scholarly journals Determinants of cerebral radiological progression in Fabry disease

2020 ◽  
Vol 91 (7) ◽  
pp. 756-763 ◽  
Author(s):  
Simon Körver ◽  
Maria G F Longo ◽  
Marjana R Lima ◽  
Carla E M Hollak ◽  
Mohamed El Sayed ◽  
...  

Background and aimIt is unclear which patients with Fabry disease (FD) are at risk for progression of white matter lesions (WMLs) and brain infarctions and whether enzyme replacement therapy (ERT) changes this risk. The aim of this study was to determine the effect of ERT and clinical characteristics on progression of WMLs and infarctions on MRI in patients with FD.MethodsMRIs were assessed for WMLs (Fazekas scale), infarctions and basilar artery diameter (BAD). The effect of clinical characteristics (renal and cardiac involvement, cardiovascular risk factors, cardiac complications, BAD) and ERT on WML and infarction progression was evaluated using mixed models.ResultsOne hundred forty-nine patients were included (median age: 39 years, 38% men, 79% classical phenotype). Median follow-up time was 7 years (range: 0–13 years) with a median number of MRIs per patient of 5 (range: 1–14), resulting in a total of 852 scans. Variables independently associated with WML and infarction progression were age, male sex and a classical phenotype. Progression of WMLs and infarctions was not affected by adding ERT to the model, neither for the whole group, nor for early treated patients. Progression was highly variable among patients which could not be explained by other known variables such as hypertension, cholesterol, atrial fibrillation and changes in kidney function, left ventricular mass or BAD.ConclusionProgression of WMLs and cerebral infarctions in FD is mainly related to age, sex and phenotype. Additional effects of established cardiovascular risk factors, organ involvement and treatment with ERT are probably small to negligible.

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Jay Pandhi ◽  
Willem J Kop ◽  
John S Gottdiener

Left ventricular systolic dysfunction without heart failure (HF), also known as asymptomatic left ventricular systolic dysfunction (ALVSD), is at least as prevalent in the general population as left ventricular systolic dysfunction with heart failure (HF-LVSD). However, the clinical characteristics of ALVSD have not been well defined in an elderly population. Our aim is to evaluate the clinical features of elderly community-based individuals with ALVSD. The Cardiovascular Health Study is a multicenter cohort study designed to assess cardiovascular risk factors and outcomes in a population 65 years and older. This study quantifies baseline demographic variables and cardiovascular risk factors in participants with ALVSD. Comparisons were made with two reference groups: individuals with HF-LVSD, and those with normal left ventricular systolic function without HF (NL-LVSF). ALVSD was present in 385 of 5152 participants (7.5%) at baseline, whereas HF-LVSD was present in 84 participants (1.6%). Among those with ALVSD, 251 (65.2%) had borderline ejection fraction (EF) (45–54%), and 134 (34.8%) had impaired EF (< 45%). ALVSD was associated with elevated cardiovascular risk factors and comorbidities compared to NL-LVSF but lower than those in individuals with HF-LVSD (see table ). Among participants with ALVSD, impaired EF was associated with male sex and higher prevalence of coronary disease and LVH compared with borderline LV function. ALVSD is more common than HF-LVSD in community-dwelling elderly individuals. Furthermore, it is characterized by more cardiac risk factors and comorbidities than those with NL-LVSF, but less than those with HF-LVSD. The severity of systolic dysfunction is associated with comorbid cardiovascular risk factors. ALVSD may identify an important group of individuals at high risk for heart failure and cardiovascular mortality. Comparison of Clinical Characteristics Between ALVSD, NL-LVSF, and HF-LVSD


2014 ◽  
pp. 26-30
Author(s):  
Huu Thinh Nguyen ◽  
Thi Thuy Hang Nguyen ◽  
Bui Bao Hoang

Background: Cardiovascular disease is the major cause of death in dialysis patients, as well as in kidney transplant patients. Assessment of cardiovascular risks of renal transplant candidates to prevent or slow the progression of cardiovascular abệnh nhânormalities. Aim: 1) Evaluating cardiovascular risk factors, electrocardiographic and echocardiographic abnormalities in renal transplant candidates. 2) Identifying the correlation between cardiac morphological parameters with a number of factors involved. Subjects and Methods: We assessed 57 patients (73.7% male, mean age 32.4±8.8) with end-stage renal disease waiting for renal transplantation at Cho Ray Hospital between Jan 2012 and Jan 2013. All patients received a physical examination, blood pressure measurement, Hb, blood glucose test, lipid profile, ECG, echocardiography. Results: The percentage of hypertension was 98.2%, smoking (69.2%), dyslipidemia 40.4% and diabetes 12.3%. All patients had sinus rhythm, left ventricular hypertrophy 61.4% in ECG. Pericardial effusion 5.3%, mitral valve insufficiency 56.1%, aortic valve insufficiency 12.3%, left ventricular hypertrophy 94.7% in echocardiography. IVSd, LVPWd, LVMI positively correlated with kidney failure time (p <0.01, p<0.001), with DBP and SBP (p <0.05) and the degree of anemia (p <0.05). Percentage the degree of hypertension associated with proportion of left ventricular hypertrophy (p <0.05). Conclusions: Identification of cardiovascular risk factors for the prevention or intervention to reduce mortality in renal transplantation. Keywords: Cardiovascular risk factors, end-stage chronic renal failure, renal transplantation.


