scholarly journals Prediction of long-term survival in haemoglobinopathies: insights from cardiac imaging and ferritin

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
V Kamperidis ◽  
M Vlachou ◽  
Z Pappa ◽  
D Pantelidou ◽  
T Karamitsos ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Aims.The data on echocardiography, cardiac magnetic resonance (CMR) and ferritin predicting long-term survival in haemoglobinopathies are scarce. The current study evaluated the association of these parameters with the 10-years survival in haemoglobinopathies. Methods.This prospective study included stable consecutive haemoglobinopathy patients .Demographics, ferritin, echocardiography and CMR parameters were prospectively collected. Results. In total, 83 patients (mean age 38.4 ± 12.0 years, 46% male) with haemoglobinopathies were included and dichotomized based on their survival status after a follow-up of 9.8 ± 1.4 years. Patients who died were older (45.3 ± 11.6 vs 37.1 ± 11.7 years, p = 0.025), had higher ferritin levels (2498 vs 754 ng/ml, p = 0.001), higher right ventricular systolic pressure (RVSP) (41 ± 10 vs 31 ± 11mmHg, p = 0.001), more frequently elevated left ventricular (LV) end-diastolic pressure (70 vs 35%, p = 0.039) and lower CMR T2* values (23 ± 12 vs 35 ± 12ms, p = 0.007). Older age (HR: 1.053, p = 0.018), ferritin >2000ng/ml (HR: 3.517, p = 0.03), and >950ng/ml (HR: 11,135, p = 0.02), elevated LV end-diastolic pressure (HR: 3.977, p = 0.046), RVSP >34mmHg(HR: 10,134, p = 0.003), CMR T2* <20msec (HR: 4.900, p = 0.018) and <36msec (HR: 9.376, p = 0.035) were associated with increased all-cause mortality. A baseline model including age was created andit became more predictive of worse survival by adding RVSP >34mmHg than elevated LV end-diastolic pressure (C index 0.777 vs. 0.757 respectively) or ferritin >950ng/ml than >2000ng/ml (C index 0.805 vs. 0.770 respectively) or CMR T2*<36msec than <20msec (C index 0.825 vs. 0.810 respectively). Conclusions. In haemoglobinopathy patients, RVSP >34mmHg, ferritin >2000ng/ml and CMR T2* <20ms were associated with worse long term survival.In the current era of advanced chelation therapy, aiming for ferritin <950ng/ml and CMR T2* >36ms appears to improve their prognosis. Abstract Figure.

2003 ◽  
Vol 23 (2_suppl) ◽  
pp. 104-108 ◽  
Author(s):  
Kai-Chung Tse ◽  
Sing-Leung Lui ◽  
Wai-Kei Lo

Objective We investigated the clinical condition and complications of patients on peritoneal dialysis (PD) and on hemodialysis (HD) for more than 12 years. Design This retrospective review was carried out in the renal unit of the Tung Wah Hospital, Hong Kong. Patients and Methods Of 103 HD and 341 PD patients who started dialysis before 1990, 14 HD and 22 PD patients were dialyzed for more than 12 years. We evaluated basic demography at the 12th year of dialysis and at the most recent follow-up, and assessed the prevalence of cardiovascular disease, bone disease, dialysis-related amyloidosis (DRA), and acquired cystic disease (ACD). Outcomes and mortality were recorded. Results The 36 patients in the study included 22 women and 14 men. The PD patients were older ( p = 0.021) and had lower levels of serum phosphate and calcium × phosphate product. Only 3 patients were diabetic. Cardiovascular disease was present in 30 patients (83.3%), the most common types being ischemic heart disease (IHD, n = 11) and left ventricular hypertrophy (LVH, n = 22). Symptomatic DRA was found in 13 patients (36.1%), more commonly in the HD group ( p = 0.014). Bone disease was present in 32 patients (88.9%), with parathyroidectomy being more frequently performed in the PD patients ( p = 0.048). Symptomatic ACD occurred in 5 patients (13.9%). At the most recent follow-up, 26 patients were still on dialysis, 3 patients had undergone renal transplantation, and 7 patients had died, the causes of death being sudden death ( n = 3), cerebrovascular accident ( n = 1), chest infection ( n = 2), and peritonitis ( n = 1). Patient survival was similar in the PD and HD groups. Age at commencement of dialysis predicted mortality ( p = 0.012), but mode of dialysis, sex, and presence of diabetes mellitus did not. Conclusions Long-term survival is possible for both dialysis modalities (PD and HD), particularly for young, non diabetic patients. Symptomatic DRA is less common in PD patients, but the prevalence of other long-term complications is similar for both groups. Cardiovascular-related problems remain the leading cause of death.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Ayyaz Ali ◽  
Amit Patel ◽  
Yasir Abu-Omar ◽  
Anila Mehta ◽  
Ziad Ali ◽  
...  

