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4D-Flow mean aortic pulse wave velocity in FH patients. The 4D-Flow through the aorta for 25 patients with FH is represented here showing increased mean pulse wave velocity (PWV) in the ascending aorta
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Familial hypercholesterolemia (FH) is an autosomal dominant disorder that affects 1 in 250 people. Aortic stiffness, measured by pulse wave velocity (PWV), is an independent predictor for cardiovascular events. Young FH patients are a unique group with early vessel wall disease that may serve to elucidate the determinants of aortic stiffness. We hy...
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Background
Young-onset hypertension is defined as hypertension diagnosed before the age of 40 years. Aortic pulse wave velocity is an indication of aortic stiffness. MRI assessment has been well verified compared to invasive pressure recordings for evaluating aortic pulse wave velocity. In this study, we aimed to determine whether aortic stiffness...
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Citations
... The main disadvantage of this method is that the AoL may not be measured accurately, especially in the setting of complex deformations of the aortic location or spine. In the field of cardiovascular surgery, measurement of aortic length and the cross-sectional area of the aorta using threedimensional aortic reconstruction with contrast-enhanced computed tomography (CT) has been shown to provide accurate results [13,14]. ...
... The AoL was defined as the length of this line from the bifurcation of the celiac artery (CA) to the inferior mesenteric artery (IMA) ( Figure 1A). This length was automatically calculated (Video 1) [13,14]. The AoA was determined as the cross-sectional area at the CA bifurcation, also calculated automatically ( Figure 1B). ...
Introduction
This research aimed to explore the relationship between spinal characteristics and the length of the abdominal aorta in adult spinal deformity (ASD) patients who underwent corrective spinal surgery. We hypothesized that adjusting spinal alignment might affect the abdominal aorta's length.
Methods
This study included thirteen patients with ASD (average age: 63.0 ± 8.9 years; four males and nine females) who received spinal correction surgery. We measured both pre-operative and post-operative spinal parameters, including thoracolumbar kyphosis (TLK), and calculated their differences (Δ). The length of the aorta (AoL) was determined using an automated process that measures the central luminal line from the celiac artery's bifurcation to the inferior mesenteric artery. This measurement was made using contrast-enhanced computed tomography for three-dimensional aortic reconstruction. We compared the pre-operative and post-operative AoLs and their differences (Δ). The study examined the correlation between changes in spinal parameters and changes in AoL.
Results
Post-operatively, there was an increase in aortic length (ΔAoL: 4.2 ± 4.9 mm). There was a negative correlation between the change in TLK and the change in AoL (R2 = 0.45, p = 0.012, β = −0.21). No significant correlations were found with other spinal parameters.
Conclusions
The abdominal aorta can elongate by 4.8% after spinal corrective surgery in patients with ASD. The degree of elongation of the abdominal aorta is associated with spinal alignment correction.
... These sex differences are explained by disease epidemiology, because ASD is more common in females, and CoA is more common in males. 37 Our ASD control group therefore included a relatively high number of females, while the CoA group included a higher number of males. Multivariable regression analysis identified male sex as a positive predictor for cIMT, which may have contributed to the higher cIMT in the CoA group compared with ASD controls. ...
Background
Adolescents with chronic disease are often exposed to inflammatory, metabolic, and hemodynamic risk factors for early atherosclerosis. Since postmortem studies have shown that atherogenesis starts in the aorta, the CDACD (Cardiovascular Disease in Adolescents with Chronic Disease) study investigated preclinical aortic atherosclerosis in these adolescents.
