bluuebunny/crossref_metadata_2025_split huggingface dataset:
1266Novel formin homology 2 domain containing 3 (FHOD3) mutations associated with the pathogenesis of hypertrophic cardiomyopathy (HCM) in an Irish population
Authors: Carron J , O"brien J , Gallagher M , Mcgorrian C , Galvin J 
Published: June 2020

Abstract

Background
The genetic cause of hypertrophic cardiomyopathy (HCM) remains unexplained in a substantial proportion of cases. Recent large sequencing studies suggest that, though not previously implicated, FHOD3 (a Formin protein responsible for sarcomere assembly) may have a role in the pathogenesis of HCM, particularly variants affecting a conserved small coil-coiled domain (amino acids 622 to 655).

Aim
To investigate the relationship between novel FHOD3 mutations, previously classified as variants of uncertain significance (American College of Medical Genetics ACMG Class III), and the development of HCM.

Methods
A single center review of HCM probands carrying mutations in the FHOD3 gene was conducted. Existing HCM patients from the family heart screening clinic database were retrospectively reviewed. Frequency of FHOD3 mutations, segregation among family members and clinical characteristics of patients were reviewed. All genetic analysis was conducted via the same internationally validated next generation sequencing lab.

Results
Of 367 HCM probands identified in our center, 9 (2.45%) were found to have ACMG Class III mutations affecting the FHOD3 gene. Five of these 9 probands (56%) displayed the same p.Arg637Gln mutation, while the remaining 4 (44%) carry the same p.Ile648Thr residue alteration. Both of these are rare mutations, found to be present in &lt;1% of controls in previous large sequencing studies and not previously reported. Among probands with the p.Ile648Thr mutation, co-segregation was confirmed in one family, another first-degree relative in the same family having suffered a sudden cardiac death (HCM confirmed on autopsy). In those with the p.Arg367Gln mutation, a strong family history was observed in two separate families, 4 first-degree members having a confirmed HCM diagnosis. Carriers of both mutation types displayed varying degrees of disease severity with rates of non-sustained ventricular tachycardia and device implantation similar to other HCM cohorts (∼40%).

Conclusions
FHOD3 is a novel gene, recently implicated in the pathogenesis of HCM, though not previously well described in the literature. Carrier frequency of FHOD3 mutations in the HCM population in our center is similar to that described in recent publication (1-2%), and all carry variants affecting the suspected small coil-coiled domain (amino-acids 622-625).

Abstract Figure.
P505Short and long-term outcomes of medical management of patients with a first episode of arrhythmic storm
Authors: Acosta Martinez J , Caballero Valderrama M , Dominguez Cano J L , Campos Pareja A , Frutos Lopez M , Garcia Riesco L , Arana Rueda E , Pedrote Martinez A 
Published: June 2020

Abstract

INTRODUCTION
There is few data about long-term outcomes of conservative management (without catheter ablation) of patients with a first episode of arrhythmic storm (AS) in the current context. This study analyzes the short and long-term outcomes of implantable cardioverter defibrillator (ICD) patients with a first episode of AS receiving non-interventional management.

METHODS
Consecutive patients admitted with a first episode of AS between January 2008 and June 2019 receiving medical management without catheter ablation were included. AS was defined as ≥ 3 appropriate ICD therapies occurring during a 24h span. Medical management included: correction of triggers, sedation/mechanical ventilation, antiarrhythmic drugs, ICD reprogramming and neuraxial modulation. Baseline clinical characteristics and follow-up data were recorded. All patients were followed every 6 months at the ICD office. The primary end-point was all-cause mortality.

RESULTS
60 patients (81% male, 62.8 ± 16.2 years, 43% ischaemic, LVEF 35.4 ± 14%) with a first episode of AS treated conservatively were included. Thirty-day survival was 96.5% and 1-year survival was 82%. During a median (interquartilic range) follow-up of 31 (6-69) months 31 (51.7%) patients died (51.6% due to cardiovascular aetiology) and 35 (58.3%) patients were readmitted (48.5% due to recurrent arrhythmic events and 45.7% due to heart failure). Age [HR 1.05 (95% confidence interval: 1.01-1.08)] and end-diastolic diameter [HR 1.05 (95% confidence interval: 1-2)] were the strongest independent predictors of all-cause mortality.

