P3446Left-ventricular assist device as a destination therapy in Duchenne cardiomyopathy: are we ready to change the natural history?

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
R Adorisio ◽  
D D'Amario ◽  
N Cantarutti ◽  
M Cicenia ◽  
A D'Amico ◽  
...  

Abstract Background Heart failure is becoming the most prominent cause of death among patients with Duchenne Muscular Dystrophy (DMD) and end-stage cardiomyopathy (CMP). Implantation of left ventricular assist devices (LVAD) as destination therapy (DT) in this group of patients is still matter of debate. No current data are available on long-term follow- up of young patients. Purpose The aim of this study was to evaluate the long-term outcome of LVAD in DMD end stage heart failure (HF) compared to a group treated optimal medical treatment (OMT) with inotropes Methods All patients affected by DMD patients presenting with end-stage HF receiving OMT or LVAD have been reviewed. All data about characteristics of HF at presentation including assessment of nutritional status and respiratory function, left ventricular ejection fraction, brain natriuretic peptide, serum sodium, in-hospital treatment and long term follow up have been collected. OMT group was constituted by 4 patients matching implantation VAD criteria but not eligible or refusing treatment. Survival was represented by Kaplan Meier analysis. Results A total of 12 DMD patients with end-stage CMP were considered for this analysis. 8 have been implanted with LVAD during the period from 2011 until mid-2017 and compared with 4 treated with OMT. Mean systolic pressure was 103 versus 91 mmHg in those not receiving LVAD (p=0.2), and left ventricular ejection fraction at the time of presentation was 16‰ for LVAD group and 22‰ for the second group (p=0.1). LVADs improved survival throughout follow-up for patients undergoing baseline inotropic infusions (P=0.0014); for the LVAD group versus the OMT group, 1-month survival was 100‰ and 67‰ respectively and 2-month survival was 100‰ versus 0‰. For LVAD group survival was 86‰ at one year, 71‰ at 2 years and 48‰ at 5 years from implantation. Analysis on short and long-term follow-up showed significant improvement of survival in DMD patients treated with LVAD (log rank<0.001), with a 5-year increase in life expectancy. Conclusions LVAD improved outcomes in patients with LVAD when compared to those without. Long-term follow-up was similar to DT LVAD adult population. Further studies are necessary to confirm these data.

Kardiologiia ◽  
2020 ◽  
Vol 60 (4) ◽  
pp. 77-85
Author(s):  
E. A. Shmidt ◽  
S. A. Berns ◽  
A. V. Ponasenko ◽  
A. V. Klimenkova ◽  
S. A. Tumanova ◽  
...  

Aim To study a relationship of several factors (clinical and genetical markers) with unfavorable outcomes in patients with non-ST-segment elevation acute coronary syndrome (NSTE-ACS) in long-term follow-up.Material and methods This full-design, prospective study included 415 patients with NSTE-ACS. 266 patients were evaluated for the presence of multifocal atherosclerosis (MFA). Typing of polymorphic variants rs1041981 LTA, rs1800629 TNF, rs4986790, and rs498679 TLR4, and also rs3024491 and rs1800872 IL10 was performed. Follow-up period lasted for 67±4 months. By the end of this period, information about clinical outcomes for 396 patients became available.Results During the entire follow-up period, unfavorable outcomes were observed in 239 (57.5 %) patients with NSTE-ACS. The following clinical signs were associated with unfavorable outcomes: history of myocardial infarction, age >56 years, left ventricular ejection fraction (LV EF) ≤50 % and GRACE score ≥100, significant stenosis of brachiocephalic arteries, MFA, carriage of genotype А / А rs1041981 LTA (OR, 6.1; р=0.02) and allele А (OR, 1.9; р=0.01). According to results of a multifactorial analysis, the most significant predictors included LV EF <50 %, MFA, and carriage of genotype А / А rs1041981 LTA.Conclusion Stratification of patients with NSTE-ACS into groups of high or low risk for having an unfavorable outcome within the next 6 years is possible using the prognostic model developed and presented in this study. The model includes the following signs: LV EF <50 %, MFA, and carriage of genotype А / А rs1041981 LTA.


Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Weijian Huang ◽  
Lan Su ◽  
Songjie Wang ◽  
Shengjie Wu ◽  
Lei Xu ◽  
...  