Methods and Results
The cross‐sectional CDACD study enrolled 114 adolescents 12 to 18 years old with chronic disorders including juvenile idiopathic arthritis, cystic fibrosis, obesity, corrected coarctation of the aorta, and healthy controls with a corrected atrial septal defect. Cardiovascular magnetic resonance was used to assess aortic pulse wave velocity and aortic wall thickness, as established aortic measures of preclinical atherosclerosis. Cardiovascular magnetic resonance showed a higher aortic pulse wave velocity, which reflects aortic stiffness, and higher aortic wall thickness in all adolescent chronic disease groups, compared with controls ( P <0.05). Age (β=0.253), heart rate (β=0.236), systolic blood pressure (β=−0.264), and diastolic blood pressure (β=0.365) were identified as significant predictors for aortic pulse wave velocity, using multivariable linear regression analysis. Aortic wall thickness was predicted by body mass index (β=0.248) and fasting glucose (β=0.242), next to aortic lumen area (β=0.340). Carotid intima‐media thickness was assessed using ultrasonography, and was only higher in adolescents with coarctation of the aorta, compared with controls ( P <0.001).
Conclusions
Adolescents with chronic disease showed enhanced aortic stiffness and wall thickness compared with controls. The enhanced aortic pulse wave velocity and aortic wall thickness in adolescents with chronic disease could indicate accelerated atherogenesis. Our findings underscore the importance of the aorta for assessment of early atherosclerosis, and the need for tailored cardiovascular follow‐up of children with chronic disease.
... Parameters of arterial stiffness as well as aortic root thickness by cardiac MRI have also been tested on heterozygous FH children. Testing 33 children aged 7-18, it was found that in comparison to a non-FH group of identical age, the PWV values of FH children were significantly higher (4.5 ± 0.8 vs. 3.5 ± 0.3 m/s; p < 0.001), and the wall thickness of the ascending aorta was higher (1.37 ± 0.18 vs. 1.3 ± 0.02 mm; p < 0.05), which suggests the importance of early statin treatment [61]. ...
Cardiovascular diseases are still the leading cause of mortality due to increased atherosclerosis worldwide. In the background of accelerated atherosclerosis, the most important risk factors include hypertension, age, male gender, hereditary predisposition, diabetes, obesity, smoking and lipid metabolism disorder. Arterial stiffness is a firmly established, independent predictor of cardiovascular risk. Patients with familial hypercholesterolemia are at very high cardiovascular risk. Non-invasive measurement of arterial stiffness is suitable for screening vascular dysfunction at subclinical stage in this severe inherited disorder. Some former studies found stiffer arteries in patients with familial hypercholesterolemia compared to healthy controls, while statin treatment has a beneficial effect on it. If conventional drug therapy fails in patients with severe familial hypercholesterolemia, PCSK9 inhibitor therapy should be administered; if these agents are not available, performing selective LDL apheresis could be considered. The impact of recent therapeutic approaches on vascular stiffness is not widely studied yet, even though the degree of accelerated athero and arteriosclerosis correlates with cardiovascular risk. The authors provide an overview of the diagnosis of familial hypercholesterolemia and the findings of studies on arterial dysfunction in patients with familial hypercholesterolemia, in addition to presenting the latest therapeutic options and their effects on arterial elasticity parameters.
... In the largest study of 267 adolescents, PWV was significantly elevated in those with high LDL-C [127]. Recently Tran et al. reported that the PWV as assessed by cardiac MRI is significantly increased (p < 0.001) in children with FH when compared to age-and sex-matched reference data [128]. Opposite to these results, Vlahos et al. found no difference in central pulse wave velocity in a group of 30 children with FH, measured noninvasively using applanation tonometry technique. ...
