4310Left ventricular myocardial mass index is a predictor of clinical outcomes in patients with Fabry disease

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
S Moiseev ◽  
E Karovaikina ◽  
N Bulanov ◽  
A Moiseev ◽  
V Fomin

Abstract Fabry disease (FD) is a rare lysosomal storage disorder characterized by severe complications in young patients. The aim of the study was to determine predictors of clinical outcomes in patients with FD. Methods The diagnosis of FD was confirmed by enzymatic and genetic studies. The outcomes included death and a composite of end-stage renal disease (ESRD), cardiac (clinically significant arrhythmia and cardioverter/pacemaker implantation) and cerebrovascular (transient ischemic attack, stroke) events. Cox regression model was used to examine the risk of clinical outcomes. Results One hundred adult patients (65 males and 35 females, median age 40 [32; 50.8] years) with a definite diagnosis of FD were enrolled in our study. The majority of patients had classic phenotype (94.2%) and missense mutations in the GLA gene (69.0%). Forty-seven patients (38 males and 9 females) experienced clinical outcomes which included arrhythmias in 10 patients (atrial fibrillation in 8 of thems), ESRD in 33 patients, and cerebrovascular events in 8 patients. The median age of the first outcome was 39 (32; 49) years. In males, the outcomes occurred more frequently than in females (58.5% vs. 25.7%, respectively, p=0.002). Eleven males died at the median age of 43 (37; 46) years. All of them had ESRD. The most frequent cause of death was sudden cardiac death (n=9). Left ventricular myocardial mass index (LVMMI) was higher among patients with outcomes than in patients without outcomes (153 g/m2 [102; 203] vs. 92 g/m2 [78; 122], respectively, p<0.0001). However, there was no correlation between LVMMI and cardiac events (p=0.12). Using Cox regression analysis, LVMMI (HR 1.01 [1.002; 1.02], p=0.015), eGFR <60 ml/min/1.73m2 (HR 3.79 [1.87; 7.66], p<0.0001) and albuminuria>300 mg/day (HR 1.29 [1.04; 1.59], p=0.022) were associated with a higher risk of a composite outcome after adjustment for gender and age. Albuminuria>300 mg/day (HR 1.57 [1.05; 2.35], p=0.029) and LVMMI (HR 1.006 [1; 1.012], p=0.038) were predictors of death after adjustment for gender. However, this effect has been lost after adjustment for hemodialysis duration. The type of mutation in the GLA gene was not a predictor of clinical outcomes. Conclusion Left ventricular myocardial mass index was a predictor of clinical outcomes in patients with FD. Poor prognosis in our cohort was related to ESRD that accounted for all deaths.

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Nixiao Zhang ◽  
Wei Hua ◽  
Xiaoping Li ◽  
Yiran Hu ◽  
Hongxia Niu ◽  
...  

Objectives. To examine the association between the echocardiographic parameters measured as left atrial diameter (LAD) and left ventricular end-diastolic diameter (LVEDD) and long-term risk of all-cause mortality in adults with hypertrophic cardiomyopathy (HCM) following pacemaker implantation. Methods. A total of 94 adult patients with HCM who underwent pacemaker implantation from November 2002 to June 2013 in our Arrhythmia Center for symptomatic bradycardia and did not receive an implantable cardiac defibrillator (ICD) or cardiac resynchronization therapy (CRT) during follow-up were retrospectively extracted. Results. After careful examination of the medical records, we retrospectively evaluated the clinical characteristics of 74 patients with LAD records (58.1 ± 14.9 years) and 76 patients with LVEDD records (57.6 ± 15.2 years). Based on the receiver-operating characteristic (ROC) curve, the values of LAD = 44 mm and LVEDD = 43 mm were identified to predict the all-cause mortality. In the Kaplan–Meier survival, LAD ≥44 mm and LVEDD ≥43 mm were both significantly associated with all-cause mortality (log-rank test P<0.05). Cox regression analysis indicated that LAD ≥44 mm (HR 3.580; 95% CI = 1.055–12.148; P=0.041) was an independent predictor of all-cause mortality, while LVEDD ≥43 mm was not significantly associated with all-cause mortality. LVOTO was also significantly associated with all-cause mortality (HR = 0.166; 95% CI = 0.036–0.771; P=0.022). Conclusions. In HCM patients with pacemaker implantation, LAD ≥44 mm was an independent predictor of all-cause mortality.


