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Aims: Risk stratification models of sudden cardiac death (SCD) are based on the assumption that risk factors of SCD affect risk to a similar extent in both sexes. The aim of the study is to evaluate differences in clinical outcomes between sexes and evaluate whether risk factors associated with appropriate device therapy (ADT) differ between men and women. Methods and results: We performed a cohort study of implantable cardioverter defibrillator (ICD) patients referred for primary or secondary prevention of SCD between 2009 and 2018. Multivariable Cox regression models for prediction of ADT were constructed for men and women separately. Of 2300 included patients, 571 (25%) were women. Median follow-up was 4.6 (inter-quartile range: 4.4–4.9) years. Time to ADT was shorter for men compared with women [hazard ratio (HR) 1.71, P

Both transesophageal echocardiography (TEE) and intracardiac echocardiography have been used to assist transvenous lead extractions. The clinical utility of continuous echocardiographic monitoring during the procedure is still debated, with different reports supporting opposite findings. In cases where the procedure is expected to be difficult, we propose adding a continuous TEE monitoring using a static 3D/ multiplane probe in mid-esophageal position, with digital remote manipulation of the field of view. This approach may improve the chances of a successful extraction, increase safety, or even guide the entire intervention. We present here a short case series where continuous monitoring by TEE played an important role.

Aims Currently, comparative data on procedural and long-term clinical outcome of outflow tract (OT) idiopathic ventricular arrhythmia (IVA) ablation with manual (MAN), contact force (CF), and magnetic navigation system (MNS) ablation are lacking. The aim of this study was to compare the procedural and long-term clinical outcome of MAN, CF, and MNS ablation of OT IVAs. Methods and results Seventy-three patients (31 MAN, 17 CF, and 25 MNS patients; consecutive per group) with OT IVA, who underwent catheter ablation in our centre were analysed. Procedural success rates (success at the end of the procedure), procedural data and long-term follow-up data were compared. Baseline patient demographics were comparable. Procedural success rates were similar (MAN 81%, 71% CF, and MNS 92%; P = 0.20). Median fluoroscopy time was shorter in the MNS group: MAN 29 (16-38), CF 37 (21-46), and MNS 13 (10-20) min (P = 0.002 for MNS vs. CF and MAN). The overall complication rate was: MAN 10%, CF 0%, and MNS 0% (P = 0.12). Median follow-up was: MAN 2184 (1672-2802), CF 1721 (1404-1913), and MNS 3031 (2524-3286) days (P

Item does not contain fulltext AIMS: Discrete subaortic stenosis (DSS) is often diagnosed early in life and known for its sometimes rapid haemodynamic progression in childhood and strong association with aortic regurgitation (AR). However, data about the evolution of DSS in adulthood are scarce. Therefore, we aimed to evaluate the natural history of DSS, and identify risk factors for the progression of DSS, AR, and intervention-free survival. METHODS AND RESULTS: Conservatively managed adult DSS patients were included in this retrospective multicentre cohort study. Mixed-effects and joint models were used to assess the progression of DSS and AR, and intervention-free survival. Longitudinal natural history data were available for 149 patients [age 20 (IQR: 18-34) years, 48% male]. Sixty patients (40.3%) had associated congenital heart defects (CHDs). The median follow-up duration was 6.3 (IQR: 3.0-12.4) years. The baseline peak left ventricular outflow tract (LVOT) gradient was 32.3 +/- 17.0 mmHg and increased by 0.8 +/- 0.1 mmHg/year. While the baseline LVOT gradient (P = 0.891) or age (P = 0.421) did not influence the progression rate, the presence of associated CHD was associated with faster progression (P = 0.005). Mild AR was common (58%), but did not significantly progress over time (P = 0.701). The median intervention-free survival was 16 years and associated with the baseline LVOT gradient [hazard ratio (HR) = 3.9 (95% CI: 2.0-7.6)], DSS progression [HR = 2.6 (95% CI: 2.0-3.5)], and AR [HR = 6.4 (95% CI 2.6-15.6)]. CONCLUSION: In contrast to children, DSS progresses slowly in adulthood. In particular, patients with associated CHD are at risk for faster progression and should be monitored cautiously. Discrete subaortic stenosis progression is not influenced by the baseline LVOT gradient or age. Mild AR is common, but non-progressive over time.