Background Aortic valve replacement (AVR) is followed by regression of LVH. More complete resolution of LVH is suggested to be associated with superior clinical outcomes, however its impact on long-term survival following AVR has not been investigated. Methods Demographic and clinical data were obtained retrospectively through casenote review. Transthoracic echocardiography was used to measure LVM pre-operatively and at annual follow-up visits. Patients were grouped according to their reduction in LVM at late follow-up: Group A < 25 grams, Group B 25–150 grams and Group C > 150 grams. Results 211 patients underwent AVR between 1 st January 1991 and 1 st January 2001. Pre-operative LVM was 295 ± 118 g in A (n=63), 346 ± 97 g in B (n=75) and 539 ± 175 g in C (n=73), P <0.001. Mean time to last echocardiogram was 6.4 ± 3.3 years. LVM at late follow-up was 351 ± 160 g in A, 265 ± 95 g in B and 270 ± 90 g in C, P <0.001. Transvalvular gradients at follow-up were not significantly differerent between groups (A: 21 ± 21 mm Hg, B: 20 ± 15 mm Hg, C: 14 ± 11 mm Hg), P = 0.10. There was no difference in the prevalence of other factors influencing LVM regression such as IHD or hypertension. Ten year actuarial survival was significantly greater in patients with enhanced LVM regression when compared with the log-rank test (A: 49% ± 7, B: 67%± 6, C: 75% ± 6), P =0.03 (Figure 1 ). LVM reduction > 150 grams was an independent predictor of long-term survival on multivariate analysis (P = 0.03). Conclusion Enhanced LVM regression at late follow-up in patients undergoing AVR is associated with improved long-term survival. Strategies to optimize post-operative LVM regression should be considered in view of potential prognostic benefit.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
M Geyer ◽  
K Keller ◽  
S Born ◽  
K Bachmann ◽  
M M Hell ◽  
...  

Abstract Background A symptomatic and prognostic benefit by Transcatheter edge-to-edge repair (TEER) for mitral regurgitation (MR) has been proven. A variety of individual factors including female sex has been suggested to be associated with adverse outcome in cardio-surgical procedures. Purpose While gender is factored in common risk factor models for adverse outcome, evidence on sex-specific differences in long-term outcome after TEER for MR is limited. We aimed to investigate the impact of gender on prognosis in a large monocentric cohort with long-term follow-up. Methods We analyzed survival stratified for gender after successful isolated edge-to-edge repair of MR in the period between 06/2010 and 03/2018 (exclusion of combined forms of TMVR) in a monocentric retrospective cohort by performing survival analyses and cox regression analyses. Results Consecutively, 627 patients (47.0% females, 57.4% functional MR; survival status was available in 96.7%) entered the study and were followed for a median follow-up period of 462 days [IQR 142–945 days]. Survival rates were 97.6% at discharge, 75.7% after 1, 54.5% after 3, 37.6% after 5 and 21.7% after 7 years. Risk score as calculated by the Logistic Euroscore I did not differ significantly between females and males (at baseline: 25.0 [IQR 18.0/34.8] vs. 27.0 [18.4/40.1]%, p=0.093) and no relevant differences were found for in-hospital (2.0 vs. 2.7%, p=0.613), 30 days (4.8 vs. 6.5%, p=0.473) and 1-year mortality (27.0 vs. 25.3%, p=0.675). At the time of procedure, women were older (79.9 [IQR 75.6/84.4] vs. 78.3 [72.9/83.4] years, p&lt;0.001), were less often affected by coronary artery disease (53.1% vs. 75.0% p&lt;0.001), diabetes mellitus (23.7% vs. 31.3%, p=0.040) and impaired left ventricular function (44.5±12.9% vs. 38.9±13.4%, p&lt;0.001). Regarding long-term survival, women had a better prognosis after MR-therapy, especially in functional etiology: e.g., 4-year survival in FMR 65.7 vs. 35.7%, p=0.006 (Figure 1). Remarkably, female sex was associated with a lower risk for long-term mortality in the Cox-regression models, especially in the FMR subgroup (total cohort: univariate HR 0.81 [0.62–1.04], p=0.101; FMR: univariate HR 0.68 [0.49–0.96], p=0.028). Conclusion In our cohort of patients undergoing TEER for MR, we found no evidence for an impaired short- and mid-term prognosis for female patients. In contrary and not as indicated by Logistic Euroscore, female sex was associated with better long-term survival in comparison to men despite higher median age, which might be partly explained by a slightly more favorable cardiovascular risk profile. FUNDunding Acknowledgement Type of funding sources: None. Figure 1


2016 ◽  
Vol 23 (6) ◽  
pp. 1003-1008 ◽  
Author(s):  
Antonio Macciò ◽  
Paraskevas Kotsonis ◽  
Giacomo Chiappe ◽  
Luca Melis ◽  
Fausto Zamboni ◽  
...  