2014 ◽  
Vol 2014 ◽  
pp. 1-7 ◽  
Author(s):  
Cheng-Jui Lin ◽  
Chi-Feng Pan ◽  
Chih-Kuang Chuang ◽  
Fang-Ju Sun ◽  
Duen-Jen Wang ◽  
...  

Background/Aims. Previous studies have reported p-cresyl sulfate (PCS) was related to endothelial dysfunction and adverse clinical effect. We investigate the adverse effects of PCS on clinical outcomes in a chronic kidney disease (CKD) cohort study.Methods. 72 predialysis patients were enrolled from a single medical center. Serum biochemistry data and PCS were measured. The clinical outcomes including cardiovascular event, all-cause mortality, and dialysis event were recorded during a 3-year follow-up.Results. After adjusting other independent variables, multivariate Cox regression analysis showed age (HR: 1.12,P=0.01), cardiovascular disease history (HR: 6.28,P=0.02), and PCS (HR: 1.12,P=0.02) were independently associated with cardiovascular event; age (HR: 0.91,P<0.01), serum albumin (HR: 0.03,P<0.01), and PCS level (HR: 1.17,P<0.01) reached significant correlation with dialysis event. Kaplan-Meier analysis revealed that patients with higher serum p-cresyl sulfate (>6 mg/L) were significantly associated with cardiovascular and dialysis event (log rankP=0.03, log rankP<0.01, resp.).Conclusion. Our study shows serum PCS could be a valuable marker in predicting cardiovascular event and renal function progression in CKD patients without dialysis.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Yoshida ◽  
A Shibata ◽  
A Tanihata ◽  
H Hayashi ◽  
Y Ichikawa ◽  
...  

Abstract Background Skeletal muscle atrophy is an independent prognostic predictor for patients with chronic heart failure, and the concept of sarcopenia is drawing attention. Furthermore, the importance of not only muscle mass but also intramuscular fat (IMF) has been pointed out. However, there is a lack of consensus on the implications of ectopic fat for the prognosis in patients with non-ischemic cardiomyopathy. Purpose We investigated whether ectopic fat in the thigh affects the prognosis with non-ischemic cardiomyopathy. Methods We recruited 105 patients who were diagnosed with non-ischemic cardiomyopathy by cardiac catheterization and echocardiographic date between September 2017 and November 2019. Finally 73 patients with reduced EF (EF 40% or less) enrolled in this prospective study. Functional status was evaluated by using cardiopulmonary exercise test at baseline. All patients were measured quantity of epicardial fat and thigh IMF percentage (%IMF) using computed tomography scan. Demographic, laboratory and echocardiographic date were collected from the patients' medical records. Clinical endpoints were unexpected readmission. Results During the follow-up period 18 patients had adverse events. The %IMF was significantly higher in the group with adverse events than without (5.57±5.70 and 3.02±2.44%, respectively; p&lt;0.01). Spearman correlation coefficient analysis showed a modest correlation between %IMF and lower limb extension strength (Spearman r=−0.280; p=0.0315), but there was no significant correlation between %IMF and exercise tolerance such as anaerobic threshold and peak oxygen uptake. Patients were divided into 2 groups according to the median values of %IMF. Kaplan-Meier analysis demonstrated that events were significantly higher in the high %IMF group (log-rank p=0.033). Multivariate Cox regression analysis adjusted for left ventricular end-diastolic diameter and peak ventricular oxygen consumption found %IMF as an independent factor of adverse events (hazard ratio 1.545; 95% confidential interval 1.151–2.087; p=0.004). Conclusions In non-ischemic cardiomyopathy patients with reduced EF, %IMF may have important adverse consequences such as increased cardiac-related events. Kaplan-Meier curves Funding Acknowledgement Type of funding source: None


Author(s):  
Sahrai Saeed ◽  
Anastasia Vamvakidou ◽  
Spyridon Zidros ◽  
George Papasozomenos ◽  
Vegard Lysne ◽  
...  

