsistently reported as a stroke risk factor during carotid artery stenting. However, at least three different methods for the definition of type III arch can be identified in the literature, related to the level of the origin of the innominate artery (IA). According to Casserly's definition, a type III arch presents with an origin of the IA below the horizontal plane of the inner curvature. According to Madhwal's definition, a type III arch has a distance greater than twice the diameter of the left common carotid artery between the highest point of the arch and the origin of the IA. According to MacDonald's definition, a type III arch presents with a distance of ≥2 cm between the highest point of the arch and the origin of the IA. Our aim was to assess the level of concordance between these different methods. METHODS: Anonymized thoracic computed tomography scans of 100 healthy patients were reviewed. Two of us independently stratified the selected cases as a type I to III arch, according to the three considered definitions. The interobserver level of concordance for each type III arch classification and level of concordance among the three definitions were assessed. RESULTS: The 100 selected patients (64% male) were 76 ± 7 years old. For each definition, the interobserver repeatability was almost perfect for all three (Madhwal, κ = 0.81; 95% confidence interval [CI], 0.71-0.99; MacDonald, κ = 0.82; 95% CI, 0.72-0.92; Casserly, κ = 0.84; 95% CI, 0.74-0.93). The level of concordance among the different definitions was very low (Madhwal vs MacDonald, 85% [P = .002]; 33% for type III arch; Madhwal vs Casserly, 60% [P < .0001]; 12% for type III arch; MacDonald vs Casserly, 75% [P < .0001]; 12% for type III arch). CONCLUSIONS: The three definitions of the type III arch have a very low level of concordance, which might account for the varying clinical relevance of this configuration. Our findings have relevant implications for risk prediction for carotid artery stenting based on the presence of a type III arch, for comparisons of the results from different studies, and for comparisons of different datasets from multicenter trials.

[Quality standards for ultrasonographic assessment of peripheral vascular malformations and vascular tumors. Report of the french society for vascular medicine. 2018 Update]. / Standards de qualité pour la pratique de l'examen écho-Doppler dans l'étude des malformations et tumeurs vasculaires. Rapport de la Société française de médecine vasculaire (SFMV) : actualisation 2018.

The quality standards of the French Society of Vascular Medicine for the ultrasonographic assessment of vascular malformations are based on the two following requirements: (1) technical know-how: mastering the use of ultrasound devices and the method of examination; (2) medical know-how: ability to adapt the methods and scope of the examination to its clinical indication and purpose, and to rationally analyze and interpret its results. AIMS OF THE QUALITY STANDARDS: To describe an optimal method of examination in relation to the clinical question and hypothesis. To homogenize practice, methods, glossary, and reporting. To provide good practice reference points, and promote a quality process. ITEMS OF THE QUALITY STANDARDS: The 3 levels of examination; their clinical indications and goals. The reference standard examination (level 2), its variants according to clinical needs. The minimal content of the examination report; the letter to the referring physician (synthesis, conclusion and proposal for further investigation and/or therapeutic management). Commented glossary (anatomy, hemodynamics, semiology). Technical bases. Setting and use of ultrasound devices. Here, we discuss ultrasonography methods of using of ultrasonography for the assessment of peripheral vascular malformations and tumors (limbs, face, trunk).

Assessment of children with vascular ring.

BACKGROUND: Vascular rings may cause pressure on the trachea and/or esophagus of varying degree, resulting in symptoms. This study assessed the presentation symptoms, diagnostic methods and treatment results after surgery in children with vascular ring. METHODS: Symptomatic vascular ring patients undergoing surgery between January 2010 and August 2014 at Mehmet Akif Ersoy Cardiovascular Research and Training Hospital, Istanbul, were retrospectively assessed. The presentation symptoms, demographic characteristics, and diagnostic tests were evaluated. Operative data and postoperative follow up, complications and problems were also examined in detail. RESULTS: Twenty-one patients underwent surgery, 13 (63%) of whom were male. Median age was 12 months (range, 1 month-8 years). Among these patients, 62% (n = 13) had double aortic arch, 24% (n = 5) had right-sided aortic arch + ligamentum arteriosum, 10% (n = 2) had left-sided aortic arch + aberrant right subclavian artery, and 4% (n = 1) had pulmonary sling abnormality. Computed tomography (CT) angiography was performed in 17/21 patients. All the patients were successfully operated on. In the early postoperative period, two patients had chylothorax and one had nerve paralysis of nervus laryngeus recurrens. One patient died in the early period, and two patients had ongoing stridor on follow up. CONCLUSIONS: CT angiography is effective for the differential diagnosis and visualization of vascular ring abnormality. In such cases, full recovery can be assured with early diagnosis and surgery.

Vascular Assessment for Transcatheter Aortic Valve Replacement: Intravascular Ultrasound Compared With Computed Tomography.

