bluuebunny/crossref_metadata_2025_split huggingface dataset:
Left ventricular involvement in arrhythmogenic right ventricular cardiomyopathy
Authors: Parashar N , Sinha M , Sharma S , Ramakrishnan S 
Published: November 2020

Abstract

Background
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is not an isolated RV disease. Left ventricular (LV) or biventricular involvement is being increasingly diagnosed in such patients. There is available research to show that the LV peak strain calculated from feature tracking (FT) cardiac MRI is impaired even before the impairment of function. If present, such involvement is an adverse prognostic marker.

Purpose
The aim of this study is to calculate LV involvement in patients with ARVC using FT cardiac MRI.

Methods
27 patients of ARVC who underwent cardiac MRI with late gadolinium enhancement (LGE) in our department were identified. 10 healthy volunteers (controls) were also assessed using non-contrast cardiac MRI. LV strain analysis was performed using Cvi42 Circle cardiovascular imaging software; and global LV peak radial, circumferential and longitudinal strain values were calculated. Patients were divided into two sub groups: those with preserved LV ejection fraction (LVEF) and those with reduced LVEF. Peak LV strain values were compared between the two groups of ARVC patients; and between ARVC patients with preserved LVEF and healthy volunteers.

Results
The LV peak global longitudinal strain (GLS), global circumferential strain (GCS) and global radial strain (GRS) were −17.7±2.47, −18.16±2.65, 31.04±6.07 respectively in healthy volunteers. LV global longitudinal (GLS), circumferential (GCS), and radial strain (GRS) were significantly impaired in ARVC/D patients (GLS: −11.97±4.34%, GCS: −14.35±4.32%, GRS: 22.1±7.39%). ARVC patients were divided into 2 subgroups: the preserved LV ejection fraction (LVEF) group (LVEF ≥55%, n=9) and the reduced LVEF group (LVEF &lt;55%, n=18). In ARVC patients with reduced LVEF, the peak GLS was −9.99±3.94, GCS was −12.88±4.08 and GRS was 19.57±7.56. With preserved LVEF these values were −15.1±3.02, −17.3±3.3 and 27.1±3.67 respectively. In ARVC patients with preserved LVEF, the peak LV strain were impaired when compared with healthy volunteers with significant difference in peak GLS in between the two groups (p=0.05).

Conclusion
In patients with ARVC, cardiac MRI feature tracking can detect early LV dysfunction and thus adverse prognostic marker. Even in patients with normal LVEF, GLS was found to be significantly impaired as compared to healthy controls.
Calculation of longitudinal strain

Funding Acknowledgement
Type of funding source: None
A novel SCN5A gene mutation causing LQT3 with unique phenotype in a large canadian kindred
Authors: MacIntyre C.J , Gray C.J , Newman V , Crowley A , Dyack S , Gardner M.J 
Published: November 2020

Abstract

Background
SCN5A gene mutations are associated with diverse clinical phenotypes including Long QT (LQT) Syndrome, Brugada Syndrome, progressive conduction system disease and cardiomyopathy.

Objective
We identified a novel SCN5A variant (T731I) in a pedigree affected by QT prolongation and ventricular arrhythmia. Clinical phenotype and co-segregation with genotype within the pedigree were characterized.

Methods
Family members were invited to attend clinics held in their indigenous community. All subjects underwent a history, physical exam, 12 lead ECG and had blood samples collected for genetic testing. Patients were assessed by an adult or pediatric cardiologist and genetic counselors over a six year period. There were 86 females and 69 males ranging in age from 1 to 82 years (mean 36 years) at the time of first assessment. ECG analysis was performed by a cardiac electrophysiologist blinded to patient identity and genotype. The QTc was calculated using Bazett's formula.

Results
One hundred and fifty five family members were evaluated (86 (55%) female; mean age 36 years (range 1 to 82 years). Mean QTc was 461 + 21 ms for the gene positive group and 419 + 22 ms for the gene negative group (P&lt;0.001) (Figure). There was no significant difference in age or sex between the gene positive and gene negative groups.
Gene positive family members were also noted to have low right atrial p waves and frequent unifocal ventricular ectopy and non-sustained monomorphic ventricular tachycardia. One gene positive individual developed dilated cardiomyopathy with high burden ventricular tachycardia and required cardiac transplantation.

