P497Arrhythmic burden in patients with filamin C (FLNC) truncating variant associated cardiomyopathy

EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Zupan Meznar ◽  
D Zizek ◽  
U D Breskvar Kac ◽  
K Writzl ◽  
M Jan ◽  
...  

Abstract Background Truncating variants in FLNC gene are associated with an overlapping phenotype of arrhythmogenic and dilated cardiomyopathy. There are reports of high arrhythmia propensity with sudden cardiac death (SCD) often being the first symptom of the disease. It has been suggested that the current European guidelines primary prevention (PP) recommendation about implantable cardioverter-defibrillator (ICD) implantation might not be applicable in these patients and that earlier intervention should be considered. Purpose We sought to investigate the arrhythmic burden in FLNC truncation carriers in our centre. Methods Adult FLNC truncation carriers diagnosed in our centre between the years 2018 and 2019 were included in the study. We retrospectively analysed clinical data, and ICD follow-up reports in the cohort. Patients implanted with an ICD were divided in 3 groups: group A (secondary prevention ICD implantation), group B (PP indication according to the current guidelines – left ventricular ejection fraction (LVEF) below 35%) and group C (early PP– FLNC truncation carrier, LVEF < 50% and late gadolinium enhancement on cardiac magnetic resonance). We report the number of patients experiencing SCD and the number of appropriate and inappropriate ICD therapies per group. Results Twenty-four adult patients from 3 different families with three distinct FLNC truncating variants were identified. Ten (42%) were male; the average age was 45 ± 14 years. There were 3 (13%) SCDs in one family (2 male and one female, 29-42 years old) and two (8%) aborted SCDs in the remaining two families (one male and one female, 66 and 51 years old). Altogether eleven (46%) patients were implanted with an ICD. There were three patients in group A (2 aborted SCDs and 1 sustained ventricular tachycardia (VT)), two patients in group B and six patients in group C. Average ICD follow-up times were 42, 48 and 6 months for groups A, B and C, respectively. Eight appropriate ICD therapies occurred in 3 patients (27%). In group A, there were four sustained VT episodes successfully converted with an anti-tachycardia pacing (ATP) in two patients (67%); the average time to first therapy was 33 months. In group B, there was one appropriate shock for ventricular fibrillation (VF), and three sustained VT episodes in one patient (50%), time to first therapy was 60 months. After six months follow-up, there were no appropriate therapies registered in group C. Two patients (18%) experienced inappropriate shocks due to sinus tachycardia, one in group A and one in group C. Conclusion One-fifth of FLNC truncation carriers in our cohort experienced SCD. When patients received an ICD according to the current guidelines, majority experienced appropriate ICD therapy. Further clinical studies with longer follow-up will be needed to define appropriate risk stratification and optimal timing for prophylactic ICD intervention in these patients.

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Vincenzo Riverso ◽  
Antonio Curcio ◽  
Alessia Tempestini ◽  
Emilia De Luca ◽  
Sabrina La Bella ◽  
...  