Author(s):  
Güzin Özden ◽  
Ayşe Esin Kibar Gül ◽  
Eda Mengen ◽  
Ahmet Ucaktürk ◽  
Hazım Alper Gürsu ◽  
...  

Abstract Objectives The objective of this study is to investigate the cardiovascular risk factors associated with metabolic syndrome (MetS), which is increasingly becoming prevalent in childhood obesity. Methods A total of 113 patients, 76 of whom were between the ages of 10 and 17 (mean age: 14.5 ± 1.8 years) and diagnosed with obesity (30 non-MetS and 46 MetS using IDF) and 37 of whom constituted the control group, participated in the study. Echocardiographic examination and atherogenicity parameters (Atherogenic index of plasma [AIP: logTG/HDL], total cholesterol/HDL, and TG/HDL ratio and non-HDL) were evaluated. Results The most common component accompanying obese MetS was found to be hypertension and low HDL. While obesity duration, body mass index (BMI), blood pressure, fasting insulin, insulin resistance, atherogenicity parameters were determined to be significantly higher in the obese-MetS group. Echocardiography showed that while the thickness, volume, and diameter of LV end-diastolic wall, left ventricular mass (LVM), LVM index (LVMI g/m2) and relative wall thickness (RWT) were significantly high in the MetS group, however, mitral E/A ratio was significantly lower (p<0.05). Change in LV geometry consistent with concentric remodeling (increased RWT, normal LVMI) was visible in obese groups. LVM were positively significantly related to BMI, waist circumference, insulin resistance, blood pressure, LDL level, and negative to mitral E/A ratio. In the obese-MetS group, LVMI was positively correlated to office systolic BP, left atrium end-diastolic volume/index. Conclusions LVMI and atherogenicity parameters that were found to be significantly higher in obese MetS exhibit increased cardiovascular risk in childhood.


Author(s):  
Jan-Per Wenzel ◽  
Ramona Bei der Kellen ◽  
Christina Magnussen ◽  
Stefan Blankenberg ◽  
Benedikt Schrage ◽  
...  

Abstract Aim Left ventricular diastolic dysfunction (DD), a common finding in the general population, is considered to be associated with heart failure with preserved ejection faction (HFpEF). Here we evaluate the prevalence and correlates of DD in subjects with and without HFpEF in a middle-aged sample of the general population. Methods and results From the first 10,000 participants of the population-based Hamburg City Health Study (HCHS), 5913 subjects (mean age 64.4 ± 8.3 years, 51.3% females), qualified for the current analysis. Diastolic dysfunction (DD) was identified in 753 (12.7%) participants. Of those, 11.2% showed DD without HFpEF (ALVDD) while 1.3% suffered from DD with HFpEF (DDwHFpEF). In multivariable regression analysis adjusted for major cardiovascular risk factors, ALVDD was associated with arterial hypertension (OR 2.0, p < 0.001) and HbA1c (OR 1.2, p = 0.007). Associations of both ALVDD and DDwHFpEF were: age (OR 1.7, p < 0.001; OR 2.7, p < 0.001), BMI (OR 1.2, p < 0.001; OR 1.6, p = 0.001), and left ventricular mass index (LVMI). In contrast, female sex (OR 2.5, p = 0.006), atrial fibrillation (OR 2.6, p = 0.024), CAD (OR 7.2, p < 0.001) COPD (OR 3.9, p < 0.001), and QRS duration (OR 1.4, p = 0.005) were strongly associated with DDwHFpEF but not with ALVDD. Conclusion The prevalence of DD in a sample from the first 10,000 participants of the population-based HCHS was 12.7% of whom 1.3% suffered from HFpEF. DD with and without HFpEF showed significant associations with different major cardiovascular risk factors and comorbidities warranting further research for their possible role in the formation of both ALVDD and DDwHFpEF.


Author(s):  
В.В. Шерстнёв ◽  
М.А. Грудень ◽  
О.В. Сенько ◽  
В.П. Карлина ◽  
А.В. Кузнецова ◽  
...  