Introduction: Left bundle branch pacing (LBBP) is a novel pacing method and has been proven to have low and stable pacing thresholds. However, data on large numbers of patients with long-term follow-up is still needed to evaluate its feasibility and safety. Hypothesis: To evaluate the feasibility and safety of LBBP in a large sample with long-term follow up. Methods: This study prospectively enrolled 632 consecutive patients with left bundle branch block, AV block or sick sinus syndrome with attempted LBBP from April 2017 to July 2019. Pacing parameters, ECG, echocardiographic measurements, complications were assessed at implant, and during follow-up of 1, 6, 12 and 24 months. Results: LBBP was successful in 618/632 (97.8%) patients according to the strict criteria for LBB capture. Mean follow-up time was 17.8±6.9 months. LBB capture threshold at implant was 0.65±0.27 [email protected] and 0.71±0.35 [email protected] at 2-year follow-up (n=166). Left ventricular ejection fraction were improved in QRS≥120ms and <120ms groups (60.62±14.22% vs. 63.95±11.29 %, p<0.001; 49.77±17.58 % vs. 58.58±12.79 %, p<0.001; respectively). The number of patients with moderate and sever tricuspid regurgitation decreased at 1-year (120 to 66;28 to 16; respectively). Permanent right bundle branch injury occurred in 55 (8.9%) patients. LBB capture threshold increased greater than 2 V in 6 patients, loss of capture in 2 patients, two lead dislodgements requiring operative revision within 1 month after implantation during 2-years follow-up. Conclusions: Feasibility and safety of LBBP were confirmed by this large sample size and long-term follow-up study. It is a reliable physiologic method for standards pacing indications.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Alessandra Leopizzi ◽  
Francesco Santoro ◽  
Adriana Mallardi ◽  
Enrica Vitale ◽  
Natale Daniele Brunetti

Abstract Aims Several studies have shown that Takotsubo syndrome (TTS) secondary to neurological disorders is associated with higher rate of in-hospital complications. Imaging brain studies found that atrophy or damage of some regions and their altered connectivity with other brain regions are typical features of TTS patients. The aim of the study is to evaluate rates of in hospital and long-term Follow-up events in TTS patients with history or acute cerebrovascular events (CVE). Methods and results 395 consecutive patients were enrolled in a multicentre prospective registry. History, clinical data, echocardiographic parameters and in-hospital and long-term follow-up events of all patients were evaluated. Prevalence of cerebrovascular events among TTS patients was 9.4% (num = 37). Compared to the control group, these patients were older (80 ± 7 vs. 71 ± 12, P = 0.01) and predominantly men (24% vs. 9%, P = 0.01). No differences in term of cardiovascular risk factors and left ventricular ejection fraction at admission and discharge were found among the two groups. The prevalence of physical stressor was higher in the CVE group (67% vs. 44% P = 0.01). The incidence of in-hospital adverse events was higher in the CVE than in the control one (70% vs. 29%), mainly driven by higher rate of Cardiogenic shock (19 vs. 8% P = 0.01) and in-hospital death (19 vs. 4%, P = 0.01). At long-term follow-up, patients in CVE group hat higher mortality rate (38% vs. 20% P = 0.01)). Patients presenting with acute CVE, 10 out of 37 patients (27%) when compared with chronic CVE had higher in-hospital mortality (40% vs. 11% P = 0.01). Conclusions TTS patients with history or acute cerebrovascular events had higher rate of in-hospital events and death at long-term follow-up. Acute cerebrovascular events in the setting of TTS are featured with worst prognosis.


2019 ◽  
Vol 13 (1) ◽  
pp. 13-23 ◽  
Author(s):  
Madeline K Mahowald ◽  
Nivedita Basu ◽  
Latha Subramaniam ◽  
Ryan Scott ◽  
Melinda B. Davis

Background: Prior studies of Peripartum Cardiomyopathy (PPCM) are limited by short-term follow-up. Contemporary long-term outcomes and change in myocardial function over time are poorly characterized. Methods and Results: This retrospective cohort study included women with PPCM at the University of Michigan (2000-2011), with follow-up on March 31, 2017. Subsequent pregnancies were excluded. Recovery was sustained left ventricular Ejection Fraction (EF) ≥55%. Major Adverse Events (MAE) included death, cardiac transplantation, left ventricular assist device, or inotrope-dependence. A total of 59 women were included (mean [SD] age at diagnosis, 29.5 [6.8]; 28.8% Black), with a mean follow-up of 6.3 years. Recovery occurred in 22 women (37%); of these, 8 women (36%) had delayed recovery (>12 months). All cause mortality was 20% (12/59) with median survival 4.2 years; of these, 9 women (75%) died after the first year (range 2 - 10 years). MAE occurred in 19 women (32%); of these, 11 women (42%) had MAE >12 months from time of diagnosis (range 2-20 years). Deterioration in EF by >10% from the time of diagnosis occurred in 16 women (27%). This group had worse long-term outcomes, including lower final EF (mean 25 vs 42%, p=0.010), less recovery (12 vs 46%, p=0.016), and higher rates of death (38 vs 14%, p=0.046) and MAE (56 vs 23%, p=0.016). Conclusion: Women with PPCM have long-term risks of mortality, MAE, and subsequent decline in EF, even in the absence of a subsequent pregnancy. Deterioration in EF is associated with adverse events; thus, long-term management is important.


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