Background— Discrete subaortic stenosis is notable for its unpredictable hemodynamic progression in childhood and high reoperation rate; however, data about adulthood are scarce. Methods and Results— Adult patients who previously underwent surgery for discrete subaortic stenosis were included in this retrospective multicenter cohort study. Mixed-effects and joint models were used to assess the postoperative progression of discrete subaortic stenosis and aortic regurgitation, as well as reoperation. A total of 313 patients at 4 centers were included (age at baseline, 20.2 years [25th–75th percentile, 18.4–31.0 years]; 52% male). Median follow-up duration was 12.9 years (25th–75th percentile, 6.2–20.1 years), yielding 5617 patient-years. The peak instantaneous left ventricular outflow tract gradient decreased from 75.7±28.0 mm Hg preoperatively to 15.1±14.1 mm Hg postoperatively ( P P =0.001). Mild aortic regurgitation was present in 68% but generally did not progress over time ( P =0.76). A preoperative left ventricular outflow tract gradient ≥80 mm Hg was a predictor for progression to moderate aortic regurgitation postoperatively. Eighty patients required at least 1 reoperation (1.8% per patient-year). Predictors for reoperation included female sex (hazard ratio, 1.53; 95% confidence interval, 1.02–2.30) and left ventricular outflow tract gradient progression (hazard ratio, 1.45; 95% confidence interval, 1.31–1.62). Additional myectomy did not reduce the risk for reoperation ( P =0.92) but significantly increased the risk of a complete heart block requiring pacemaker implantation (8.1% versus 1.7%; P =0.005). Conclusions— Survival is excellent after surgery for discrete subaortic stenosis; however, reoperation for recurrent discrete subaortic stenosis is not uncommon. Over time, the left ventricular outflow tract gradient slowly increases and mild aortic regurgitation is common, although generally nonprogressive over time. Myectomy does not show additional advantages, and because it is associated with an increased risk of complete heart block, it should not be performed routinely.

Aims: To describe smoking habits in adults with congenital heart disease (ACHD) and to assess the relationship between smoking exposure and cardiovascular mortality.Methods: Data on smoking history and cardiovascular mortality were extracted from the Euro Heart Survey on adult congenital heart disease a retrospective cohort study, that included patients diagnosed with 1 of 8 subgroups of ACHD (Atrial Septal Defects, Ventricular Septal Defects, Marfan Syndrome, Aortic Coarctation, Tetralogy of Fallot (ToF), Transposition of the Great Arteries (TGA), Fontan circulation, and Cyanotic disease).Results: Complete data of 3375 ACHD patients (median age 28 years) were available for analysis. At inclusion, 9.3% (n=314) were current smokers and 4.2% (n=142) of the patients had smoked in the past. During a median follow-up of 5.1 years, 101 patients (3%) died. In the majority of cases the cause of death was cardiovascular (n=81; 80%). Kaplan-Meier and Cox survival analysis for each of the defects separately showed a significantly increased age and sex-adjusted cardiovascular mortality associated with smoking exposure in TGA patients (Hazard ratio 4.2 (95% CI 1.0-16.8); P=0.044). Also in ToF mortality was higher amongst smokers, though not significantly (HR 3.4 (95% CI 0.6-18.5); P=0.15). In the remaining defects no relationship between smoking and cardiovascular mortality was observed.Conclusion: The prevalence of smoking amongst ACHD patients is relatively low. Smoking exposure is associated with increased cardiovascular mortality in patients with TGA. Prospective long-term follow-up studies are necessary. (C) 2008 Elsevier Ireland Ltd. All rights reserved.