CONCLUSION
Despite the severity of this entity, medical management (without catheter ablation) of a first episode of AS is reasonable given its good 30-day and 1-year survival. However, a high rate of AS recurrence and readmissions are observed during long-term follow-up. Efforts are needed in order to identify those patients with a first episode of AS that could benefit from an early catheter ablation strategy.
P490Time boundaries of three-phase time-sensitive model for ventricular fibrillation cardiac arrest
Authors: Goto Y , Funada A , Maeda T , Okada F , Goto Y 
Published: June 2020

Abstract

Funding Acknowledgements
Japan Society for the Promotion of Science (KAKENHI Grant No. 18K09999)

Background
Recent clinical evidence has suggested that the pathophysiology of ventricular fibrillation (VF) cardiac arrest may consist of three time-sensitive phases, namely electrical, circulatory, and metabolic. According to this model of cardiopulmonary resuscitation (CPR), the optimal treatment of cardiac arrest is phase-specific. The potential survival benefit of bystander cardiopulmonary resuscitation (BCPR) depends in part on ischemic time (i.e., the collapse-to-shock interval), with the greatest benefit occurring during the circulatory (second) phase. However, the time boundaries between phases are not precisely defined in the current literature.

Purpose
The purpose of the present study was to determine the time boundaries of the three-phase time-sensitive model for VF cardiac arrest.

Methods
We reviewed 20,741 adult patients with initial VF after witnessed out-of-hospital cardiac arrest from a presumed cardiac origin who were included in the All-Japan Utstein-style registry from 2013 to 2017. We excluded patients who underwent bystander defibrillation prior to arrival of emergency medical services personnel. The study end point was 1-month neurologically intact survival (Cerebral Performance Category scale 1 or 2). Collapse-to-shock interval was defined as the time from collapse to first shock delivery by emergency medical services personnel. Patients were divided into two groups, BCPR (n = 11,606, 56.0%) and non-BCPR (n = 9135, 44.0%), according to whether they had received BCPR or not.

Results
The rate of 1-month neurologically intact survival in the BCPR group was significantly higher than that in the non-BCPR group (27.9% [3237/11,606] vs 17.9% [1632/9135], P &lt; 0.0001; adjusted odds ratio [OR], 1.90; 95% confidence interval [CI], 1.75–2.07; P &lt; 0.0001). Overall, increased collapse-to-shock interval was associated with significantly decreased adjusted odds of 1-month neurologically intact survival (adjusted OR for each 1-minute increase, 0.94; 95% CI, 0.93–0.95; P &lt; 0.0001). In the BCPR group, the ranges of collapse-to-shock interval that were associated with increased adjusted 1-month neurologically intact survival were from 7 minutes (adjusted OR, 1.95; 95% CI, 1.44–2.63; P &lt; 0.0001) to 17 minutes (adjusted OR, 2.82; 95% CI, 1.62–4.91; P = 0.0002) as compared with those in the non-BCPR group. However, the increase in neurologically intact survival of the BCPR group became statistically insignificant as compared with that of the non-BCPR group when the collapse-to-shock interval was outside these ranges.

Conclusions
The above-mentioned findings suggest that the time boundaries of the three-phase time-sensitive model for VF cardiac arrest may be as follows: electrical phase, from collapse to &lt;7 minutes; circulatory phase, from 7 to 17 minutes; and metabolic phase, &gt;17 minutes onward from collapse.
P1353Echocardiographic predictor of sick sinus syndrome following catheter ablation of persistent atrial fibrillation
Authors: Yoshie K , Okada A , Seki S , Tabata H , Shoin W , Kobayashi H , Okano T , Motoki H , Shoda M , Kuwahara K 
Published: June 2020

Abstract

Funding Acknowledgements
Abbott Medical Japan, Medtronic Japan, Boston Scientific Japan, Biotronic Japan, Japan Life Line

Background / Introduction
Although sick sinus syndrome(SSS) can be associated with atrial fibrillation(AF), predictive factors of SSS following catheter ablation(CA) of persistent atrial fibrillation(perAF) are not well known.

Purpose
We investigated echocardiographic parameters to predict occurrence of SSS after restoration of sinus rhythm by CA for perAF patients.