2017 ◽  
Vol 44 ◽  
pp. 16-21 ◽  
Author(s):  
Michael H. Antoni ◽  
Jamie M. Jacobs ◽  
Laura C. Bouchard ◽  
Suzanne C. Lechner ◽  
Devika R. Jutagir ◽  
...  

2009 ◽  
Vol 37 (06) ◽  
pp. 1059-1068 ◽  
Author(s):  
Min Ge ◽  
Shanfeng Ma ◽  
Liang Tao ◽  
Sudong Guan

The relationship between changes of cardiac function and the gene expressions of two major myocardial skeleton proteins, titin and nebulin, and the effect of gypenosides on these gene expressions in diabetic cardiomyopathy rat were explored in the present study. Forty Sprague-Dawley rats were randomly divided into three groups: control group, diabetic cardiomyopathy group and gypenosides-treated diabetic cardiomyopathy group. The diabetic cardiomyopathy was induced in rats by injecting streptozotocin (STZ, 55 mg/kg) intraperitoneally. Seven weeks after the rats suffered from diabetes, the rats were treated with gypenosides 100 mg/kg per day orally for six weeks in gypenosides-treated group. In the meanwhile, the pure water was given to diabetic cardiomyopathy and the control groups. Subsequently, the cardiac functions, including left ventricular systolic pressure (LVSP), left ventricular end diastolic pressure (LVEDP), ± dP/dtmax and t–dP/dmaxt, as well as the mRNA content and proteins of titin and nebulin in myocardium were determined. The results indicated that (1) the diabetic cardiomyopathy rats had decreased LVSP and ± dP/dtmax, increased LVEDP, and prolonged t–dP/dtmax than normal rats; (2) LVSP and ± dP/dtmax in diabetic cardiomyopathy rats treated with gypenosides were significantly higher and LVEDP and t–dP/dtmax were significantly lower than those without giving gypenosides; (3) the mRNA contents and proteins of titin and nebulin in diabetic cardiomyopathy rats were remarkably lower than those in the control rats and gypenosides had no effect on mRNA and protein expression levels of titin and nebulin in diabetic cardiomyopathy rats. We conclude that (1) the cardiac function as well as the mRNA expressions of titin and nebulin decreased in diabetic cardiomyopathy rats; (2) gypenosides secure cardiac muscles and their function from diabetic impairment and these beneficial effects of gypenosides are not by changing the expressions of titin and nebulin.


2017 ◽  
Vol 22 (4) ◽  
pp. 307-315 ◽  
Author(s):  
Kavita B Khaira ◽  
Ellen Brinza ◽  
Gagan D Singh ◽  
Ezra A Amsterdam ◽  
Stephen W Waldo ◽  
...  

The impact of heart failure (HF) on long-term survival in patients with critical limb ischemia (CLI) has not been well described. Outcomes stratified by left ventricular ejection fraction (EF) are also unknown. A single center retrospective chart review was performed for patients who underwent treatment for CLI from 2006 to 2013. Baseline demographics, procedural data and outcomes were analyzed. HF diagnosis was based on appropriate signs and symptoms as well as results of non-invasive testing. Among 381 CLI patients, 120 (31%) had a history of HF and 261 (69%) had no history of heart failure (no-HF). Within the HF group, 74 (62%) had HF with preserved ejection fraction (HFpEF) and 46 (38%) had HF with reduced ejection fraction (HFrEF). The average EF for those with no-HF, HFpEF and HFrEF were 59±13% vs 56±9% vs 30±9%, respectively. The likelihood of having concomitant coronary artery disease (CAD) was lowest in the no-HF group (43%), higher in the HFpEF group (70%) and highest in the HFrEF group (83%) ( p=0.001). Five-year survival was on average twofold higher in the no-HF group (43%) compared to both the HFpEF (19%, p=0.001) and HFrEF groups (24%, p=0.001). Long-term survival rates did not differ between the two HF groups ( p=0.50). There was no difference in 5-year freedom from major amputation or freedom from major adverse limb events between the no-HF, HFpEF and HFrEF groups, respectively. Overall, the combination of CLI and HF is associated with poor 5-year survival, independent of the degree of left ventricular systolic dysfunction.


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