Abstract Aims Complications of acute myocardial infarction (MI) can be life-threatening leading to sudden cardiac death. While guidelines recommend prompt revascularization and prolonged intensive care hospitalization, predictors of major adverse cardiovascular outcomes are yet poorly understood. The role of implantable cardioverter-defibrillators, even in cases of non-sustained arrhythmias is still debated. To date, it is unknown how to follow-up patients with mild cardiac dysfunction after MI. Implantable cardiac monitors (ICMs) can be helpful for stratifying patients in the early discharge period, and remote monitoring might speed up arrhythmia recognition and treatment. We investigated the role of remote monitoring of ICMs to detect arrhythmic events in post-MI patients without overt cardiac dysfunction. Methods and results We enrolled 13 patients (9 males; 69.8 years) after either ST-segment (N = 7) or non-ST-segment elevation (N = 6) MI with a left ventricular ejection fraction (LVEF) >35%, admitted to our coronary care unit for urgent revascularization between September 2019 and September 2021. Twelve patients underwent percutaneous myocardial revascularization, whereas one was treated with medical therapy only. All patients received an ICM during hospitalization according to echo and EKG parameters. We considered LVEF ≤ 40% as sole risk factor or LVEF between 40% and 50% in addition to either PQ length prolongation, or QRS widening, or pathologic heart rate variability, or non-sustained ventricular tachycardia/paroxysmal advanced second degree atrioventricular block. Patients with multiple revascularization procedures and several hospital admissions were excluded. Implanted ICM were frequently monitored both remotely and in-office when required. During follow-up, brady- and tachy-arrhythmias were recorded in four patients (30.8%). The remote monitoring of the ICM documented new-onset atrial fibrillation, high-degree atrioventricular block, severe bradycardia, and sustained ventricular tachycardia. Three patients required hospitalization and upgrade of the implanted device with pacemakers and cardioverter/defibrillator. For arrhythmic risk stratification, patients were divided into two subgroups; group A included patients with LVEF 40% associated with heart rate > 60 b.p.m., PQ length >160 ms and QRS width >86 ms (N = 4); group B included patients with EF 41%/50%, PQ length <159 and QRS width <85 ms (N = 10). First group experienced more advanced rhythm disorders than group B (P < 0.05). Device implantation was significantly higher in group A (P < 0.05%). Conclusions OFF-label implementation of ICMs coupled with remote device monitoring may be effective for early detection of serious adverse cardiac rhythm alterations in patients after MI and LVEF higher than 35%. Further monitoring is ongoing for assessing the occurrence of multiple arrhythmias or their increased occurrence.


2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Antonio Leon-Justel ◽  
Jose I. Morgado Garcia-Polavieja ◽  
Ana Isabel Alvarez-Rios ◽  
Francisco Jose Caro Fernandez ◽  
Pedro Agustin Pajaro Merino ◽  
...  

Abstract Background Heart failure (HF) is a major and growing medical and economic problem, with high prevalence and incidence rates worldwide. Cardiac Biomarker is emerging as a novel tool for improving management of patients with HF with a reduced left ventricular ejection fraction (HFrEF). Methods This is a before and after interventional study, that assesses the impact of a personalized follow-up procedure for HF on patient’s outcomes and care associated cost, based on a clinical model of risk stratification and personalized management according to that risk. A total of 192 patients were enrolled and studied before the intervention and again after the intervention. The primary objective was the rate of readmissions, due to a HF. Secondary outcome compared the rate of ED visits and quality of life improvement assessed by the number of patients who had reduced NYHA score. A cost-analysis was also performed on these data. Results Admission rates significantly decreased by 19.8% after the intervention (from 30.2 to 10.4), the total hospital admissions were reduced by 32 (from 78 to 46) and the total length of stay was reduced by 7 days (from 15 to 9 days). The rate of ED visits was reduced by 44% (from 64 to 20). Thirty-one percent of patients had an improved functional class score after the intervention, whereas only 7.8% got worse. The overall cost saving associated with the intervention was € 72,769 per patient (from € 201,189 to € 128,420) and €139,717.65 for the whole group over 1 year. Conclusions A personalized follow-up of HF patients led to important outcome benefits and resulted in cost savings, mainly due to the reduction of patient hospitalization readmissions and a significant reduction of care-associated costs, suggesting that greater attention should be given to this high-risk cohort to minimize the risk of hospitalization readmissions.