Conclusion
This novel SCN5A variant co-segregated with an abnormal phenotype consisting of significant QTc prolongation, low atrial rhythm, and ventricular arrhythmia. This pedigree highlights the phenotypic heterogeneity of SCN5A gene mutations and the importance of cascade family screening to identify at risk family members.
Figure 1. QTc intervals

Funding Acknowledgement
Type of funding source: None
Usefulness of subcutaneous Implantable Loop Recorder in Brugada Syndrome. A single UK centre experience
Authors: Scrocco C , Ben-Haim Y , Tome-Esteban M , Papadakis M , Sharma S , Behr E.R 
Published: November 2020

Abstract

Background
Experience with implantable loop recorder (ILR) in Brugada Syndrome (BrS) is limited.

Purpose
We sought to evaluate the usefulness of continuous monitoring in BrS patients in a UK based Inherited Cardiac Conditions centre.

Methods
Subjects with BrS were consecutively recruited and demographic, clinical, device and follow-up data acquired.

Results
Of 452 subjects with BrS recruited in our centre, 41 (9%) received an ILR. Twenty-one (51%) were males. Twenty-four subjects (59%) had a high probability of BrS based on the Shanghai score. Mean age at implantation was 45.3±15.5 years. The indications for the implantation were: syncope or pre-syncope in 28 subjects (68%), palpitations in 12 (29%), family history of SADS in 1 case. Sixteen subjects presented with a spontaneous type 1 pattern, of whom 12 (75%) underwent an electrophysiological study for risk stratification which was negative. During a mean follow-up of 82±46 months (range 4.0–182), 5 new diagnoses of atrial fibrillation (AF) or supraventricular tachycardia were made; 3 subjects had symptomatic pathological pauses (in one case associated with fast AF) and underwent permanent pacemaker or ICD implantation; one subject experienced an asymptomatic episode of fast, non-sustained monomorphic ventricular tachycardia. In 2 cases the device was explanted prematurely due to site infection. Patients who received an ECG diagnosis following interrogation of the ILR were older (mean age 55±11 vs 43±16m p=0.04), although there were no differences in sex or Shanghai score.

Conclusions
In a large UK cohort of BrS patients, the use of ILR was low. However, continuous subcutaneous monitoring yielded a diagnosis of tachy- or brady-arrhythmic episodes in 22% of cases, and therefore can be helpful in guiding the management of low/intermediate risk BrS patients.

Funding Acknowledgement
Type of funding source: Foundation. Main funding source(s): Robert Lancaster Memorial Fund
Novel insights on Andersen-Tawil syndrome type 1
Authors: Mazzanti A , Guz D , Trancuccio A , Pagan E , Chargeishvili T , Biernacka E.K , Sacilotto L , Zorio E , Brugada G , Nof E , Anastasakis A , Sansone V , Jimenez-Jaimez J , Cruz F , Priori S.G 
Published: November 2020

Abstract

Background
Andersen-Tawil Syndrome type 1 (ATS1) in a rare arrhythmogenic disease caused by loss-of-function mutations in the KCNJ2 gene and characterized by ventricular arrhythmias, dysmorphic features and episodes of periodic paralysis. Although the prognosis of ATS1 patients is typically considered benign, definitive outcome data are lacking.

Purpose
We aimed to: 1) define the risk of life-threatening arrhythmic events (LAEs); 2) identify risk factors for such events; 3) assess the efficacy of anti-arrhythmic drugs in preventing LAEs.

Methods
We included 118 ATS1 patients from 57 families with confirmed pathogenic or likely pathogenic KCNJ2 mutations. Clinical and genetical data were acquired by investigators from 23 centers in 9 countries.

Results
Baseline characteristics of the population are presented in the Table. Over a follow-up of 6.2 years, 17/118 (14%) patients experienced a first LAE, with a 5-year cumulative probability of 7.9% (Figure). Cox multivariable analysis demonstrated that a previous history of syncope (HR 4.5, p=0.02), the documentation of sustained VT (HR 9.3, p=0.001) and the administration of amiodarone (HR 268, p&lt;0.001) were associated with an increased risk of LAE. The baseline rate of LAE was not reduced by beta-blockers alone (1.37 per 100 py; p=1), or in combination with class Ic antiarrhythmic drugs (1.46 per 100 py, p=1).