Methods
Ninety-eight consecutive perAF patients from June 2014 to May 2018 treated with CA were retrospectively reviewed. Twelve patients(12%, SSS group) developed SSS after successful CA and 86 patients(88%, non-SSS group) did not. Baseline characteristics, blood exam, and echocardiographic findings(RA area size, LA area size, EF, etc) before AF CA were analyzed using Student’s t test, Mann-Whitney test, Chi-square test and Univariate analysis. Multivariate logistic analysis was then performed using those parameters. The atrial area size was calculated from 4 chamber view at the atrial end- systole.

Results
The multivariate analysis for predictive factors of SSS is shown in the table. Right atrium(RA) area could predict SSS(17.3 ± 4.8cm2 vs. 14.7 ± 3.6cm2, odds ratio 1.468; 95% confidence interval 1.088 to 1.981, p = 0.012). Gender (female) was also an independent predictor(4/12 (33%) vs. 8/86 (9%), odds ratio 39.832; 95% confidence interval 2.589 to 612.938, p = 0.008). The other echocardiographical findings(LA area size, EF, etc), baseline characteristics and blood exam results were not related to SSS after successful CA of perAF patients.

Conclusions
The large RA area size and gender (female) could predict SSS in perAF patients after restoration of sinus rhythm by successful CA. We may need to inform possible SSS after CA to female patients with a large RA before CA.
Multivariate Logistic analysis Total(N = 98) SSS group (N = 12) Non SSS group (N = 86) Odds ratio 95% CI P-value Age 64(58-69) 68(60-72) 63(57-69) 1.032 0.929-1.145 0.560 Gender/Female 12(12%) 4(33%) 8(9%) 39.832 2.589-612.938 0.008 CKD 27(28%) 6(50%) 21(24%) 1.264 0.179-8.945 0.814 BNP 91(53-180) 206(167-304) 82(48-169) 1.003 0.993-1.012 0.609 RDW 45.1 ± 3.9 46.4 ± 4.8 44.9 ± 3.8 1.242 0.971-1.588 0.085 RA area 15.1 ± 3.8 17.3 ± 4.8 14.7 ± 3.6 1.468 1.088-1.981 0.012 LA area 24.2(17.0-24.9) 24.4(17.7-26.3) 24.1(16.8-24.4) 0.967 0.803-1.165 0.726 Right atrium area and gender were the independent predictor of SSS in persistent atrial fibrillation patients after restoration of sinus rhythm
P1417Short and long term efficiency of different therapeutic approaches to perimitral atrial flutter
Authors: Kupo P , Pap R , Bencsik G , Saghy L 
Published: June 2020

Abstract

Introduction
Catheter ablation of perimitral flutter can be challenging, owing to difficult anatomy. The most commonly applied procedure is the creation of a mitral isthmus line (between the lower left pulmonary vein and the mitral anulus) or an anteroseptal line (between the upper right pulmonary vein and the anterior mitral anulus).

Purpose
Our study aimed to compare the short and long term efficacy of two different ablation methods.

Methods
In our retrospective study 45 consecutive patients diagnosed with perimitral flutter were included between 2009 and 2018.

Results
Radiofrequency ablation was performed in 48 cases in 31 patients (mitral isthmus line (n = 25, 52.1%); anteroseptal line (n = 23, 47.9%)). Arrhythmia-termination and sinus rhythm restoration could be achieved in 64.6% of the cases (mitral isthmus line: 16/25 (64.0%), anteroseptal line: 15/23 (65.2%). Comparing two different techniques, there was no significant difference (p = 0.85) in acute success rates. During 24.3 months of follow-up period, in 60.0% of the patients no recurrence occurred. The arrhythmia recurred in 6 cases (40.0%) after anteroseptal line ablation, and in 8 cases (53.3%) after mitral isthmus line ablation. No difference was found in the long term efficiency of two different ablation techniques (p = 0.211).

Conclusion
In our retrospective study we found no significant difference in the short and long term efficiency of two different therapeutic approaches to perimitral atrial flutter.
P1431Radiofrequency ablation of left atrium in patients with mitral valve replacement. The future of surgical treatment for atrial fibrillation in mitral valve disease?
Authors: Utyasheva A , Abdulyanov I V , Sungatullin M A , Vagizov I I 
Published: June 2020

Abstract

Aims
The evaluation of sinus rhythm preservation after surgical treatment of atrial fibrillation by left-atrial RFA in patients with mitral valve prosthetics.