Heterozygous familial hypercholesterolaemia (FH) is among the most common genetic metabolic lipid disorders characterised by elevated low-density lipoprotein cholesterol (LDL-C) levels from birth and a significantly higher risk of developing premature atherosclerotic cardiovascular disease. The majority of the current pediatric guidelines for clinical management of children and adolescents with FH does not consider the impact of genetic variations as well as characteristics of vascular phenotype as assessed by recently developed non-invasive imaging techniques. We propose a combined integrated approach of cardiovascular (CV) risk assessment and clinical management of children with FH incorporating current risk assessment profile (LDL-C levels, traditional CV risk factors and familial history) with genetic and non-invasive vascular phenotyping. Based on the existing data on vascular phenotype status, this panel recommends that all children with FH and cIMT ≥0.5 mm should receive lipid lowering therapy irrespective of the presence of CV risk factors, family history and/or LDL-C levels Those children with FH and cIMT ≥0.4 mm should be Citation: Bjelakovic, B.; Stefanutti, C.; Reiner, Ž.; Watts, G.F.; Moriarty, P.; Marais, D.; Widhalm, K.; Cohen, H.; Harada-Shiba, M.; Banach, M. Risk Assessment and Clinical Management of Children and Adolescents with Heterozygous F amilial Hypercholesterolaemia. A
... The aorta wall simulation is solved using linear elastic constitutive equations [24], with a wall viscosity of 2.1 Pa s [5], and a wall density of 1.09g/cm 3 , following experimental measurements from X-ray phase-contrast tomography [38]. The wall thickness was set to be uniform at 1.3 mm based on the experimental measurements performed by Tran et al. [34]. Although their experiments had an average patient age of 14.6, Liu et al. [18] The implications of two outlet boundary conditions on blood flow Fig. 5 Spatially and phase-averaged blood flow rate (q * ) and normal Reynolds stress (u u ) at 0.25 s along the aorta pathway (d * ) for four spatial grids. ...
The blood flow in a normal aorta is simulated directly based on patient-specific data for boundary conditions. This study provides insight into the implications of using two different outlet boundary conditions in accurate modeling of the blood flow based on using pressure or flow rate outlet boundary conditions. Both boundary conditions at the outlet provided similar blood flow characteristics through the main aortic pathway with a reasonable accuracy of 4% relative to the laboratory measurements. Compared to the pressure outlet boundary condition, however, specifying the flow rate at the outlet underestimates pressure in the aortic arch and the flow rates through the outlets of the aortic arch. Additionally, using the flow rate boundary condition leads to an overestimation of peak local Reynolds Number in the aortic arch, and an inaccurate prediction of transition to turbulence.
... Moreover, hypercholesterolemia may inhibit the effect of vasodilators like endothelium-derived relaxing factor (nitric oxide) on the vascular smooth muscle of resistance vessels resulting in elevated blood pressure during exercise. Higher aortic pulse wave velocity compared to controls has been demonstrated in ehFH patients, with increased thickness and reduced compliance in the ascending aorta suggesting possible early cholesterol deposition [9]. Decreased aortic strain assessed by tissue Doppler imaging or speckle tracking echocardiography was also shown in hypertensive patients [10]. ...
Dyslipidemia is known as a strong risk factor for premature atherosclerotic cardiovascular disease and increased morbidity and mortality and can have an adverse effect on left ventricular function due to direct or indirect macrovascular and/or microvascular damage. Speckle-tracking echocardiography allows the assessment of subclinical cardiac dysfunction in different diseases on the basis of myocardial deformation indices, and decrease in longitudinal and circumferential strain was shown in patients with heterozygous familial hypercholesterolemia (heFH) without comorbidities. In this issue of the journal a new study presents the results in a well-defined population which included asymptomatic treatment-naive heFH individuals without known coronary/peripheral arterial disease, with normal left ventricular ejection fraction and no other risk factors as formal arterial hypertension or diabetes mellitus. A slight impairment of global longitudinal strain was present, despite normal standard echocardiographic parameters. Also, the higher rise in systolic and diastolic blood pressure of heHF patients during exercise treadmill test might reflect early preclinical hypertension. High cholesterol level may have produced endothelial dysfunction, which has been shown to be related to the extent of atherosclerotic process and cardiovascular damage. Relevant findings are reported on left ventricular strain reduction and increase in systolic/diastolic blood pressure in asymptomatic heFH males. The relationship between myocardial strain impairment and developing systemic arterial hypertension in hypercholesterolemic patients could be the subject of further subsequent investigation.