Author(s):  
Rory Hachamovitch ◽  
Benjamin Nutter ◽  
Manuel D Cerqueira ◽  

Background . The use of implantable cardiac defibrillators has been associated with improved survival in several well-defined patient (pt) subsets. Its utilization for primary prevention in eligible pts, however, is unclear. We sought to examine the frequency of ICD implantation (ICD-IMP) for primary prevention in a cohort prospectively enrolled in a prospective, multicenter registry of ICD candidates. Methods . We identified 961 pts enrolled in the AdreView Myocardial Imaging for Risk Evaluation in Heart Failure (ADMIRE-HF) study, a prospective, multicenter study evaluating the prognostic usefulness of 123I-mIBG scintigraphy in a heart failure population. Inclusion criteria limited patients to those meeting guideline criteria for ICD implantation; these criteria included left ventricular ejection fraction ≤35% and New York Heart Association functional class II-III. We excluded pts with an ICD at the time of enrollment, leaving a study cohort of 934 patients. Pts were followed up for 24 months after enrollment. Pts undergoing ICD-IMP after enrollment for secondary prevention were censored at the time of intervention. The association between ICD-IMP utilization and demographic, clinical, laboratory, and imaging data was examined using Cox proportional hazards analysis (CPH). Results . Of 934 pts, 196 (21%) were referred for ICD-IMP over a mean follow-up of 612±242 days. Implantations occurred 167±164 days after enrollment. Patients referred for ICD were younger (61±12 vs. 63±12), but did not differ with respect to proportion female (17% vs. 21%), African-American race (12% vs. 15%), diabetics (37% vs. 36%) (All p=NS). The frequency of ICD-IMP did not differ as a function of age, race, sex, LVEF, or imaging result (All p=NS). CPH revealed that a model including age, race, sex, diabetes, smoking, BMI, NYHA class, hypertension, heart failure etiology, and prior MI identified none of these as predictive of ICD-IMP. Conclusion: This analysis of prospective registry data reveals that in patients who are guideline-defined candidates for ICD-IMP, only about one in five receive an ICD over a two year follow-up interval. Multivariable modeling failed to identify any factor associated with ICD use.


2007 ◽  
Vol 25 (23) ◽  
pp. 3525-3533 ◽  
Author(s):  
Melinda L. Telli ◽  
Sharon A. Hunt ◽  
Robert W. Carlson ◽  
Alice E. Guardino

Purpose To assess the spectrum and reversibility of the cardiotoxicity observed in the adjuvant trastuzumab trials. Design The design and efficacy of the major adjuvant trastuzumab trials was assessed, including the National Surgical Adjuvant Breast and Bowel Project (NSABP) B-31, North Central Cancer Treatment Group N9831, Herceptin Adjuvant, Breast Cancer International Research Group 006, and Finland Herceptin trials. The cardiotoxicity data were evaluated with a focus on the follow-up cardiac evaluations of women who were diagnosed with cardiotoxicity. Proposed mechanisms of trastuzumab-related cardiotoxicity were considered. The natural history of congestive heart failure (CHF) was reviewed with the goal of placing the trastuzumab experience in context. Results Up to 4% of patients enrolled onto the adjuvant trastuzumab trials experienced severe CHF during treatment. In these trials, early stopping rules that identified an unacceptable level of cardiotoxicity were never reached. Despite this, a large number of patients on these trials experienced some form of cardiotoxicity that ultimately required discontinuation of trastuzumab. Approximately 14% of patients in the NSABP B-31 trial discontinued trastuzumab because of asymptomatic decreases in left ventricular ejection fraction (LVEF). Results of follow-up cardiac evaluations of patients diagnosed with any degree of cardiotoxicity in the NSABP B-31 trial document that a clinically significant proportion of patients have sustained decrements in their LVEF to less than 50%. Conclusion Adjuvant trastuzumab provides substantial benefits to patients with human epidermal growth factor receptor 2–positive breast cancer, however, competing immediate and long-term cardiovascular risks are a great concern. Continued cardiac follow-up of these women is of critical importance.


2021 ◽  
Vol 7 (5) ◽  
pp. 3087-3092
Author(s):  
Youlin Fu ◽  
Zhongming Yang ◽  
Chongrong Qiu

This paper investigates the effect of rehabilitation training on the clinical outcome and prognosis of patients with acute myocardial infarction after coronary artery intervention. There was no significant difference in daily living ability score and left ventricular ejection fraction between group A before intervention (P>0.05). The score of daily living ability of group A was (76.58±3.27) significantly higher than that of group B after intervention (73.7). ±3.4) (P<0.05); left ventricular ejection fraction after intervention (55.75±4.4) was significantly higher than that of group B (52.41 ±4.19) (P<0.05); total satisfaction rate of patients in group A (93.02%) was significantly higher In group B (69.77%), the difference between the groups was statistically significant (P<0.05); the total incidence of adverse reactions and mortality in group A (11.63%, 2.33%) was significantly lower than that in group B (53.49%, 16.28%).), the difference was statistically significant (P < 0.05). In patients with acute myocardial infarction, after interventional coronary artery intervention, immediate intervention with rehabilitation training can improve left ventricular ejection fraction, improve daily living ability and nursing satisfaction, and reduce postoperative adverse reactions and death. Medical staff should be used reasonably in the clinic according to the actual situation of the patient.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Yoshimori An ◽  
Kenji Ando ◽  
Michio Nagashima ◽  
Masato Fukunaga ◽  
Kenichi Hiroshima ◽  
...  