Objectives: To investigate outcome of pregnancy and fertility in women with double outlet right ventricle (DORV). Methods: Using 2 congenital heart disease registries, 21 female patients with DORV (aged 18–39 years) were retrospectively identified. Detailed recordings of each patient and their completed (>20 weeks gestation) pregnancies were recorded. Results: Overall, 10 patients had 19 pregnancies, including 3 spontaneous miscarriages (16%). During the 16 live birth pregnancies, primarily (serious) noncardiac complications were observed, e.g. premature labor/delivery (n = 7 and n = 3, respectively), small for gestational age (n = 4), preeclampsia (n = 2) and recurrence of congenital heart disease (n = 2). Except for postpartum endocarditis and deterioration of subpulmonary obstruction, only mild cardiac complication pregnancies were recorded. Two women with children reported secondary female infertility. Several menstrual cycle disorders were reported: secondary amenorrhea (n = 4), primary amenorrhea (n = 3) and oligomenorrhea (n = 2). Conclusion: Successful pregnancy in women with DORV is possible. Primarily noncardiac complications were observed and only few (minor) cardiac complications. Infertility and menstrual cycle disorders appear to be more prevalent.

After the introduction of the Biotronik Linox S/SD high-voltage lead, several cases of early failure have been observed.The purpose of this article was to assess the performance of the Linox S/SD lead in comparison to 2 other contemporary leads.We used the prospective Erasmus MC ICD registry to identify all implanted Linox S/SD (n = 408), Durata (St. Jude Medical, model 7122) (n = 340), and Endotak Reliance (Boston Scientific, models 0155, 0138, and 0158) (n = 343) leads. Lead failure was defined by low- or high-voltage impedance, failure to capture, sense or defibrillate, or the presence of nonphysiological signals not due to external interference.During a median follow-up of 5.1 years, 24 Linox (5.9%), 5 Endotak (1.5%), and 5 Durata (1.5%) leads failed. At 5-year follow-up, the cumulative failure rate of Linox leads (6.4%) was higher than that of Endotak (0.4%; P.0001) and Durata (2.0%; P = .003) leads. The incidence rate was higher in Linox leads (1.3 per 100 patient-years) than in Endotak and Durata leads (0.2 and 0.3 per 100 patient-years, respectively; P.001). A log-log analysis of the cumulative hazard for Linox leads functioning at 3-year follow-up revealed a stable failure rate of 3% per year. The majority of failures consisted of noise (62.5%) and abnormal impedance (33.3%).This study demonstrates a higher failure rate of Linox S/SD high-voltage leads compared to contemporary leads. Although the mechanism of lead failure is unclear, the majority presents with abnormal electrical parameters. Comprehensive monitoring of Linox S/SD high-voltage leads includes remote monitoring to facilitate early detection of lead failure.

A search of peer-reviewed literature was conducted to identify reports that provide data on complications associated with pregnancy in women with structural congenital heart disease (CHD). This review describes the outcome of 2,491 pregnancies, including 377 miscarriages (15%) and 114 elective abortions (5%). Important cardiac complications were seen in 11% of the pregnancies. Obstetric complications do not appear to be more prevalent. In complex CHD, premature delivery rates are high, and more children are small for gestational age. The offspring mortality was high throughout the spectrum and was related to the relatively high rate of premature delivery and recurrence of CHD.

Introduction: Recent lead failures have resulted in large recalls with a significant global impact, not only financially, but also physically and mentally for those carrying an implantable cardiac defibrillator (ICD). Following the introduction of the ‘Linox S’ and ‘Linox SD’ ICD leads in 2006 by manufacturer ‘Biotronik’, we observed several cases with early failure. The objective of this study was to determine the failure rate of ‘Biotronik Linox’ leads implanted at our centre, and to compare them with the performance of two comparators, the Durata (St. Jude Medical) and the Endotak (Boston Scientific) lead. Methods: As of 2012, all ‘Linox S or SD’ (n=408), Durata (model 7122, n=340) and Endotak (model 0125, 0138 and 0158, n=343) leads were identified form the Erasmus MC prospective ICD registry. Lead failure was defined by low/high-voltage impedance, failure to capture, sense or defibrillate, or the presence of non-physiological signals not due to external interference. Results: A total of 1091 high-voltage defibrillation leads were implanted between April 2000 and May 2012 in 1066 patients (78% males, median age 62 years). The median time from implantation to lead failure was 3.6 years (IQR, 1.5 to 4.8 years). ‘Linox’ leads demonstrated a sudden increase in failure at 3-3.5 years after implantation and their estimated failure rates were 5.5% and 10.1%, at 5 and 8 years after implantation, respectively. Durata and Endotak demonstrated gradual malfunction and failure rates were 2.1% and 2.1%, and 0.4% and 0.8%, at 5 and 8 years, respectively. The mechanism of failure was noise in the majority of failed leads (50%) followed by impedance out-of-range (29%). Conclusions: ‘Linox S/SD’ leads demonstrate a significant high failure rate. Remote monitoring is reasonable as electrical dysfunction (noise or impedance out-of-range) is observed in the majority of cases. National device safety and advisory committees worldwide might be facing a new challenge.