BACKGROUND: Assessment of the femoral and iliac arteries is essential prior to transcatheter aortic valve replacement (TAVR). It is critical for establishing candidacy for a femoral approach, and can help predict vascular complications. Although computed tomography angiography (CTA) is the standard imaging modality, it has limitations. OBJECTIVE: This study compared CTA with intravascular ultrasound (IVUS) in patients undergoing TAVR evaluation. METHODS: Fifteen patients undergoing pre-TAVR coronary angiography and hemodynamic assessment were recruited. Following coronary angiography, patients underwent distal aortography, bilateral iliac and femoral arteriography, and IVUS assessment. Vascular tortuosity, minimum lumen diameter, and cross-sectional area were obtained and the findings were compared with those obtained from CTA. RESULTS: Correlation between IVUS and CTA was strong for minimum luminal diameter (r=0.62). Concordance was also strong between CTA and invasive iliofemoral angiography for assessment of tortuosity (r=0.75). Utilizing Bland-Altman analysis, vessel diameters obtained by IVUS were consistently greater than those obtained by CTA. The angiography and IVUS strategy was associated with a lower overall mean contrast utilization (29 cc vs 100 cc; P<.001), reduced mean radiation exposure (527 mGy vs 998 mGy; P=.045), and no significant difference in mean test duration (13.3 minutes vs 10 minutes; P=.12). CONCLUSIONS: For femoral and iliac arterial assessment prior to TAVR, IVUS is a viable alternative to CTA with comparable accuracy, and the potential for less contrast use and less radiation exposure. IVUS is also a valuable adjunct to CTA in patients with borderline femoral access diameters or considerable CTA artifacts.

Gastrointestinal bleeding from vascular malformations: Is octreotide effective to rescue difficult-to-treat patients?

Gastrointestinal vascular malformations are responsible for 2-8% of all cases of bleeding and 30-40% of all obscure hemorrhages, being the most frequent cause of occult bleeding in older people. The aim of this review was to provide an up-to-date report about the use of octreotide in bleeding from both hereditary and acquired vascular malformations of the gastrointestinal tract. A systematic literature search was performed, using the keywords "gastrointestinal vascular malformation", "octreotide", "angiodysplasia", "portal hypertensive gastropathy", "gastric antral vascular ectasia", and "hereditary vascular malformations". The first line therapy of acute/chronic bleeding from digestive vascular malformations is endoscopy, followed by angiographic embolization and surgical resection when this is unsuccessful. In the setting of difficult-to-treat patients, octreotide has been proposed as an alternative therapeutic strategy. Studies reported in the literature show a high efficacy and safety of octreotide, but described only a small number of enrolled patients, heterogeneous therapeutic schedules and short-term follow-up, with the exception of acute bleeding from esophageal varices. As a consequence, the use of octreotide is not approved in this setting and it is currently still prescribed as an off-label drug. Studies in larger populations are needed to confirm the promising results observed in the small case series reports, so as to provide physicians with a treatment option for patients without available alternatives. Octreotide could also determine a strong decrease in the management costs of these clinical conditions, and especially, could dramatically reduce hospital admission costs.

Significance of CT scan and color Doppler duplex ultrasound in the assessment of Abernethy malformation.

BACKGROUND: Abernethy malformation is a rare congenital vascular abnormality in which the portal vein bypasses the liver and drains directly into the inferior vena cava. Diagnosis is complex and requires good quality imaging methods to identify details in systemic and portal circulation in order to establish diagnostic confirmation and treatment strategy. In this study we highlight the significance of the use of CT scans and Color Doppler Duplex Ultrasound for the diagnosis, treatment and evolution assessment in two adults with Abernethy malformation. CASE PRESENTATION: The diagnosis and the treatment of two patients with Abernethy malformation by CT scan and Color Doppler Duplex Ultrasound is described. One patient was submitted to liver transplantation due to chronic liver disease and multiple nodules diagnosed as adenoma. The other patient had normal liver function and a mild neurological and psychomotor dysfunction, therefore we adopted clinical treatment and close liver parenchyma evaluation and nodule surveillance, using an imaging approach involving intercalating CT scan and Color Doppler Duplex Ultrasound every 6 months. We highlight some important direct and indirect findings of non-invasive imaging methods. CONCLUSION: Abernethy malformation requires meticulous image diagnosis to improve treatment and avoid iatrogenic procedures. CT scans and Color Doppler Duplex Ultrasound are both efficient methods for diagnosis, treatment planning and evolution assessment of patients with Abernethy malformation.

Assessment of the interplay between blood and skin vascular abnormalities in adult purpura fulminans.