Abstract Aims It is not known whether transaortic flow rate (FR) in aortic stenosis (AS) differs between men and women, and whether the commonly used cut-off of 200 mL/s is prognostic in females. We aimed to explore sex differences in the determinants of FR, and determine the best sex-specific cut-offs for prediction of all-cause mortality. Methods and results Between 2010 and 2017, a total of 1564 symptomatic patients (mean age 76 ± 13 years, 51% men) with severe AS were prospectively included. Mean follow-up was 35 ± 22 months. The prevalence of cardiovascular disease was significantly higher in men than women (63% vs. 42%, P &lt; 0.001). Men had higher left ventricular mass and lower left ventricular ejection fraction compared to women (both P &lt; 0.001). Men were more likely to undergo an aortic valve intervention (AVI) (54% vs. 45%, P = 0.001), while the death rates were similar (42.0% in men and 40.6% in women, P = 0.580). A total of 779 (49.8%) patients underwent an AVI in which 145 (18.6%) died. In a multivariate Cox regression analysis, each 10 mL/s decrease in FR was associated with a 7% increase in hazard ratio (HR) for all-cause mortality (HR 1.07; 95% CI 1.03–1.11, P &lt; 0.001). The best cut-off value of FR for prediction of all-cause mortality was 179 mL/s in women and 209 mL/s in men. Conclusion Transaortic FR was lower in women than men. In the group undergoing AVI, lower FR was associated with increased risk of all-cause mortality, and the optimal cut-off for prediction of all-cause mortality was lower in women than men.


2021 ◽  
pp. 152660282199672
Author(s):  
Giovanni Tinelli ◽  
Marie Bonnet ◽  
Adrien Hertault ◽  
Simona Sica ◽  
Gian Luca Di Tanna ◽  
...  

Purpose: Evaluate the impact of hybrid operating room (HOR) guidance on the long-term clinical outcomes following fenestrated and branched endovascular repair (F-BEVAR) for complex aortic aneurysms. Materials and Methods: Prospectively collected registry data were retrospectively analyzed to compare the procedural, short- and long-term outcomes of consecutive F-BEVAR performed from January 2010 to December 2014 under standard mobile C-arm versus hybrid room guidance in a high-volume aortic center. Results: A total of 262 consecutive patients, including 133 patients treated with a mobile C-arm equipped operating room and 129 with a HOR guidance, were enrolled in this study. Patient radiation exposure and contrast media volume were significantly reduced in the HOR group. Short-term clinical outcomes were improved despite higher case complexity in the HOR group, with no statistical significance. At a median follow-up of 63.3 months (Q1 33.4, Q3 75.9) in the C-arm group, and 44.9 months (Q1 25.1, Q3 53.5, p=0.53) in the HOR group, there was no statistically significant difference in terms of target vessel occlusion and limb occlusion. When the endograft involved 3 or more fenestrations and/or branches (complex F-BEVAR), graft instability (36% vs 25%, p=0.035), reintervention on target vessels (20% vs 11%, p=0.019) and total reintervention rates (24% vs 15%, p=0.032) were significantly reduced in the HOR group. The multivariable Cox regression analysis did not show statistically significant differences for long-term death and aortic-related death between the 2 groups. Conclusion: Our study suggests that better long-term clinical outcomes could be observed when performing complex F-BEVAR in the latest generation HOR.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
K Matsushita ◽  
B Marchandot ◽  
M Kibler ◽  
C Sato ◽  
J Heger ◽  
...  

Abstract Introduction Paravalvular leakage (PVL) following transcatheter aortic valve replacement (TAVR) is associated with greater mortality. In clinical practice, determining PVL severity after TAVR remains challenging and often requires multiparametric assessment. Purpose This study sought to evaluate the respective value of various modalities of PVL assessments, including transthoracic echocardiography (TTE), cine-angiography, aortic regurgitation index (ARI), and closure time with adenosine diphosphate (CT-ADP), in the prediction of adverse clinical outcomes. Methods We included 1044 patients from our prospective TAVR registry between February 2010 and May 2019. Major adverse cardiac and cerebrovascular events (MACCE) was defined as a composite of all-cause death, myocardial infarction, stroke, and heart failure hospitalization within 1-year. Established cutoff values of ARI (&lt;25) and CT-ADP (&gt;180 sec) were used to assess the presence of PVL after TAVR. Results Moderate to severe PVL occurred in 14.2% and 5.2% of patients as measured by TTE and angiography. The rate of patients with ARI &lt;25 and CT-ADP &gt;180 sec were 36.5% and 24.9%, respectively. Among the four modalities, PVL evaluated by angiography predicted poorer clinical outcomes (Log rank test; p=0.001), whereas TTE, ARI &lt;25, and CT-ADP &gt;180 sec were not associated with 1-year MACCE. By multivariate Cox regression analysis, moderate to severe PVL by angiography was an independent predictor of 1-year MACCE (hazard ratio: 1.96; 95% confidence interval: 1.22–3.00; p=0.007). Conclusions Paravalvular leakage measured by angiography was evidenced as the most meaningful modality in the prediction of adverse clinical outcomes. Future multicenter studies are warranted to ensure these findings in the current TAVR era. Figure 1 Funding Acknowledgement Type of funding source: None


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Kairav Vakil ◽  
Rebecca Cogswell ◽  
Sue Duval ◽  
Wayne Levy ◽  
Peter Eckman ◽  
...  

