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The new 5-year ventricular arrhythmia (VA) occurrence risk model is a major breakthrough for arrhythmia risk stratification in the challenging population of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). In the original study, the model resulted in a 20.6% reduction in implantable cardioverter-defibrillator (ICD) placement compared with the 2015 consensus, for the same protection level. However, only internal validation was performed, limiting generalisation. We externally validated the model in a European tertiary care cohort of 128 patients with ARVC with restrictive indications for primary prevention ICD placement. Overall, 74% were men, none had VA history, and a single patient had an ICD at baseline. Median age at diagnosis was 38 years (interquartile range [IQR] 28-50). During a median follow-up of 7.8 years (IQR 6.1-9.7), 15 patients (12%) experienced VA. The model provided good discrimination, with a C-index for 5-year VA risk prediction of 0.84 (95% confidence interval 0.74-0.93). However, the model led to an overestimation of the 5-year VA risk when applying thresholds < 50%. With a < 10% predicted risk, no patient showed VA. With a 7.5% predicted risk, the ICD:VA ratio was 6.3 vs 3.4 in the original study. The model still outperformed the 2015 International Task Force Consensus. Overall, in a relatively large European ARVC cohort with restrictive indications for ICD placement, the ARVC model for VA prediction successfully identified ARVC patients with VA during follow-up. Yet, our study underscores the need for careful threshold selection, considering the model's associated risk overestimation in low- to intermediate-risk patients., (Copyright © 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.)

Introduction: The evolution of atrioventricular conduction disorders after transcatheter aortic valve implantation (TAVI) remains poorly understood. We sought to identify factors associated with late (occurring ≥7 days after the procedure) high-grade atrioventricular blocks after TAVI, based on specific pacemaker memory data., Methods and Results: STIM-TAVI (NCT03338582) was a prospective, multicentre, observational study that enrolled all patients (from November 2015 to January 2017) implanted with a specific dual chamber pacemaker after TAVI, with the SafeR algorithm activated, allowing continuous monitoring of atrioventricular conduction. The primary endpoint was the occurrence of centrally adjudicated late high-grade atrioventricular blocks during the year after TAVI. Among 197 patients, 138 (70.1%) had ≥1 late high-grade atrioventricular block. Whereas oversizing (p = .005), high-grade atrioventricular block during TAVI (p < .001), and early (within 6 days) high-grade atrioventricular block (p < .001) were associated with occurrence of late high-grade atrioventricular block, self-expanding prothesis (p = .88), prior right bundle branch block (p = .45), low implantation (p = .06), and new or wider left bundle branch block and lengthening of PR interval (p = .24) were not. In multivariable analysis, only post-TAVI early high-grade atrioventricular block remained associated with late high-grade atrioventricular blocks (Days 0-1: odds ratio [OR], 3.25; 95% confidence interval [CI], 1.57-6.74; p = .001; Days 2-6: OR, 4.13; 95% CI, 2.06-8.31; p < .001), whereas other conventionally used predictors were not., Conclusion: One-third of pacemaker-implanted patients do not experience late high-grade atrioventricular block. Our findings suggest that post-TAVI early high-grade atrioventricular block is the main factor associated with occurrence of late high-grade atrioventricular blocks., (© 2021 Wiley Periodicals LLC.)