Conclusions
Our data demonstrate that the clinical course of patients with ATS1 is characterized by a high rate of LAE. A history of unexplained syncope, and documentation of sustained ventricular tachycardia are independently associated with a higher risk of LAE. Amiodarone is proarrhythmic and should be avoided in ATS1 patients.
ATS1: Diagnosis, Outcome, Risk Factors

Funding Acknowledgement
Type of funding source: Public grant(s) – EU funding. Main funding source(s): ERN Guard-Heart European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart
Endomyocardial biopsy: what future in arrhythmogenic right ventricular dysplasia diagnosis?
Authors: Maragna R , Trombara F , Frappampina A , Dello Russo A , Gasperetti A , Catto V , Conte E , Vettor G , Sicuso R , Sommariva E , Natale A , Andreini D , Basso C , Tondo C , Casella M 
Published: November 2020

Abstract

Background
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a leading cause of sudden cardiac death, but its diagnosis is challenging and the role of endomyocardial biopsy (EMB) is controversial and has been recently questioned.

Purpose
We aimed to 1) analyse the role of EMB in improving the diagnostic performance of 2010 Task Force Criteria (TFC) in the diagnosis of ARVC; 2) assess EMB safety in our population.

Methods
We retrospectively analysed data from 54 consecutive patients admitted to our Hospital with a clinical suspicion of ARVC undergoing endomyocardial biopsy. During hospitalization a complete assessment was performed for every patient (including electrocardiogram, echocardiogram, cardiac MRI, genetic analysis, and electroanatomic-mapping-guided endomyocardial biopsy). ARVC diagnosis was assessed for every patient using both traditional 2010 TFC and a non-invasive modified TFC (2010 TFC criteria excluding biopsy).

Results
Overall, 9/54 (17%) patients showed a left-dominant variant of ARVC and were therefore excluded from the analysis. Non-invasive modified TFC allowed 16/45 (36%) patients to receive a definite diagnosis; when biopsy results were added the number of definite diagnosis increased to 22/45 (49%), increasing the number of patients with a definite diagnosis by 13%. More specifically: 8/11 patients not reaching a possible diagnosis were reclassified as either possible (4/8) or borderline (4/8); 3/9 patients with a possible diagnosis were reclassified as borderline; 6/9 borderline patients received a definite diagnosis of ARVC. Globally, in 6 out of 29 patients with a non-definite diagnosis, EMB confirmed ARVC diagnosis and 17/45 (38%) patients received an upgrade in their diagnostic status with EMB. Notably, EMB also revealed the coexistence of myocarditis and fibro-fatty replacement in 5/45 (9%) patients. No patient experienced complications related to EMB.

Conclusions
Endomyocardial biopsy is a safe, reliable, and useful tool for ARVC diagnosis, allowing to upgrade the diagnostic status of 38% of our patients with a suspect of ARVC diagnosis. It should be performed in experienced centers and it should be guided by electro-anatomic mapping, to maximize its diagnostic power.

Funding Acknowledgement
Type of funding source: None
Long-term follow up after renal denervation for the electrical storm management
Authors: Gorev M , Rachkova I.U.I , Gaidukova N.A , Makarycheva O.V , Nardaia S.H.G , Rzaev F.G , Vasilieva E , Shpektor A 
Published: November 2020

Abstract

Background/Introduction
Electrical storm (ES) is a potentially lethal state developing in 4–28% of ICD patients. Neuroaxial modulation (beta-blockers, deep sedation, epidural anesthesia, stellate ganglion blockade etc) is used to decrease arrhythmogenic predisposition of ventricular myocardium to trigger and maintain ventricular arrhythmias. Catheter renal arterial denervation (RDN) is recently described to prevent atrial fibrillation recurrence after pulmonary vein isolation. We used this procedure in addition to standard care to improve the results of ES treatment.

Purpose
This retrospective single-center case-series is aimed to analyze ES patient population and describe the effect of RDN on the in-hospital number of ventricular tachycardia (VT) episodes and ICD shocks and on the mid-term survival.

Methods
Pts admitted to the CCU for more than 3 sustained VT episodes or more than 3 appropriate ICD shocks during the 24h period in 2017–18 were enrolled into study. Demographic, echocardiographic, ICD check up, renal arteries anatomy and mid-term mortality/transplant data were retrospectively analyzed.