Background: Current guidelines do not support routine use of implantable cardioverter-defibrillators (ICDs) in patients (pts) with end-stage heart failure (HF), unless these pts are awaiting advanced HF therapies such as left ventricular assist devices (LVADs) or a heart transplantation (HT). Whether ICDs improve survival in end-stage HF pts awaiting HT has not been previously examined in a large, multicenter cohort. Hypothesis: Presence of ICDs at time of listing for HT is associated with lower waitlist mortality. Methods: The United Network for Organ Sharing registry was used to identify adults (≥18 years) listed for HT between January 4, 1999 & September 30, 2014. Pts with congenital heart disease, total artificial heart, restrictive cardiomyopathy, prior HT, or missing covariates were excluded. Cox regression analysis was used to assess the impact of an ICD at the time of listing on waitlist mortality. Results: The analysis included 36,397 pts (mean age 53±12; 77% males) listed for HT. The prevalence of ICDs at listing has steadily increased over time before reaching a plateau in 2006 (27% in 1999, and range 76-82% between 2006-2014). In the unadjusted model, ICD use was associated with a 36% reduction in waitlist mortality (HR 0.64, 95% CI 0.60-0.68, p<0.001). After adjustment for covariates such as age, sex, race, creatinine, ischemic cardiomyopathy, and listing status, this association was nearly unchanged (HR 0.67, 95% CI 0.62-0.72, p<0.001). Test for interaction by listing era (pre- and post-2006) was non-significant (p=0.28). In the final adjusted model, that included listing era and LVAD status in addition to the above listed covariates, ICD use continued to remain associated with a mortality benefit on the waitlist for HT (HR 0.84, 95% CI 0.78-0.91, p<0.001). Conclusion: ICDs are increasingly prevalent in pts listed for HT; however many pts are still listed for HT without these devices. The presence of an ICD at the time of listing is associated with lower mortality on the waitlist. Although the magnitude of ICD efficacy diminishes slightly, its benefit continues to remain significant even after adjustment for listing era and LVAD use. Further analyses are required to identify specific sub-groups of pts where ICD use is most beneficial and appropriate.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
S Zhang ◽  
X Xie ◽  
C He ◽  
X Lin ◽  
M Luo ◽  
...  

Abstract Background Late left ventricular remodeling (LLVR) after the index acute myocardial infarction (AMI) is a common complication, and is associated with poor outcome. However, the optimal definition of LLVR has been debated because of its different incidence and influence on prognosis. At present, there are limited data regarding the influence of different LLVR definitions on long-term outcomes in AMI patients undergoing percutaneous coronary intervention (PCI). Purpose To explore the impact of different definitions of LLVR on long-term mortality, re-hospitalization or an urgent visit for heart failure, and identify which definition was more suitable for predicting long-term outcomes in AMI patients undergoing PCI. Methods We prospectively observed 460 consenting first-time AMI patients undergoing PCI from January 2012 to December 2018. LLVR was defined as a ≥20% increase in left ventricular end-diastolic volume (LVEDV), or a &gt;15% increase in left ventricular end-systolic volume (LVESV) from the initial presentation to the 3–12 months follow-up, or left ventricular ejection fraction (LVEF) &lt;50% at follow up. These parameters of the cardiac structure and function were measuring through the thoracic echocardiography. The association of LLVR with long-term prognosis was investigated by Cox regression analysis. Results The incidence rate of LLVR was 38.1% (n=171). The occurrence of LLVR according to LVESV, LVEDV and LVEF definition were 26.6% (n=117), 31.9% (n=142) and 11.5% (n=51), respectively. During a median follow-up of 2 years, after adjusting other potential risk factors, multivariable Cox regression analysis revealed LLVR of LVESV definition [hazard ratio (HR): 2.50, 95% confidence interval (CI): 1.19–5.22, P=0.015], LLVR of LVEF definition (HR: 16.46, 95% CI: 6.96–38.92, P&lt;0.001) and LLVR of Mix definition (HR: 5.86, 95% CI: 2.45–14.04, P&lt;0.001) were risk factors for long-term mortality, re-hospitalization or an urgent visit for heart failure. But only LLVR of LVEF definition was a risk predictor for long-term mortality (HR: 6.84, 95% CI: 1.98–23.65, P=0.002). Conclusions LLVR defined by LVESV or LVEF may be more suitable for predicting long-term mortality, re-hospitalization or an urgent visit for heart failure in AMI patients undergoing PCI. However, only LLVR defined by LVEF could be used for predicting long-term mortality. FUNDunding Acknowledgement Type of funding sources: None. Association Between LLVR and outcomes Kaplan-Meier Estimates of the Mortality


