A severe case of -related kyphoscoliotic Ehlers-Danlos syndrome associated with several arterial and venous complications: A case report.

Kyphoscoliotic Ehlers-Danlos syndrome (kEDS) is a rare genetic disorder combining congenital hypotonia, congenital/early onset and progressive kyphoscoliosis, and generalized joint hypermobility. Vascular fragility is another characteristic of the disease rarely described. We report a severe case of kEDS-PLOD1 with several vascular complications leading to difficulties in disease management.

Spontaneous Cervical Artery Dissection in Vascular Ehlers-Danlos Syndrome: A Cohort Study.

Background And Purpose: Vascular Ehlers-Danlos syndrome is a rare inherited connective tissue disorder because of pathogenic variants in the gene. Arterial complications can affect all anatomic areas and about 25% involve supra-aortic trunks (SATs) but no systematic assessment of cervical artery lesions has been made. The primary objective was to determine an accurate prevalence of spontaneous SAT lesions in a large series of patients with vascular Ehlers-Danlos syndrome at diagnosis and during follow-up.

Resistant Hypertension and Atherosclerotic Renal Artery Stenosis: Effects of Angioplasty on Ambulatory Blood Pressure. A Retrospective Uncontrolled Single-Center Study.

The effect of renal artery angioplasty on blood pressure in patients with true resistant hypertension and atherosclerotic renal artery stenosis has not been fully investigated due to the exclusion of these patients from most trials. In this study, we assessed the benefits of renal angioplasty on daytime ambulatory blood pressure (dABP) in this subgroup of patients. Medical records of our hypertension department were retrospectively analyzed from 2000 to 2016.

Vascular Ehlers-Danlos Syndrome: Long-Term Observational Study.

Background: Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic connective tissue disorder secondary to pathogenic variants within the COL3A1 gene, resulting in exceptional arterial and organ fragility and premature death. The only published clinical trial to date demonstrated the benefit of celiprolol on arterial morbimortality.

Objectives: The authors herein describe the outcomes of a large cohort of vEDS patients followed ≤17 years in a single national referral center.

Successful segmental thermal ablation of varicose saphenous veins in a patient with confirmed vascular Ehlers-Danlos syndrome.

We describe here the successful scheduled treatment of varicose veins by radiofrequency segmental thermal ablation in a 43-year-old patient with vascular Ehlers-Danlos syndrome. Her venous disease started at the age of 16 years, 1 year prior to her first major Ehlers-Danlos syndrome-related event which led to the diagnosis of her genetic condition. Surgical stripping was contra-indicated because of Ehlers-Danlos syndrome at the age of 18 years.

The type of variants at the COL3A1 gene associates with the phenotype and severity of vascular Ehlers-Danlos syndrome.

Vascular Ehlers-Danlos syndrome (vEDS) is a rare and severe autosomal dominant disorder caused by variants at the COL3A1 gene. Clinical characteristics and course of disease of 215 molecularly proven patients (146 index cases and 69 relatives) were analysed. We found 126 distincts variants that were divided into five groups: (1) Glycine substitutions (n=71), (2) splice-site and in-frame insertions-deletions (n=36), (3) variants leading to haplo-insufficiency (n=7), (4) non-glycine missense variants within the triple helix (n=4 variants), and (5) non-glycine missense variants or in-frame insertions-deletions, in the N- or C-terminal part of the protein (n=8).

Atheromatous plaque: quantitative analysis of the echogenicity of different layers.

Introduction And Objectives: Echographic studies of the composition of atheromatous plaque make use of the median gray level, which provides an overall measure of echogenicity. We propose adding an additional dimension to this approach by dividing the lesion into layers and generating a curve that shows the variation in echolucency with depth.

Methods: Femoral and carotid plaque in asymptomatic patients was investigated using both the median gray level and new layer

Methods: Interobserver variability was assessed for both

Methods: Three risk factors were studied: age, gender and smoking status.

Evaluation of a new disinfection procedure for ultrasound probes using ultraviolet light.

Following 183 ultrasound examinations, a randomized trial was conducted to compare three procedures for disinfection of probes under routine conditions: dry wiping with a soft, dry, non-sterile paper towel, antiseptic wiping with a towel impregnated with disinfectant spray and dry wiping followed by a 10 min ultraviolet C (UVC) cycle in a disinfection chamber. After ultrasonography, swabs were taken from transducer heads before and after cleaning and streaked onto plates that were then cultured. The number of colonies per plate was counted and organisms identified.