В настоящее время доказано, что предгипертензия, характеризующая состояние лиц с артериальным давлением (АД) от 120/80 мм рт.ст. до 139/89 мм рт.ст., имеет высокую и постоянно возрастающую распространенность среди населения во всем мире и является независимым фактором риска формирования артериальной гипертонии, развития сердечно-сосудистой заболеваемости и смертности. Это делает актуальным изучение взаимосвязи развития предгипертензии с наличием других факторов риска сердечно-сосудистых заболеваний. Целью данного исследования явилось изучение гендерных особенностей взаимосвязи факторов риска сердечно-сосудистых заболеваний и развития предгипертензии. Методы исследования. Проведен сравнительный и корреляционный анализ показателей модифицируемых и немодифицируемых факторов риска сердечно-сосудистых заболеваний у 115 обследованных лиц мужского (n = 49) и женского пола (n = 66), средний возраст которых составил 47,6 ± 0,7 года с оптимальным АД (n = 63, АД < 120/80 мм рт.ст.) и предгипертензией (n = 52, АД 120-139/80-89 мм рт.ст.). Результаты исследования. Обнаружено, что мужчины и женщины с оптимальным АД и с предгипертензией различаются спектром факторов риска и структурой взаимосвязи исследованных показателей. У мужчин предгипертензия ассоциирована с повышенным содержанием креатинина в крови и табакокурением, тогда как у женщин - с гипертрофией левого желудочка сердца, стрессом и наследственной отягощенностью по гипертонической болезни. Развитие предгипертензии у женщин по сравнению с мужчинами характеризуется более выраженными количественными и качественными изменениями структуры взаимосвязей показателей исследованных факторов риска сердечно-сосудистых заболеваний. Заключение. Полученные результаты представляют интерес для понимания механизмов патогенеза предгипертензии и разработки стратегии её персонифицированной немедикаментозной и медикаментозной профилактики, основанной на устранении или ослаблении определенных факторов риска сердечно-сосудистых заболеваний. Currently it is proved that prehypertension characterizing the condition of individuals with blood pressure (BP) from 120/80 mm Hg to 139/89 mm Hg has a high, continuously increasing prevalence worldwide and is an independent risk factor for development of arterial hypertension and cardiovascular morbidity and mortality, which justifies studying the relationship between prehypertension and the presence of other risk factors for cardiovascular diseases. The aim of this study was to investigate gender features of the relationship between risk factors for cardiovascular disease and development of prehypertension. Methods. Comparative and correlation analyses of modifiable and non-modifiable cardiovascular risk factors were performed for 115 males (n = 49) and females (n = 66) aged 47.6 ± 0.7 with optimal BP (n = 63, BP < 120/80 mm Hg) or prehypertension (n = 52, AD = 120-139/80-89 mm Hg). Results. Men and women with optimal BP and prehypertension differed in the array of risk factors and the structure of relationship between the studied indices. In men, prehypertension was associated with an elevated blood creatinine and tobacco smoking whereas in women - with left ventricular hypertrophy, stress, and hereditary burden of hypertension. The development of prehypertension in women compared to men was characterized by more pronounced quantitative and qualitative changes in the structure of interrelationships between indices of the studied cardiovascular risk factors. Conclusion. The obtained results provide insight into pathogenetic mechanisms of prehypertension. They can be used for developing a strategy for individualized non-drug and drug prevention of hypertension based on elimination or reduction of certain cardiovascular risk factors.


Author(s):  
Andrew H. Tran ◽  
Thomas R. Kimball ◽  
Philip R. Khoury ◽  
Lawrence M. Dolan ◽  
Elaine M. Urbina

Objective: Pulse wave analysis estimates arterial wave reflections relating to left ventricular dysfunction and cardiovascular event risk in adults. Forward and backward waves (Pf and Pb) may improve risk stratification for cardiovascular events. Data in youth are lacking. We hypothesized that a significant difference in wave reflections would be identified in young subjects with adverse cardiovascular risk factors. Approach and Results: Vital signs and labs were obtained in 551 patients aged 10 to 24 years who were lean (L=199), obese (O=173), or had type 2 diabetes (T=179). Wave separation was performed. Differences in cardiovascular risk factors and wave reflections were assessed using ANOVA. General linear models were constructed to elucidate independent predictors of wave reflections. O and T subjects had an adverse cardiovascular risk profile versus L. O and T subjects had higher Pf and Pb versus L ( P ≤0.05). When adjusted for adiposity and other cardiovascular risk factors, reflection magnitude increased from L to O to T with higher T versus L values ( P ≤0.05) and near-significant O versus L values ( P =0.06). Adiposity and blood pressure were major determinants of wave reflections. Pb influenced log left ventricular mass index, log E/e′, and log composite carotid intima-media thickness. Conclusions: Adolescents and young adults with obesity and type 2 diabetes have altered forward and backward wave reflections versus lean controls related to adiposity, BP, and insulin levels. These parameters may help risk stratify patients with adverse cardiovascular risk factors.


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