2021 ◽  
Vol 8 ◽  
Author(s):  
Jian Hua Chen ◽  
Guo Yao Chen ◽  
Hong Zheng ◽  
Quan He Chen ◽  
Fa Yuan Fu ◽  
...  

Objective: The present study aims to investigate the incidence and predictors of atrial high-rate events (AHREs) in patients with permanent pacemaker implants.Methods: A total of 289 patients who were implanted with a dual-chamber pacemaker due to complete atrioventricular block or symptomatic sick sinus syndrome (SSS) and had no previous history of atrial fibrillation were included in the present study. AHREs are defined as events with an atrial frequency of ≥175 bpm and a duration of ≥5 min. The patients were divided into two groups according to whether or not AHREs were detected during the follow-up: group A (AHRE+, n = 91) and group N (AHRE–, n = 198).Results: During the 12-month follow-up period, AHREs were detected in 91 patients (31.5%). The multivariate Cox regression analysis revealed that patient age [odds ratio [OR] = 1.041; 95% confidence interval [CI], 1.018–1.064; and P &lt; 0.001], pacemaker implantation due to symptomatic SSS (OR = 2.225; 95% CI, 1.227–4.036; and P = 0.008), and the percentage of atrial pacing after pacemaker implantation (OR = 1.010; 95% CI, 1.002–1.017; and P = 0.016) were independent AHRE predictors.Conclusion: The AHRE detection rate in patients with pacemaker implants was 31.5%. Patient age, pacemaker implantation due to symptomatic SSS, and the percentage of atrial pacing after pacemaker implantation were independent AHRE predictors.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Sung Ai Kim ◽  
Jong-Won Ha ◽  
Hyeon Chang Kim ◽  
Sungha Park ◽  
Eui-Young Choi ◽  
...  

Background : Previous studies of the prognosis of myocardial infarction (MI) have focused primarily on patients with left ventricular (LV) systolic dysfunction. Little is known about the prognosis of patients with MI and preserved ejection fraction (EF), which is increasing. Since the ratio of mitral inflow (E) and annular velocity (E′) to stroke volume (E/E′/SV) has been reported as an index of diastolic elastance (Ed), we hypothesized that Ed has prognostic implication in patients with acute MI and preserved EF. Method : Between May 2005 and January 2007, a total of 421 patients with acute MI were prospectively enrolled in Infarction Prognosis Study (IPS) registry. Among 358 patients who had comprehensive echocardiographic study, 42 patients with significant valvular heart disease or coexisted cardiomyopathy and 50 patients with decreased EF (<40%) were excluded. This left a total of 266 patients, who constituted the study population. The primary end-point was cardiovascular mortality. Results : Median follow-up duration was 12 months. Of 266 patients, cardiovascular death occurred in 11 (4.1%) patients. Age (p< 0.001), LA volume index (p=0.001), the severity of diastolic dysfunction (grade ≥ 2, p=0.04), Ed (p=0.003) were univariate predictors of cardiovascular mortality. However, in multivariate Cox regression analysis, age (p=0.008, HR; 1.14, 95% CI; 1.03–1.25) and Ed (p=0.009, HR; 1.72, 95% CI; 1.14 –2.58) were found to be independent predictors for cardiovascular mortality in patients with acute MI and preserved EF. Cut-off value of Ed for cardiovascular mortality determined by Kaplan-Meier method (p<0.001 by log-rank test) and ROC curve (AUC 0.87, sensitivity 90%, specificity 74%) was 0.25. Conclusion : Non-invasively determined ventricular diastolic elastance is a novel and powerful independent predictor of cardiovascular mortality in patients with acute MI and preserved EF.


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