[Vascular Ehlers-Danlos syndrome].

The vascular type of Ehlers-Danlos syndrome (EDS) is a rare genetic disease transmitted as an autosomal dominant trait. It is distinguished from other forms of EDS by its unstable acrogeric morphotype and by vascular, gastrointestinal, and obstetrical complications. Diagnosis is based on various clinical signs, noninvasive imaging, and on the identification of a mutation of the COL3A1 gene, which provides diagnostic certainty but has a sensitivity of only 61%.

Relationship of circulating biomarkers of inflammation and hemostasis with preclinical atherosclerotic burden in nonsmoking hypercholesterolemic men.

Background: Relations of mediators of inflammation and hemostasis with preclinical atherosclerosis have been poorly analyzed. The aim of this study was to test potential associations of these blood markers with indicators of cardiovascular risk and atherosclerotic burden in asymptomatic, nonsmoking, hypercholesterolemic men.

Methods: A total of 87 men underwent cardiovascular risk assessment by means of 10-year Framingham risk calculation (median 9%) and atherosclerotic burden evaluation by means of ultrasonographic measurement of common carotid intima-media thickness and assessment of atherosclerotic plaques at three arterial sites (three-site plaques).

Influence of hypertension on early carotid artery remodeling.

Objective: We aimed to analyze the influence of hypertension on early large artery remodeling.

Methods And Results: Carotid intima-media thickness (IMT) and diameter were measured ultrasonographically in 394 normotensive subjects and 327 untreated and 528 treated hypertensive patients. IMT and diameter were increased in hypertensive groups, treated or untreated, compared with the normotensive group (P<0.

Determinants of progression of coronary artery calcifications in asymptomatic men at high cardiovascular risk.

Extended coronary artery calcifications (CAC) are predictive for cardiovascular complications but little is known about factors likely to influence CAC deposit. An analysis was undertaken to assess the cardiovascular risk factors that are capable of predicting CAC change over time. A retrospective analysis of CAC change was carried out in 55 asymptomatic men who underwent sequential electron beam computed tomographic measurement of CAC score a mean of 3.

Do arterial effects of antihypertensive drugs depend on subject's serum cholesterol?

Effects of antihypertensive treatment on large arteries may be influenced by the type of drug and concomitant risk factors such as blood cholesterol. To explore these possibilities we investigated the common carotid artery of 20 subjects with low cholesterol and 19 subjects with high cholesterol, all with essential hypertension, randomly allocated to 3 months of treatment with nitrendipine (20 mg/d) or trandolapril (2 mg/d). Carotid parameters were determined by recording instantaneous pressure (applanation tonometry) and diameter (echotracking device) and by modeling the pressure-diameter loop to obtain the Peterson modulus, stiffness index, measured and isobaric compliances, and wall viscosity.

Difference in carotid artery wall structure between Swedish and French men at low and high coronary risk.

Background And Purpose: We attempted to detect a group-specific north-south difference in carotid artery intima-media thickness (IMT), a marker of subsequent cardiovascular complication, by means of a case (high risk)-control (low risk) study in French and Swedish men.

Methods: The selection of high-risk and low-risk subjects was performed within the lower and upper percentiles of the Framingham risk distribution of 2 samples of 1000 white, male auto workers (45 to 50 years of age) in France (Renault) and Sweden (Volvo). In total, 299 men at low risk (79 French, 76 Swedish) and high risk (61 French, 83 Swedish), free from sustained hypertension, definite hypercholesterolemia, and cardiovascular disease, were included.

Gender difference in the influence of smoking on arterial wall thickness.

Background: We hypothesized that arterial wall thickening, an early atherogenic alteration, might be associated with smoking differently according to gender, considering the cardiovascular protection of female sex hormones.

Methods And Results: We measured ultrasonographically carotid and femoral intima-media thickness (IMT) in 194 men and 330 women without risk factors other than smoking. In men: (i) current smokers had greater carotid and femoral IMT (P<0.

Association of plasma viscosity and carotid thickening in a French working cohort.