Introduction: Poor catheter-to-myocardial contact can lead to ineffective ablation lesions and suboptimal outcome. Contact force (CF) sensing catheters in ventricular tachycardia (VT) ablations has not been studied for their long term efficacy. Hypothesis: The hypothesis is that CF ablation is superior to manual ablation (MAN) and non-inferior to remote magnetic navigation (RMN) ablation for safety and efficacy in acute and long term outcome. Methods: A total of 249 consecutive patients underwent VT ablation, with the use of MAN, CF or RMN catheters were included in this single center cohort study from January 2007 until March 2014. The primary endpoints were procedural success, acute major complications and VT recurrences at follow-up. The average follow-up period was ± 20 months. Results: Acute success was achieved in 191 out of 249 procedures (75.9%). Acute success in manual ablation, CF ablation and RMN ablation was 70.1%, 72.3% and 85.2% respectively (P = 0.038). Major complications occurred in 3.2% and there was a trend towards less major complications (P = 0.055) in the RMN group. Thirty-six percent of the patients with an initially successful procedure had a recurrence during follow-up (CF 41.2% MAN 37.5% RMN 32.0% P = NS). Conclusions: The use of CF sensing catheters does not improve the procedural outcome or safety profile in comparison to non-CF sensing ablation in ventricular arrhythmias. RMN non-CF sensing ablation has the highest procedural success rate. Future studies are necessary to investigate the role of CF in VT ablation and to define the optimal force.

Item does not contain fulltext OBJECTIVES/BACKGROUND: Congenital aortic stenosis (AS) is the most common obstructive left heart lesion in the young adult population and often complicated by aortic dilatation. Our objective was to evaluate accuracy of aortic imaging with transthoracic echocardiography (TTE) compared with cardiac magnetic resonance (CMR). METHODS: Aortic diameters were measured at 4 levels by CMR and TTE. Agreement and concordance were assessed by Pearson's correlation and Bland-Altman analysis. RESULTS: Fifty-nine patients (age 33 +/- 8 years; 66% male) with congenital AS and a bicuspid aortic valve (BAV) were included. Aortic diameters were generally smaller with TTE than with CMR. The best correlation was found at the level of the sinotubular junction (R(2) = 0.78) with a bias of 1.46 mm (limits of agreement: -5.47 to +8.39 mm). In patients with an aortic aneurysm >40 mm (n = 29) the correlation and agreement between TTE and CMR were found to be less good when compared with patients with normal aortic diameters, especially at the level of the proximal ascending aorta. The correlation and agreement between both imaging modalities were better in patients with type 1 BAV compared with type 2 BAV. Intra- and interobserver variability was smaller with CMR (1.8-5.9%) compared with TTE (6.9-15.0%). CONCLUSIONS: CMR was found to be superior to TTE for imaging of the aorta in patients with congenital AS, especially at the level of the proximal ascending aorta when an aortic aneurysm is present. Therefore, ideally CMR should be performed at least once to ensure an ascending aortic aneurysm is not missed.

A search of peer-reviewed literature was conducted to identify reports that provide data on complications associated with pregnancy in women with structural congenital heart disease (CHD). This review describes the outcome of 2,491 pregnancies, including 377 miscarriages (15%) and 114 elective abortions (5%). Important cardiac complications were seen in 11% of the pregnancies. Obstetric complications do not appear to be more prevalent. In complex CHD, premature delivery rates are high, and more children are small for gestational age. The offspring mortality was high throughout the spectrum and was related to the relatively high rate of premature delivery and recurrence of CHD.