Background: Friedreich's ataxia (FA) is a rare autosomal recessive mitochondrial disease resulting of a triplet repeat expansion guanine-adenine-adenine (GAA) in the frataxin (FXN) gene, exhibiting progressive cerebellar ataxia, diabetes and cardiomyopathy. We aimed to determine the relationship between cardiac biomarkers, serum N-terminal pro-brain natriuretic peptide (NT-proBNP), and serum cardiac high-sensitivity troponin (hsTnT) concentrations, and the extent of genetic abnormality and cardiac parameters., Methods: Between 2013 and 2015, 85 consecutive genetically confirmed FA adult patients were prospectively evaluated by measuring plasma hsTnT and NT-proBNP concentrations, electrocardiogram, and echocardiography., Results: The 85 FA patients (49% women) with a mean age of 39 ± 12 years, a mean disease onset of 17 ± 11 years had a mean SARA (Scale for the Assessment and Rating of Ataxia) score of 26 ± 10. The median hsTnT concentration was 10 ng/L (3 to 85 ng/L) and 34% had a significant elevated hsTnT ≥ 14 ng/L. Increased septal wall thickness was associated with increased hsTnT plasma levels ( p < 0.001). The median NT-proBNP concentration was 31 ng/L (5 to 775 ng/L) and 14% had significant elevated NT-proBNP ≥ 125 ng/L. Markers of increased left ventricular filling pressure (trans mitral E/A and lateral E/E' ratio) were associated with increased NT-proBNP plasma levels ( p = 0.01 and p = 0.01). Length of GAA or the SARA score were not associated with hsTnT or NT-proBNP plasma levels., Conclusion: hsTnT was increased in 1/3 of the adult FA and associated with increased septal wall thickness. Increased NT-proBNP remained a marker of increased left ventricular filling pressure. This could be used to identify patients that should undergo a closer cardiac surveillance.

Predictive genetic testing (PGT) is offered to asymptomatic relatives at risk of hereditary heart disease, but the impact of result disclosure has been little studied. We evaluated the psychosocial impacts of PGT in hereditary heart disease, using self-report questionnaires (including the State-Trait Anxiety Inventory) in 517 adults, administered three times to the prospective cohort (PCo: n = 264) and once to the retrospective cohort (RCo: n = 253). The main motivations for undergoing PGT were "to remove doubt" and "for their children". The level of anxiety increased between pre-test and result appointments ( p <0.0001), returned to baseline after the result (PCo), and was moderately elevated at 4.4 years (RCo). Subjects with a history of depression or with high baseline anxiety were more likely to develop anxiety after PGT result ( p = 0.004 and p <0.0001, respectively), whatever it was. Unfavourable changes in professional and/or family life were observed in 12.4% (PCo) and 18.7% (RCo) of subjects. Few regrets about PGT were expressed (0.8% RCo, 2.3% PCo). Medical benefit was not the main motivation, which emphasises the role of pre/post-test counselling. When PGT was performed by expert teams, the negative impact was modest, but careful management is required in specific categories of subjects, whatever the genetic test result., Competing Interests: The authors declare no conflict of interest.

Background: Therapeutic strategies for electrical storm (ES) in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) are not well defined., Objective: The purpose of this study was to report the acute and long-term results of ventricular tachycardia (VT) radiofrequency catheter ablation (RFCA) as a treatment of ES in patients with ARVC., Methods: This multicenter study retrospectively enrolled 23 consecutive patients with ARVC (mean age 43.6 ± 16.7 years; all men) who underwent 24 RFCA procedures for ES between 2003 and 2015., Results: Thirteen patients (57%) had a previous VT RFCA procedure; 14 (61%) had right ventricular dysfunction and 7 (30%) left ventricular ejection fraction ≤ 50%. The clinical VT was inducible in 19 procedures (79%). Epicardial ablation was performed in 4 procedures (17%). The median number of targeted VTs was 1 (range 1-6). Complete acute success (no VT inducible) was achieved in 11 procedures (46%) and partial acute success (clinical VT nor inducible) in 11 (46%). After a median follow-up of 3.9 years (range 1 month-10 years), ES recurred in 2 patients and end-stage heart failure developed in 4 (17%), leading to 1 death and 3 heart