Plasma viscosity and intima-media thickness (IMT) are frequently associated with cardiovascular disease and its risk factors. We evaluated the association of rheologic and vascular factors in asymptomatic subjects. Plasma viscosity (coaxial cylinder viscometry) and both preintrusive and intrusive atherosclerosis in the carotid arteries (ultrasonography) were investigated in 246 men and 337 women aged 17 to 65 years from the AXA study, a prospective cohort of healthy workers.

Distribution of ultrasonographically-assessed dimensions of common carotid arteries in healthy adults of both sexes.

Our objective was to provide a description of 'normal' carotid artery dimensions which are increasingly used for detecting early atherosclerosis and predicting clinical complications. Far wall intima-media thickness (IMT), lumen diameter and cross-sectional area intima-media thickness (CSA-IMT) were measured on 1 cm-distal common carotid artery segments on both sides by B-mode ultrasound, using an automated computerized edge-detection program, in 133 men and 216 women aged 17-65 years and free from cardiovascular disease and traditional risk factors. IMT and CSA-IMT increased with age in both sexes and on both sides, while diameter did not, except on the right side in men.

Predictive value of waist-to-hip ratio on cardiovascular risk events.

Background: A central distribution of adipose tissue is frequently associated with cardiovascular disease (CVD) and its risk factors.

Methods: Clinical usefulness of waist-to-hip ratio (WHR) for predicting the risk of cardiovascular events, estimated with models based on data from the Framingham and Prospective Cardiovascular Münster (PROCAM) studies was evaluated.

Subjects: These were 552 men and 160 women, asymptomatic and at risk for CVD, aged 30-74 y, recruited from an ongoing risk factor screening program conducted at worksites.

Evidence of carotid artery wall hypertrophy in homozygous homocystinuria.

Background: We aimed to determine whether intima-media thickness (IMT) was increased in the carotid artery of subjects with homocystinuria to better understand the in vivo contribution of homocysteine to early atherogenesis.

Methods And Results: We investigated ultrasonographically the right common carotid artery in 14 subjects with homozygous homocystinuria aged 3 to 34 years (mean, 13 years) and in 15 of their heterozygous parents aged 32 to 47 years (mean, 41 years) by comparison with 2 control groups of 15 healthy subjects of the same age. Far-wall IMT and lumen diameter were measured with a computerized program, and the cross-sectional area of the intima-media complex (CSA-IMC) was calculated from IMT and diameter.

Utility of electrocardiogram for predicting increased left ventricular mass in asymptomatic men at risk for cardiovascular disease.

The objective of this study was to test the value of electrocardiogram for predicting left ventricular mass (LVM), assessed echographically in 136 asymptomatic men with at least one major cardiovascular risk factor. We measured the Sokolow-Lyon and Cornell voltages, as well as the ratio of Cornell voltage to QRS voltage in lead II. The prevalence of left ventricular hypertrophy (LVH), defined as LVM of > or = 125 g/m2, was 6%, whereas that of increased LVM, defined as LVM of > or = 99 g/m2, the 90th upper percentile of a control group, was 29%.

Sex and topographic differences in associations between large-artery wall thickness and coronary risk profile in a French working cohort: the AXA Study.

Previous reports have investigated associations between carotid intima-media thickness (IMT) and cardiovascular risk factors. Our objective was to investigate this question in greater depth by measuring both femoral and carotid IMT in relation to sex and multifactorial coronary risk. We investigated carotid and femoral artery IMT by using ultrasonography in 326 men and 462 women, 17 to 65 years old.

Aortic stiffening does not predict coronary and extracoronary atherosclerosis in asymptomatic men at risk for cardiovascular disease.

Stiffness of aortic walls has been shown to be a marker of coronary and cerebrovascular diseases in patients with myocardial infarction or stroke. However, its value for predicting preclinical atherosclerosis has not been demonstrated. Therefore, this study tested the association of aortic wall stiffness and coronary and extracoronary atherosclerosis in the absence of clinical cardiovascular disease.

Preclinical changes of extracoronary arterial structures as indicators of coronary atherosclerosis in men.

Background: Carotid artery structure change was associated with coronary artery stenosis by angiography of subjects who were for the most part symptomatic.

Objective: To determine whether structural changes at multiple extracoronary sites were associated with noninvasively detected coronary calcium for 94 asymptomatic high-risk men.

Methods And Results: B-mode ultrasonography allowed us to detect plaque at three sites (carotid, femoral, and abdominal aorta) and to measure intima-medial thickness both in common carotid and in femoral arteries.