Materials and methods
A prospective, randomized study was performed in the interregional clinical and diagnostic center of Kazan in 2011-2018 which were included 136 patients with mitral valve pathology and persistent AF. The average age of patients was 57 ± 5, the gender division was dominated by female patients and made up 97 (58.4%).
All patients underwent either mitral valve prosthesis with mechanical prosthesis Meding-2 which made up 96 (70.5%) or biological prosthesis Carpantier-Edwards which made up 40 (29.4%).
Radiofrequency isolation of the left atrium and pulmonary veins was performed by standard procedure by Cox-IV which doesn’t include the right atrium tissue.
Results and discussion: In the early post surgical period, recovery of sinus rhythm was observed in 111 (81.6%) patients. Cardiac Pacemaker Implantation was performed in 1 (0.73%) case. The results of the study were assessed after surgical treatment in 22 ± 5 months, the sinus rhythm was preserved in 99 patients from the study group and made up 72.7%.

Conclusion
Radiofrequency ablation the left atrium provides long-term preservation of sinus rhythm in the treatment of patients with atrial fibrillation and mitral valve pathology.
P472Cerebral thromboembolic risk in atrial fibrillation ablation: a direct comparison of vitamin K antagonists versus non-vitamin K-dependent oral anticoagulants
Authors: Petzl A M , Derndorfer M , Kollias G , Moroka K , Aichinger J , Martinek M , Puererfellner H 
Published: June 2020

Abstract

Aims
Cerebral thromboembolic events are well-known complications of pulmonary vein isolation (PVI) and can manifest as stroke or silent cerebral embolic lesions. Over the last years, the preferred oral anticoagulation in atrial fibrillation (AF) shifted from vitamin K antagonists (VKA) to non-vitamin K-dependent oral anticoagulants (NOAC). The aim of this study was to compare the incidence of cerebral embolic lesions after AF ablation in patients on VKA versus patients on NOAC, and to identify corresponding clinical and procedural risk factors.

Methods
A total of 421 patients undergoing PVI (by radiofrequency catheter or cryoballoon) were prospectively included into the study. Of these, 43.7% were on VKA and 56.3% on NOAC treatment. In the NOAC group 38% of patients had an interruption of anticoagulation for 24-36 hours. All patients underwent pre- and postprocedural cerebral magnetic resonance imaging.

Results
Periprocedural cerebral lesions occurred in 13.1% overall. Of these, three (0.7%) resulted in symptomatic cerebrovascular accidents. Incidence of cerebral lesions was significantly higher in patients on NOAC compared to VKA (16% vs. 9.2% respectively, p = 0.04), as well as in patients that had intraprocedural cardioversions compared to no cardivoersions (19.5% vs. 10.4% respectively, p = 0.03). In multivariate analysis both parameters were found to be independent risk factors for cerebral embolism. No significant difference between interrupted and uninterrupted NOAC administration could be detected.

Conclusions
In patients undergoing AF ablation, we identified the use of NOAC and intraprocedural cardioversion as independent risk factors for the occurrence of periprocedural cerebral embolic lesions.
Abstract Figure. Incidence of cerebral embolic lesions
P521Conversion test during Subcutaneous Implantable Cardioverter-Defibrillator Implantation in clinical practice: in-hospital and mid-term outcome
Authors: Bianchi V , Diemberger I , Tavoletta V , Perrotta L , Ottaviano L , Migliore F , Francia P , Ammendola E , De Bonis S , Ferrari P , Dello Russo A , Palmisano P , Salzano G , Lovecchio M , Viani S 
Published: June 2020

Abstract

Funding Acknowledgements
No funding

OnBehalf
RHYTHM DETECT Registry

Background
With subcutaneous implantable cardioverter–defibrillators (S-ICD), conversion test (CT) is still recommended at implantation. However, prior works found that adherence to this recommendation is declining in clinical practice.

Purpose
To describe current practice regarding CT at S-ICD implantation, and also measure in-hospital outcome of patients who underwent CT and mid-term outcome of patients without CT.

Methods
We analyzed 1652 consecutive patients (49 ± 15 years old, 80% male, 51% with ischemic or non-ischemic dilated cardiomyopathy, 45% with ejection fraction ≤35%) who underwent S-ICD implantation in 60 Italian centers from 2013 to 2019.