transplantations. At 1-year follow-up, the probability of freedom from VT recurrence was 75% and did not significantly predict long-term survival. At the last evaluation, 8 patients (35%) were free of non-β-blocker antiarrhythmic drugs as compared with 1 (4%) at baseline (P = .02)., Conclusion: Catheter ablation was efficient to prevent ES recurrence in patients with ARVC. However, these patients were at high risk of evolution toward ARVC-related heart failure that was not associated with VT recurrence., (Copyright © 2019 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Objectives: The purpose of this study was to identify clinical factors associated with arrhythmic events and sudden cardiac death (SCD), and to evaluate the prognostic value of electrophysiological study (EPS) in arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients without implantable cardioverter-defibrillators (ICDs)., Background: ARVC/D is an inherited cardiomyopathy characterized by a risk of SCD. Few studies have evaluated predictive factors of ventricular arrhythmias (VAs) in patients without ICDs., Methods: Between 2000 and 2010, all consecutive patients with ARVC/D without ICDs and with EPS at diagnosis were enrolled. Patients that received an ICD during follow-up were censored at the date of implantation, and in that case, only VAs that occurred before ICD implantation were analyzed. Risk factors for any VA event were determined by Cox regression. Patients that only experienced SCD or aborted cardiac arrest (ACA) were reported., Results: A total of 137 consecutive patients (78% male) diagnosed with ARVC/D without ICD were enrolled. 31% had sustained ventricular tachycardia at diagnosis. After mean follow-up of 42 ± 31 months, 19 patients experienced an episode of sustained VA and 5 patients experienced a SCD/ACA. No event occurred in asymptomatic patients. Left ventricular ejection fraction ≤50% (p = 0.024), positive EPS (p = 0.017), and physical activity >6 h/week (p = 0.025) were independently associated with occurrence of VAs. SCD/ACA exclusively occurred in male probands with definite diagnosis and syncope., Conclusions: In this cohort of ARVC/D patients without ICD, left ventricular ejection fraction ≤50%, positive EPS, and physical activity >6 h/week were independent predictors of VAs, whereas asymptomatic patients at diagnosis were at low risk. EPS predicted all VAs but had limited value to predict SCD/ACA., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Aims: The Cardiomyopathy Registry of the EURObservational Research Programme is a prospective, observational, and multinational registry of consecutive patients with four cardiomyopathy subtypes: hypertrophic cardiomyopathy (HCM), dilated cardiomyopathy (DCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and restrictive cardiomyopathy (RCM). We report the baseline characteristics and management of adults enrolled in the registry., Methods and Results: A total of 3208 patients were enrolled by 69 centres in 18 countries [HCM (n = 1739); DCM (n = 1260); ARVC (n = 143); and RCM (n = 66)]. Differences between cardiomyopathy subtypes (P < 0.001) were observed for age at diagnosis, history of familial disease, history of sustained ventricular arrhythmia, use of magnetic resonance imaging or genetic testing, and implantation of defibrillators. When compared with probands, relatives had a lower age at diagnosis (P < 0.001), but a similar rate of symptoms and defibrillators. When compared with the Long-Term phase, patients of the Pilot phase (enrolled in more expert centres) had a more frequent rate of familial disease (P < 0.001), were more frequently diagnosed with a rare underlying disease (P < 0.001), and more frequently implanted with a defibrillator (P = 0.023). Comparing four geographical areas, patients from Southern Europe had a familial disease more frequently (P < 0.001), were more frequently diagnosed in the context of a family screening (P < 0.001), and more frequently diagnosed with a rare underlying disease (P < 0.001)., Conclusion: By providing contemporary observational data on characteristics and management of patients with cardiomyopathies, the registry provides a platform for the evaluation of guideline implementation. Potential gaps with existing recommendations are discussed as well as some suggestions for improvement of health care provision in Europe.