RATIONALE: Purpura fulminans in adults is a rare but devastating disease. Its pathophysiology is not well known. OBJECTIVES: To understand the pathophysiology of skin lesions in purpura fulminans, the interplay between circulating blood and vascular alterations was assessed. METHODS: Prospective multicenter study in four intensive care units. Patients with severe sepsis without skin lesions were recruited as control subjects. MEASUREMENTS AND MAIN RESULTS: Twenty patients with severe sepsis and purpura fulminans were recruited for blood sampling, and skin biopsy was performed in deceased patients. High severity of disease and mortality rates (80%) was observed. Skin biopsies in purpura fulminans lesions revealed thrombosis and extensive vascular damage: vascular congestion and dilation, endothelial necrosis, alteration of markers of endothelial integrity (CD31) and of the protein C pathway receptors (endothelial protein C receptor, thrombomodulin). Elevated plasminogen activating inhibitor-1 mRNA was also observed. Comparison with control patients showed that these lesions were specific to purpura fulminans. By contrast, no difference was observed for blood hemostasis parameters, including soluble thrombomodulin, activated protein C, and disseminated intravascular coagulation markers. Bacterial presence at the vascular wall was observed specifically in areas of vascular damage in eight of nine patients tested (including patients with Streptococcus pneumoniae, Neisseria meningitidis, Escherichia coli, and Pseudomonas aeruginosa infection). CONCLUSIONS: Thrombi and extensive vascular damage with multifaceted prothrombotic local imbalance are characteristics of purpura fulminans. A "vascular wall infection" hypothesis, responsible for endothelial damage and subsequent skin lesions, can be put forward.

[Angiographic assessment of the incidence rate of atypical variants of the development of crural and plantar arteries].

OBJECTIVE: assessing the incidence rate of atypical anatomical variants of the development of the popliteal artery and plantar arteries according to the findings of digital subtraction angiography. MATERIAL: Retrospective analysis of angiograms of 2,456 lower limbs in 1,305 patients. The character of the arterial lesion and the quality of the angiograms allowed of evaluating the anatomy of the popliteal artery and plantar arteries on a total of 1,609 extremities (65%). RESULTS: The incidence of atypical ramification of the popliteal artery amounted to 9%. Altered blood supply of the foot was observed in 7.6% of cases. The total incidence rate of atypical anatomical variants in men and women did not differ significantly. In the presence of a typical anatomical variant on one extremity, the prevalence of atypical blood supply of the other limb amounted to 16.3%. In the presence of an atypical anatomical variant on one limb, the incidence of atypical blood supply of the other amounted to 42%. In case of atypical blood supply of the foot on one limb, the incidence rate of the same-type abnormality on the other equalled 48%. CONCLUSION: In a typical variant of blood supply of one extremity, the probability of typical blood supply of the other amounted to 83 .7%, in an atypical variant decreasing to 58%. In case of atypical blood supply of one foot, one should suppose an analogous anatomical variant on the other foot in half of the cases.

[Airway obstruction caused by large blood vessel anomalies: assessment by flexible bronchoscopy].

OBJECTIVE: To evaluate the diagnostic value and safety of flexible bronchoscopy in congenital great vessel diseases complicated with airway compression. METHOD: The medical records of patients with great vessels abnormalities who were admitted to the neonatal intensive care unit (NICU) from October 2005 to June 2009 were retrospectively reviewed; 34 cases were diagnosed as airway compression by flexible bronchoscopy, 10 cases as vascular ring, 24 cases as aortal arch obstruction. The age of the patients was 6 d - 11 m, body weight 2.2 - 8.7 kg [(4.6 +/- 1.4) kg]. Recorded airway abnormalities detected by bronchoscopy and CT, cardiac vascular defects and airway compression were consistent with the findings on operation. The relation between the airway compression and cardiac vascular abnormalities, treatment of the airway compression and outcome were analysed. RESULT: Bronchoscopic assessment was successfully performed in NICU or operating room for all the patients. (1) Initial presentation of the 34 cases were tachypnea, stridor, refractory lung infection and prolonged mechanical ventilation. (2) Extrinsic compression was found in all the 10 cases with vascular ring by bronchoscopy initially which indicated vascular ring, airway compression was mainly of lower part of trachea. Diagnosis of 9 cases was consistent with CT diagnosis and in 1 case the diagnosis was confirmed by surgery; among these cases, 7 had congenital tracheal stenosis. (3) In the 24 cases with aortic obstructive lesion, 5 were detected to have tracheal stenosis by CT before correction of vascular abnormality, among whom one case was indicated to have tracheal stenosis by bronchoscopy, the other 19 cases were found with airway compression by bronchoscopy during or after vascular correction. Among the 24 cases, 21 had left main bronchial stenosis, 2 had congenital tracheal stenosis. Airway compression diagnosed by bronchoscopy agreed with the findings of CT. Two cases developed transient decrease of oxygen saturation, 5 cases developed transient tachycardia. CONCLUSION: Flexible bronchoscopy plays an important role in assessment of the airway compression complicated with great vessel abnormalities. Bronchoscopy is an accurate, convenient, safe and rapid way for airway assessment, but further examination of the peripheral structure and vascular malformation need combined examination with CT.