Background: There are still limited data on the mortality for a long-term follow-up and the clinical factors influencing appropriate therapies in Japanese patients with implantable cardioverter-defibrillator (ICD) for primary prevention, who satisfied the criteria in Multicenter Automatic Defibrillator Implantation Trial 2 (MADIT2). Methods: Between January 2000 and December 2012, a total of 436 patients without prior ventricular arrhythmic event underwent ICD implantation for primary prevention at our institution. Among these patients, we enrolled consecutive 122 patients (69±10 years, male: 84%, biventricular-pacing: 54%, median follow-up: 1390 days) who met the MADIT2 criteria; left ventricular ejection fraction (LVEF) ≤30% with ischemic heart disease, more than 4 weeks after myocardial infarction. Results: At the 3 years of follow-up, the mortality rate (21%) was comparable with that of the original MADIT2 ICD group (20%). The Kaplan-Meier event rate for appropriate ICD therapy (shock and anti-tachycardia pacing therapy) (35%) was also similar to that of the original MADIT2 ICD group (32%). Multivariate analysis by Cox regression model revealed that left ventricular diastolic diameter (LVDd) ≥60mm (Hazard Ratio [HR]: 1.65, 95% Confidence Interval [CI]: 1.16-2.14, P=0.004) and non-sustained ventricular tachycardia (NSVT) (HR: 1.55, 95%CI: 1.13-2.15, P=0.007) were independent predictors for appropriate ICD therapy. On the other hand, LVEF, NYHA class, biventricular-pacing, amiodarone or inducibility of ventricular arrhythmia was not associated with appropriate ICD therapy. Conclusion: Appropriate ICD therapy was delivered in Japanese primary prevention patients as often as in the original MADIT2 ICD group and strongly predicted by dilated left ventricle and NSVT.


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.


1982 ◽  
Vol 53 (2) ◽  
pp. 380-383 ◽  
Author(s):  
C. Foster ◽  
D. S. Dymond ◽  
J. Carpenter ◽  
D. H. Schmidt

Sudden strenuous exercise (SSE) has been shown to produce ischemic electrocardiographic (ECG) responses, abnormalities of myocardial blood flow, and decreases in left ventricular ejection fraction. Prior exercise taken as warm-up has been shown to ameliorate the ECG and myocardial blood flow abnormalities induced by SSE. The purpose of this study was to determine whether warm-up would normalize the responses of the left ventricular ejection fraction to SSE. Twenty healthy male volunteers performed SSE (400-W bicycle exercise) either with (group A, n = 10) or without (group B, n = 10) warm-up. Ejection fraction was measured using first-pass radionuclide angiography under control conditions and during SSE. During SSE ejection fraction decreased from control values in both group A (70.5 +/- 6.3 to 64.8 +/- 8.2%) and group B (70.3 +/- 10.1 to 57.7 +/- 7.7%), although ejection fraction was significantly higher during SSE in group A. The results are consistent with the hypothesis that the abnormal responses to SSE are attributable to subendocardial ischemia secondary to a delay in autoregulation of myocardial blood flow. However, the decrease in ejection fraction during SSE even following warm-up suggests that the mechanism for the abnormal response to SSE is more complicated than previously hypothesized.


2016 ◽  
Vol 72 (3) ◽  
Author(s):  
Laura Crespi ◽  
Monica Bosco ◽  
Naika Scalabrino ◽  
Massimo Baravelli ◽  
Anna Picozzi ◽  
...  