Objectives: This study sought to assess the relationship between an immediate invasive strategy and survival after an out-of-hospital cardiac arrest (OHCA) of presumed cardiac cause, according to prognosis evaluated on hospital arrival., Background: An immediate coronary angiogram (CAG) may be associated with better outcome after OHCA in neurologically preserved patients but could be futile in other cases., Methods: From May 2011 to May 2015, we collected data for all patients admitted in hospital after OHCA in Paris and its suburbs (France). Risk of in-hospital death was retrospectively calculated using the validated Cardiac Arrest Hospital Prognosis score, which includes age, setting, initial rhythm, durations from collapse to basic life support and from basic life support to return of spontaneous circulation, pH, and epinephrine dose. Independent predictors of survival at discharge (including immediate CAG) were assessed in multivariate logistic regression in each of the 3 pre-defined subgroups of Cardiac Arrest Hospital Prognosis score: low risk (<150 points), medium risk (150 to 200 points), and high risk (>200 points) for in-hospital death., Results: A total of 1,410 patients were included and overall survival rate at hospital discharge was 32%. Distribution in the low-, medium-, and high-risk Cardiac Arrest Hospital Prognosis subgroups was 667 (47%), 469 (33%), and 274 patients (20%), respectively. The rate of early CAG was 86%, 66%, and 47% in the low-, medium-, and high-risk subgroups, respectively (p < 0.001). Early invasive strategy was independently associated with better survival in low-risk patients (odds ratio: 2.3; 95% confidence interval: 1.4 to 3.9; p = 0.001), but not in medium-risk (p = 0.55) and high-risk (p = 0.43) patients. Sensitivity analysis found consistent results., Conclusions: In cardiac arrest patients, our results suggest that investigations regarding early CAG after OHCA should focus on patients with preserved neurological status., (Copyright © 2018 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Organized atrial arrhythmias (OAAs) are common after orthotopic heart transplantation (OHT). Some controversies remain about their clinical presentation, relationship with atrial anastomosis and electrophysiologic features. The objectives of this retrospective study were to determine the mechanisms of OAAs after OHT and describe the outcomes of radiofrequency catheter ablation (RFCA)., Methods: Thirty consecutive transplanted patients (mean age 48 ± 17 years, 86.6% male) underwent 3-dimensional electroanatomic mapping and RFCA of their OAA from 2004 to 2012 at our center., Results: Twenty-two patients had biatrial anastomosis and 8 had bicaval anastomosis. Macro-reentry was the arrhythmia mechanism for 96% of patients. The electrophysiologic diagnoses were: cavotricuspid isthmus (CTI)-dependent atrial flutter (AFL) in 93% of patients (n = 28); perimitral AFL in 3% (n = 1); and focal atrial tachycardia (FAT) in 3% (n = 1). In 5 patients with biatrial anastomosis, a right FAT was inducible. Primary RFCA success was obtained in 93% of patients. Mean follow-up time was 39 ± 26.8 months. Electrical repermeation between recipient and donor atria, present in 20% of patients (n = 6), did not account for any of the OAAs observed. Survival without OAA relapse at 12, 24 and 60 months was 93%, 89% and 79%, respectively., Conclusions: CTI-dependent AFL accounted for most instances of OAA after OHT, regardless of anastomosis type. Time from transplantation to OAA was shorter with bicaval than with biatrial anastomosis. RFCA was safe and provided good long-term results., (Copyright © 2017 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: Diagnosis of short QT syndrome (SQTS) remains difficult in case of borderline QT values as often found in normal populations. Whether some shortening of refractory periods (RP) may help in differentiating SQTS from normal subjects is unknown., Methods and Results: Atrial and right ventricular RP at the apex and right ventricular outflow tract as determined during standard electrophysiological study were compared between 16 SQTS patients (QTc 324±24 ms) and 15 controls with similar clinical characteristics (QTc 417±32 ms). Atrial RP were significantly shorter in SQTS compared with controls at 600- and 500-ms basic cycle lengths. Baseline ventricular RP were significantly shorter in SQTS patients than in controls, both at the apex and right ventricular outflow tract and for any cycle length. Differences remained significant for RP of any subsequent extrastimulus at any cycle length and any pacing site. A cut-off value of baseline RP <200 ms at the right ventricular outflow tract either at 600- or 500-ms cycle length had a sensitivity of 86% and a specificity of 100% for the diagnosis of SQTS., Conclusions: Patients with SQTS have shorter ventricular RP than controls, both at baseline during various cycle lengths and after premature extrastimuli. A cut-off value of 200 ms at the right ventricular outflow tract during 600- and 500-ms basic cycle length may help in detecting true SQTS from normal subjects with borderline QT values., (© 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.)