Results
Twelve pts (10 male, age 69,3±9,8 years; 2 female, age 72,0±8,5) were included. Nine pts (75%) had CAD, 3 pts (25%) – DCM. Echocardiography revealed LVEF 30±9%, and LV EDV – 216±38 ml. Time from ICD implantation to ES onset was from 4 mos to 13 yrs. In 2 pts RDN was performed after VT ablation, in 1 pt – both RDN and VT ablation were performed during the same procedure, in 4 pts – RDN was done prior to VT ablation and in 5 pts - without VT ablation. Mean number of ablations was 4,8 per artery. The power was 2 to 11 Wt and lesion duration was 90 sec. In 1 pt RF-balloon-based technology was used to achieve RDN. Dramatic decrease in the number of VT episodes (49c52 vs. 6±2) was found in 6/12 pts directly after RDN procedure. In-hospital rhythm control was accessed in 10/12 pts, 1 pt with DCM was transferred for heart transplant despite VT ablation and RDN. There were no RDN procedure related complications in this group of pts. During mean 22 mos of follow-up 2/11 pts needed repeat hospitalization due to ES recurrence (VT ablation and 2-chamber ICD to CRT-D upgrade, respectively, were performed), 2/11 died (1-probably arrhythmic death, 1-other cause), 1/11 was lost for follow up. Total efficacy of RDN after almost 2 years in this study was 50%. Mean LVEF increased to 38±7%.

Conclusion(s)
Catheter renal artery denervation is effective and safe additional procedure to treat ES patients. It allows decreasing the number of ICD shocks and decreases arrhythmogenic potential of ventricular myocardium. Prospective registry is warranted.

Funding Acknowledgement
Type of funding source: None
Causes of recurrences after stereotactic radio-ablation for refractory ventricular tachycardia
Authors: Herrera Siklody C , Jumeau R , Ozsahin M , Moeckli R , Le Bloa M , Porretta A.P , Pascale P , Domenichini G , Haddad C , Bourhis J , Pruvot E 
Published: November 2020

Abstract

Introduction
Stereotactic radio-ablation (STAR) has been recently introduced for the management of ventricular tachycardia (VT) refractory to antiarrhythmic drugs (AADs) and catheter ablation (CA). The incidence and mechanisms of VT recurrences after STAR remain unknown. We report causes of recurrence in patients (pts) after STAR.

Methods
From 09.2017 to 01.2020, 12pts suffering from refractory VT were enrolled. The underlying cardiopathy was ischemic in 3, inflammatory in 3 and idiopathic in 6 pts. Before STAR, an invasive electro-anatomical mapping (Carto3) of the VT substrate (VT-sub) was performed. A mean dose of 22±2Gy was delivered to the VT-sub using the Cyberknife® system.

Results
The ablation volume was 24±7cc and involved the interventricular septum (IVS) in 10. After a median follow-up of 9±7 months, VT burden decreased by 78% (mean value, from 89 to 20 VT/semester). Out of the 12 pts, 9 (75%) presented some form of VT recurrence (table): 1) that spontaneously resolved in 2 pts; 2) remote from the VT-sub in 2 cases; 3) managed with AADs that had failed before STAR in 2 cases; 4) within the treated VT-sub in 3 cases. In the latter 3 cases, one recurrence came from a site adjacent to the circumflex artery (mean dosis 14.4 Gy), and two were located within the treated IVS (one displaying marked fibrosis, and one with sarcoidosis). Only 4/12 (33%) pts required additional CA.

Conclusion
STAR led in our patients to a strong VT burden reduction. Recurrences occurred at sites remote from the irradiated volume, within the IVS or in under-dosed sites adjacent to critical structures.

Funding Acknowledgement
Type of funding source: None
Periodic repolarization dynamics for prediction of mortality: a systematic review and meta-analysis
Authors: Cheng S , Cai M , Liu X , Zhang N , Jin R , Yang S , Hu Y , Hua W , Zhang S 
Published: November 2020

Abstract

Background
Prediction of death is the philosopher's stone of arrhythmology. The electrophysiology has proven to be an important tool to predict the risk of death. Periodic repolarization dynamics (PRD) is a novel electrocardiographic marker that indicates the sympathetic effect on repolarization. PRD qualifies the low-frequency oscillations of cardiac repolarization instability using high-resolution 12 channel 24-h Holter recording. Several studies showed that PRD was an independent predictor of all-cause mortality and cardiac mortality. However, the prediction value of PRD has not been established.

Purpose
To evaluate the prediction value of PRD as an approach of risk stratification that selects patients at a higher risk of death.