Background. Patients following major cardiac surgery are increasingly elderly and present many comorbidities. For these reasons their post-operative phase is often burdened by several complications requiring a long stay in Critical Care and prolonged mechanical ventilation. Most of these patients, when transferred to our Intensive Cardiac Rehabilitation Unit, still have a percutaneous tracheostomy due to respiratory mechanical dysfunction. The aim of our work is to present new rehabilitative care strategies in such compromised patients. Methods and materials. We studied 27 elderly critically ill tracheostomized patients who were split into 2 Groups (A = 11 and B = 16). The Groups were homogeneous for age and for left ventricular ejection fraction. Group A received a standard treatment including cautious mobilisation and respiratory unspecific physiotherapy. Group B received an earlier and more aggressive treatment with a specific respiratory physiotherapy including Positive Expiration Pressure (PEP) directly connected to the tracheostomy cannula. A protocol for tracheostomy decannulation by assessment of the Peak Expiratory Flow during cough (PCEF≥ 180 L/min.) has been defined in order to verify the patients ability to develop a mechanically effective cough to obtain weaning from tracheostomy. Besides, in the patients of Group B, we carried out a screening of the swallowing dysfunction. Results. Four patients of Group A deceased while in Group B there were no deaths. Furthermore patients of Group B showed a statistically significant improvement of mobility and respiratory indexes. In Group B only one patient was discharged with tracheostomy cannula in site because he did not reach standard criteria for decannulation and his PCEF value was not satisfactory. This patient underwent percutaneous gastrostomy. Conclusions. A precocious and intensive rehabilitation, based on specific respiratory physiotherapy, significantly improves mobility and respiratory indexes of patients with tracheostomy. The PCEF and the swallowing deficit evaluation allows an earlier tracheostomy decannulation with lower risk of complications.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Tavernese ◽  
V Cammalleri ◽  
A Sanseviero ◽  
P De Vico ◽  
S Muscoli ◽  
...  

Abstract Background Chronic kidney disease (CKD) has been shown to impact negatively the prognosis of patients with heart failure, coronary artery or valvular heart disease and emerged as predictor of poor outcomes in mitraclip population. Purpose Aim of our study was to evaluate three-year echocardiographic outcomes in CKD patients with severe mitral regurgitation (MR) treated with mitraclip. Methods This in an observational study including patients treated with mitraclip in our institution, who completed three years of follow up. Patients population was divided into two groups according to basal creatinine clearance (CrCl): group A, including patients with normal/mild decline of renal function (CrCl &gt; 60 ml/min) and group B, including patients with CKD (CrCl &lt; 60 ml/min). Demographic and procedural characteristics were compared, as well as echocardiographic data, including grade of MR, left ventricular ejection fraction (LVEF), mean transmitral gradient and systolic pulmonary artery pressure (sPAP). Kaplan-Meier survival curves were obtained. Results The study population consists of 107 patients (mean age 71 ± 9 years, 69% male): 57 belonging to group A and 50 to group B. Patients of group B had higher values of LogEuroScore (22 ± 10 vs.15 ± 9 p = 0,0002), systemic hypertension (92% vs. 74%, p = 0,026), complicated diabetes (46% vs. 24% p = 0,034) and NYHA IV before the procedure (24% vs 9 %, p = 0,059). Additionally, patients of group B had lower baseline LVEF (35 ± 11 vs. 41 ± 13; p = 0,012). Procedural success was similar between the two groups without significant difference in degree of MR reduction after mitraclip implantation. Echocardiographic follow-up showed that in group B, the LVEF did not improve after the treatment (more than 50% had LVEF &lt; 35% at 1,2 and 3 years) while in the group A it improved significantly (LVEF &lt; 35% from 47,6% at discharge to 29%, 32% and 31% at 1, 2 and 3 years, respectively). In comparison to group A, in group B a progressive increase in residual MR grade was observed (moderate-to-severe MR from 2% at discharge to 14%, 15%, and 27% at 1, 2 and 3 years, respectively) as well as in the mean transmitral gradient (from 3,90 ±1,6 mmHg after the mitraclip implantation to 5,28 ± 1,7; 5,73 ± 1,75; 6,06 ±1,75 at 1, 2 and 3 years, respectively) and sPAP (from 47 ± 12 mmHg at discharge to 49 ± 21; 51 ± 20; 48 ± 22 at 1, 2 and 3 years, respectively). Kaplan Meier estimate of survival free from in-hospital readmission was 77% in group A and 61% in group B (Log-Rank 4.563, p = 0,033) and survival free from cardiovascular death was 95% and 81,5%, in group A and B, respectively (Log-Rank 4.806, p = 0,028). Conclusion Our results suggest that CKD patients have poorer outcomes after mitraclip implantation with worsening of some echocardiographic parameters, particularly for residual MR degree, mean transmitral gradient and sPAP, without improvement in LVEF at one, two and three years of follow-up.


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