Background and Aim: It was hypothesized that the pattern of coronary occlusion (thrombus composition) might contribute to the onset of ventricular arrhythmia and sudden cardiac death (SCD) in myocardial infarction (MI)., Methods: The TIDE (Thrombus and Inflammation in sudden DEath) study included patients with angiographically-proven acute coronary occlusion as the cause of a ST elevation MI (STEMI) complicated by Sudden Cardiac Death (SCD group) or not (STEMI group). Thrombi were obtained by thrombo-aspiration before primary percutaneous coronary stenting and analyzed with a quantitative method using scanning electron microscopy. We compared the composition of the thrombi responsible for the coronary occlusion between the two groups and evaluated factors influencing its composition., Results: We included 121 patients and found that thrombus composition was not different between the SCD group (n=23) and the STEMI group (n=98) regarding content of fibrin fibers (60.3±18.4% vs. 62.4±18.4% respectively, p=0.68), platelets (16.3±19.2% vs. 15.616.7±%, p=0.76), erythrocytes (14.6±12.5% vs. 13±12.1%, p=0.73) and leukocytes (0.6±0.9% vs. 0.8±1.5%, p=0.93). Thrombus composition did not differ between patients receiving upstream-use of glycoprotein IIb/IIIa platelet receptor inhibitors (GPI) and patients free of GPI. The only factor found to influence thrombus composition was the ischemic time from symptom onset to primary PCI, with a decreased content in fibrin fibers (57.8±18.5% vs. 71.9±10.1%, p=0.0008) and a higher platelet content (19.2±19.1% vs. 7.9±5.7% p=0.014) in early presenters (<3h of ischemic time) vs. late presenters (>6h of ischemic time)., Conclusion: Composition of intracoronary thrombi in STEMI patients does not differ between those presenting with and without SCD. Time from symptom onset to coronary reperfusion seems to be the strongest factor influencing thrombus composition in MI., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Purpose: Cardiogenic shock due to post-resuscitation myocardial dysfunction is a major cause of mortality among patients hospitalized after cardiac arrest (CA). Veno-arterial extracorporeal membrane oxygenation (VA-ECMO) has been proposed in the most severe cases but the level of evidence is very low. We assessed characteristics, outcome and prognostic factors of patients treated with VA-ECMO for post-CA shock., Methods: Using a large regional registry, we focused on all CA admitted in ICU. Among those who developed a post-CA shock, prognostic was compared according to VA-ECMO use, using logistic regression and propensity score. Specific prognostic factors were identified among VA-ECMO patients., Results: Among 2988 patients admitted after CA, 1489 developed a post-CA shock, and were included. They were mostly male (68%), with mean age 63 years (SD=15). Fiflty-two patients (3.5%) were treated with VA-ECMO, mostly patients with ischemic cause of CA (67%). Among patients with post-CA shock, 312 (21%) were discharged alive (25% in VA-ECMO group, 21% in control group, P=0.45). After adjustment for pre-hospital and in-hospital factors, survival did not differ among patients treated with VA-ECMO (OR for survival=0.9, 95%CI 0.4-2.3, P=0.84). After propensity-score matching, results were consistent. Among patients treated with VA-ECMO, initial arterial pH (OR=1.7 per 0.1 increase, 95%CI 1.0-2.8, P=0.04) and implantation of VA-ECMO over 24h after ROSC (OR=20.0, 95%CI 1.4-277.3, P=0.03) were associated with survival., Conclusions: Post-CA shock is frequent and is associated with a high mortality rate. When used in selected patients, we observed that VA-ECMO could be an appropriate treatment., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Aims: Survival after out-of-hospital cardiac arrest (OHCA) remains disappointingly low. Among patients admitted alive, early prognostication remains challenging. This study aims to establish a stratification score for patients admitted in intensive care unit (ICU) after OHCA, according to their neurological outcome., Methods and Results: The CAHP (Cardiac Arrest Hospital Prognosis) score was developed from the Sudden Death Expertise Center registry (Paris, France). The primary outcome was poor neurological outcome defined as Cerebral Performance Category 3, 4, or 5 at hospital discharge. Independent prognostic factors were identified using logistic regression analysis and thresholds defined to stratify low-, moderate-, and high-risk groups. The CAHP score was validated in both a prospective and an external data set (Parisian Cardiac Arrest Registry). The developmental data set included 819 patients admitted from May 2011 to December 2012. After multivariate analysis, seven variables were independently associated with poor neurological outcome and subsequently included in the CAHP score (age, non-shockable rhythm, time from collapse to basic life support, time from basic life support to return of spontaneous circulation, location of cardiac arrest, epinephrine dose, and arterial pH). Three risks groups were identified: low risk (score ≤150, 39% of unfavourable outcome), medium risk (score 150-200, 81% of unfavourable outcome) and high-risk group (score ≥200, 100% of unfavourable outcome). The AUC of the CAHP score were 0.93, and the discrimination value in the validation data sets was consistent (respectively, AUC 0.91 and 0.85)., Conclusion: The CAHP score represents a simple tool for early stratification of patients admitted in ICU after OHCA. A high-risk category of patients with very poor prognosis can be easily identified., (Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2015. For permissions please email: journals.permissions@oup.com.)