... In the presented study, the condition of the arterial wall was assessed by measuring the pulse wave velocity in the aorta in children with FH and healthy children of the same age. The study design differs from previous studies of PWV in the pediatric population presented in the literature [33][34][35][36] in distribution of participants into age subgroups. In our opinion, such an analysis of the PWV values increases the correctness and statistical significance of the obtained results. ...
Familial hypercholesterolemia (FH) is the genetic disease which characterized by an increase of level total cholesterol and low density lipoproteins since childhood. The aim of the study was to assess arterial stiffness in children with heterozygous FH by measuring the pulse wave velocity (PWV) in the aorta. The study involved 118 children, 60 healthy children in the control group and 58 children with heterozygous FH in the main group. Both groups were divided into 3 age subgroups: 5–7 years old, 8–12 years old and 13–17 years old. The diagnosis of FH was made using British criteria by Simon Broome. The lipid profile was determined for all children, blood pressure was monitored daily with an estimate of the minimum, mean and maximum PWV (PWVmin, mean PWV, PWVmax) in aorta using oscillometric method. Correlation analysis in patients with FH revealed direct correlation between PWVmin, mean PWV and PWVmax with total cholesterol (r = 0.46, r = 0.46 and r = 0.464, respectively, p < 0.001). The study demonstrates an increase in the PWV in the aorta in children with FH compared with healthy peers from 8–12 years of age and a progression of arterial stiffness most significant in the group of 13–17 years.
Evidence accumulated over the past four decades clearly indicates that atherosclerotic cardiovascular disease (ASCVD) processes begin early in life. Data generated from basic, clinical, and population-based studies conducted in the U. S. and globally underscore the importance of life course approaches to promoting cardiovascular health and preventing emergence of risk for CVD. This chapter presents the evidence supporting the importance of primordial and primary prevention beginning early in life and continuing through childhood, adolescence, and emerging adulthood. Evidence-based guidelines applicable across clinical and community-based settings are highlighted with implications for nurses, nursing, and multidisciplinary teams. Emphasis is placed on multifaceted, multi-contextual efforts focused on promoting ideal cardiovascular health in childhood and adolescence. Directions for future research designed to advance the science needed to guide and inform optimal approaches for promoting cardiovascular health in children and adolescents from diverse populations are highlighted.
Background and aims:
Children with familial hypercholesterolaemia (FH) have elevated low-density lipoprotein cholesterol (LDL-C) concentrations since birth, which increases the risk of cardiovascular disease in adulthood. Arterial injury and stiffness parameters, including carotid intima media thickness (cIMT), pulse wave velocity (PWV) and distensibility (DIST), can be detected early in childhood. We studied the associations between cIMT, PWV and DIST with the lipoprotein profile assessed by proton nuclear magnetic resonance (1H NMR) and with influential variables such as blood pressure (BP) or body mass index (BMI) in children with FH.
Methods and results:
In this cross-sectional study, we included 201 children (96 with FH and 105 non-FH controls). Clinical history, physical examination and standard biochemical studies were performed. FH genetic testing was performed when clinically indicated. Carotid ultrasonography and an advanced lipoprotein profile by 1H NMR were performed. Multivariate and classification methods were used. There were no differences between cIMT, PWV and DIST between FH and non-FH children. FH children presented more total LDL and large, medium and small particles. Small LDL particles, BMI and systolic BP determined the presence of pathological IMT in the FH group. LDL size, high-density lipoproteins and very low-density lipoprotein particles together with blood pressure determined the presence of pathological arterial wall elasticity.
Conclusions:
Alterations in lipoprotein parameters assessed by are associated with early structural and functional arterial characteristics in children with FH. BMI and BP act as boosting factors. Cardiovascular prevention should start early in children with FH, encompassing all components of a healthy lifestyle.