Results
CT data were missing in 27 patients. CT was performed in 1300 patients. Successful conversion with ≤65J was obtained in 97.4% of patients. Shock at 80J was not effective in 12 (0.9%) patients. In 10 of these patients the CT was successful after device repositioning, while in 2 patients it was decided to implant a transvenous ICD. Two (0.15%) episodes of electromechanical dissociation (1 fatal) were reported as consequence of CT. CT was not performed in 325 patients (for clinical reasons in 182 patients, for facility preference in 71, ventricular fibrillation not inducible in 72 patients). As compared to the CT group, these patients were older (51 ± 16 vs. 48 ± 15 years; p &lt; 0.01) and had lower ejection fraction (37 ± 16% vs. 46 ± 16%; p &lt; 0.01). 243 non-CT patients had at least 6 months follow-up (median 15 months). In this group, 12 (4.9%) patients had appropriate shocks to treat VT/VF (all successfully terminated with the first shock), and 9 (3.7%) patients had inappropriate shocks.

Conclusions
This analysis showed that CT is frequently omitted in current clinical practice, especially in older patients with worse systolic function. Shocks at CT are very frequently effective and system revision after CT is rarely required. CT is also safe, although serious adverse events cannot be excluded. A strategy that omits CT did not appear to compromise the effectiveness of the S-ICD, but larger populations and longer follow-up are needed to confirm this finding.
P1181Lead dependent tricuspid dysfunction- mechanism and treatment
Authors: Polewczyk A , Nowosielecka D , Tomaszewski A , Brzozowski W , Szczesniak-Stanczyk D , Polewczyk M , Jachec W , Kutarski A 
Published: June 2020

Abstract

Background
Lead dependent tricuspid dysfunction (LDTD) is a hardly recognizable severe complication of permanent cardiac pacing.

Purpose
Aim of the study was to assess the mechanism of lead dependent tricuspid dysfunction (LDTD) and effect of transvenous leads extraction (TLE) in theese patients.

Methods
We analyzed the clinical data of 3110 patients undergoing TLE from 01/2006 to 09/2019 in a single center. Echocardiographic assessment was performed in 2559 patients. LDTD mechanism and improvement of valve function after unblocking (by removing the lead) were analyzed.

Results
LDTD was recognized in 98 (7,3%) patients. The most common mechanism of LDTD was pulling up the leaflet of tricuspid valve by the lead. Improvement of tricuspid valve function was observed in all patients with recognized LDTD. 18 patients with slight improvement were directed to cardiosurgery after TLE.

Conclusions
LDTD should be considered in patients with implanted pacing system and severe tricuspid regurgitation. Transvenous leads extraction can improve function of tricuspid valve in theese patients.
Abstract Table
P1147Complications in young ICD patients - a retrospective analysis
Authors: Zweiker D , Puntus T , Egger F , Kriz R , Koch J , Hellebart P , Huber K , Nurnberg M 
Published: June 2020

Abstract

Introduction
In specific situations implantable cardioverter defibrillator (ICD) therapy is recommended for patients under the age of 40 years. Due to the active lifestyle of this patient population, complication rates in devices with conventional transvenous electrodes may be higher than for the remaining population.

Methods
The ICD-YOUNG study is a retrospective analysis of consecutive patients ≤ 40 years undergoing transvenous or subcutaneous ICD (s-ICD) implantation, device change or lead revision at our centre between July 2006 and December 2017. Rehospitalization for lead failure or device battery depletion was documented.

Results
Out of 586 patients undergoing ICD implantation, 35 patients (6.0%) were ≤ 40 years. Mean age was 30.0 ± 7.2 years, 48.6% were female, 37.1% received ICD therapy for primary prevention and 11.4% primarily received s-ICD. Median follow up was 7.3 (interquartile range, 1.8-12.0) years, with a lower follow up duration in s-ICD patients than conventional ICD patients (median, 2.9 vs. 9.0 years). Over the course of follow-up, 37.1% received successful anti-tachycardia therapy. 19.4% of patients in the conventional ICD group had right ventricular lead problems requiring intervention, while none of the s-ICD patients had to be revised. Time to first device change due to battery depletion and/or device upgrade was similar in young and remaining patients (median 5.4 vs 6.0 years, p = 0.23).

Discussion
Young patients requiring ICD have a high rate of lead problems. In most young patients, s-ICD therapy is an encouraging alternative to conventional ICD therapy with a lower lead failure rate.