Background: Implantable cardioverter-defibrillators (ICDs) are recommended in patients with low ejection fraction. However, the survival benefit of ICDs in patients with end-stage heart failure listed for heart transplantation is unclear., Aim: To evaluate the ICD benefit on mortality in this population., Methods: Three hundred and eighty consecutive patients listed for heart transplantation between 2005 and 2009 in one tertiary heart transplant centre were enrolled in a retrospective registry; 122 patients received an ICD before or within 3 months after being listed for heart transplantation (ICD group). Predictors of death on the waiting list were assessed by Cox regression., Results: Overall, 15.6% of patients died while awaiting heart transplantation. Non-ICD patients presented more often haemodynamic compromise requiring mechanical circulatory support (29.1% vs. 9.8%; P<0.001), and were more likely to die while on the waiting list (19.0% vs. 8.3%; log-rank P=0.001). However, in the multivariable model, ICD did not remain an independent predictor of death. Need for mechanical circulatory support (P<0.001), low ejection fraction (P=0.001) and registration on the regular list (P=0.008) were the only independent predictors of death. Death was mainly caused by haemodynamic compromise (76.3% of deaths), which occurred more frequently in the non-ICD group (14.7% vs. 5.8%; log-rank P=0.002). Unknown/arrhythmic deaths did not differ significantly between the two groups (3.9% vs. 1.7%; log-rank P=0.21). ICD-related complications occurred in 21.4% of patients, mainly as a result of postoperative worsening of heart failure (11.9%)., Conclusion: Haemodynamic failure appears as the main determinant of mortality in patients with end-stage heart failure awaiting heart transplantation. ICD seems to have little benefit on survival in this population., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Introduction: Twenty-five to 32% of patients with synovial sarcoma (SS) relapse after appropriate treatment, and experience a poor outcome. Patients who can be salvaged by second-line therapy need to be more clearly identified., Patients and Methods: Data of patients treated in SFCE (Société Française des Cancers de l'Enfant) centers with an initial diagnosis of localized SS before the age of 18 years and treated from 1/1988 to 12/2008, and who experienced at least one relapse, were retrieved. After descriptive analysis, statistical analysis was performed to determine prognostic factors., Results: Thirty-seven patients were identified. First relapse occurred after a median interval of 24 months and was localized in 73.0% of cases and metastatic in 24.3% of cases. Treatment of relapse consisted of new surgery in 75.7% of cases, second-line chemotherapy in 73.0% of cases and radiotherapy in 48.6% of cases. Response rate to ifosfamide-based regimens was 36.4%. Overall, 70.3% patients achieved a second complete remission. Median 5-year-event-free survival was 32.8% and 5-year overall survival was 42.1%. Factors significantly correlated with better survival were primary tumor involving the limbs, age less than 12 years at diagnosis, absence of chemotherapy or radiotherapy as initial treatment and local relapse., Conclusion: Despite its poor overall outcome, relapse of synovial sarcoma sometimes remains curable. Aggressive surgery, when possible, in combination with chemotherapy and radiotherapy is the recommended treatment. Ifosfamide-based regimens may remain effective in patients with relapsed SS. However, alternative therapies should be proposed in patients with poor prognostic factors., (© 2014 Wiley Periodicals, Inc.)