Methods
We conducted electronic searches of MEDLINE (PubMed), Embase, Cochrane Register of Controlled Trials (CENTRAL), Science Citation Index Expanded, WHO International Clinical Trials Registry platform (ICTRP) and ClinicalTrials.gov from inception to January 9th, 2020. We also screened for relevant abstracts from conferences including ACC Annual Scientific Sessions, ESC Congress and Annual Congress of the EHRA for the last five years (2014–2019). The primary outcome was all-cause mortality and secondary outcome was cardiac mortality. We included study with large sample size while more than one study were found based on the same originated population. We extracted data from included studies and reported pooled outcomes as hazard ratios (HRs) with 95% confidential intervals (CI) for time-to-event outcomes using DerSimonian-Laird random-effects model. We did statistical analyses using Stata version 12.0 and R version 3.6.1.

Results
5 studies including 6758 patients met all selection criteria for our meta-analysis. Follow-up period ranged from 20.4 to 75.1 months. Among 5 studies, 3 studies considered PRD as dichotomous variable and the cut-off value was 5.75 deg2, while 2 studies considered PRD as continuous variable and coefficient was expressed in standardized units (increase per standard deviation). We did subgroup analysis according to the type of variable because of heterogeneity. There was a significant higher risk of all-cause mortality in PRD ≥5.75 deg2 patients compared with PRD &lt;5.75 deg2 patients (HR 2.37, 95% CI 1.77–3.17). As for continuous variable, increased PRD was a predictor for all-cause death (HR 1.28, 95% CI 1.14–1.42) (Figure). The cardiac mortality was significantly increased in patients with PRD ≥5.75 deg2 vs PRD &lt;5.75 deg2 (HR 3.06, 95% CI 1.66–5.65). Increased PRD was associated with cardiac mortality in continuous variable subgroup (HR 1.34, 95% CI 1.21–1.48) (Figure).

Conclusion
Our findings suggest PRD is a significant predictor of all-cause mortality and cardiac mortality. PRD provides new additional electrophysiological indicator for risk stratification until further investigations are available.

Funding Acknowledgement
Type of funding source: None
Contacts to the healthcare system prior to out-of-hospital cardiac arrests
Authors: Zylyftari N , Moller S.G , Wissenberg M , Folke F , Barcella C.A , Moller A.L , Mills E.H.A , Tan H.L , Kober L , Lippert F , Gislason G.H , Pedersen C.T 
Published: November 2020

Abstract

Background
Patients who suffer a sudden out-of-hospital cardiac arrest (OHCA) may be preceded by warning symptoms and healthcare system contact. Though, is currently difficult early identification of sudden cardiac arrest patients.

Purpose
We aimed to examine contacts with the healthcare system up to two weeks and one year before OHCA.

Methods
OHCA patients were identified from the Danish Cardiac Arrest Registry (2001–2014). The pattern of healthcare contacts (with either general practitioner (GP) or hospital) within the year prior to OHCA of OHCA patients was compared with that of 9 sex- and age-matched controls from the background general population. Additionally, we evaluated characteristics of OHCA patients according to the type of healthcare contact (GP/hospital/both/no-contact) and the including characteristics of contacts, within two weeks prior their OHCA event.

Results
Out of 28,955 OHCA patients (median age of 72 (62–81) years and with 67% male) of presumed cardiac cause, 16,735 (57.8%) contacted the healthcare system (GP and hospital) within two weeks prior to OHCA. From one year before OHCA, the weekly percentages of contacts to GP were relatively constant (26%) until within 2 weeks prior to OHCA where they markedly increased (54%). In comparison, 14% of the general population contacted the GP during the same period (Figure). The weekly percentages of contacts with hospitals gradually increased in OHCA patients from 3.5% to 6.5% within 6 months, peaking at the second week (6.8%), prior to OHCA. In comparison, only 2% of the general population had a hospital contact in that period (Figure). Within 2 weeks of OHCA, patients contacted GP mainly by telephone (71.6%). Hospital diagnoses were heterogenous, where ischemic heart disease (8%) and heart failure (4.5%) were the most frequent.

Conclusions
There is an increase in healthcare contacts prior to “sudden” OHCA and overall, 54% of OHCA-patients had contacted GP within 2 weeks before the event. This could have implications for developing future strategies for early identification of patients prior to their cardiac arrest.
Figure 1. The weekly percentages of contacts to GP (red) and hospital (blue) within one year before OHCA comparing the OHCA cases to the age- and sex-matched control population (N cases = 28,955; N controls = 260,595).

Funding Acknowledgement
Type of funding source: Public grant(s) – EU funding. Main funding source(s): European Union's Horizon 2020