Prognostic significance of a low T/R ratio in Brugada syndrome.

OBJECTIVES: To determine the prognostic value of a low T/R ratio, defined as the amplitude ratio between the T waves and the R waves, in patients (pts) with a spontaneous type-1 Brugada pattern (SBT1). BACKGROUND: Abnormalities of myocardial repolarization may play a key role in the initiation of ventricular fibrillation (VF) in Brugada syndrome (BrS). Recent studies have shown that the height of the T waves and the T/R ratio are inversely proportional to sudden cardiac arrest (SCA) risk in early repolarization syndrome and hypertrophic cardiomyopathy. METHODS: In an international retrospective study, we reviewed 115 pts. (105 males, 91.3%). 45 had VF and/or SCA (38.7 ±â€¯11.5 years old, all males), while 70 (49.3 ±â€¯12.0 years, 10 women) remained free of ventricular arrhythmia. 6 ECG markers plus the T/R ratio in leads V5 & II were studied. RESULTS: The T/R ratio among leads II & V5 was significantly lower in the VF/SCA group (0.24 [0.14; 0.38]vs. 0.34 [0.24; 0.45]; p = 0.006). 44.4% of pts. in the VF/SCA group had a lowest T/R ratio among leads II & V5 ≤ 0.17 compared to 11.4% in the non-VF/SCA group (p < 0.001). In multivariate analysis, a lowest T/R ratio among leads II & V5 ≤ 0.17 was independently associated with VF/SCA (OR 6.10, 95% CI 1.92-19.40; p = 0.002). Type 1 Brugada pattern in the peripheral leads (OR 10.78) and early repolarization (OR 3.60) were other independent markers of VF/SCA. CONCLUSION: A low T/R ratio among leads II & V5 is an independent marker for VF/SCA risk in patients with type-1 Brugada pattern.

Prediction of ventricular arrhythmias in patients with a spontaneous Brugada type 1 pattern: the key is in the electrocardiogram.

AIMS: There is currently no reliable tool to quantify the risks of ventricular fibrillation or sudden cardiac arrest (VF/SCA) in patients with spontaneous Brugada type 1 pattern (BrT1). Previous studies showed that electrocardiographic (ECG) markers of depolarization or repolarization disorders might indicate elevated risk. We aimed to design a VF/SCA risk prediction model based on ECG analyses for adult patients with spontaneous BrT1. METHODS AND RESULTS: This retrospective multicentre international study analysed ECG data from 115 patients (mean age 45.1 ± 12.8 years, 105 males) with spontaneous BrT1. Of these, 45 patients had experienced VF/SCA and 70 patients did not experience VF/SCA. Among 10 ECG markers, a univariate analysis showed significant associations between VF/SCA and maximum corrected Tpeak-Tend intervals ≥100 ms in precordial leads (LMaxTpec) (P < 0.001), BrT1 in a peripheral lead (pT1) (P = 0.004), early repolarization in inferolateral leads (ER) (P < 0.001), and QRS duration ≥120 ms in lead V2 (P = 0.002). The Cox multivariate analysis revealed four predictors of VF/SCA: the LMaxTpec [hazard ratio (HR) 8.3, 95% confidence interval (CI) 2.4-28.5; P < 0.001], LMaxTpec + ER (HR 14.9, 95% CI 4.2-53.1; P < 0.001), LMaxTpec + pT1 (HR 17.2, 95% CI 4.1-72; P < 0.001), and LMaxTpec + pT1 + ER (HR 23.5, 95% CI 6-93; P < 0.001). Our multidimensional penalized spline model predicted the 1-year risk of VF/SCA, based on age and these markers. CONCLUSION: LMaxTpec and its association with pT1 and/or ER indicated elevated VF/SCA risk in adult patients with spontaneous BrT1. We successfully developed a simple risk prediction model based on age and these ECG markers.

Electrocardiographic characteristics of patients with funnel chest before and after surgical correction using pectus bar: A new association with precordial J wave pattern.

OBJECTIVE: Abnormal ECG findings suggestive of cardiac disease are frequent in patients with funnel chest, although structural heart disease is rare. Electrocardiographic characteristics and changes following new surgical treatments in young adults are not described so far. The aim of the study was to analyze electrocardiographic characteristics of patients with funnel chest before and after minimally invasive funnel chest correction by the Nuss procedure. METHODS: Twenty-six patients with surgical correction of funnel chest using pectus bar were included. Twelve-lead ECGs before and later than one year after surgery were analyzed. RESULTS: In postoperative ECGs, amplitude of P wave in lead II and negative terminal amplitude of P wave in lead V1 decreased from 0.13 to 0.10mV (p=0.03), and from 0.10 to 0.04mV (p<0.001), respectively. Mean QRS duration decreased from 108ms to 98ms (p=0.003) after correction. A pathological left and right Sokolow-Lyon index was observed in 35% and 23% of patients before, versus 8% (p=0.04) and 0% (p=0.01) after correction, respectively. In contrast, the rate of patients with J wave pattern in precordial leads V4-V6 increased from 8% before to 42% after surgery (p=0.004). CONCLUSIONS: ECG abnormalities in patients with funnel chest are frequent, and can normalize after surgical correction by the Nuss procedure. De novo J wave pattern in precordial leads V4-V6 is a frequent finding after surgical funnel chest correction using pectus bar.

Radio-frequency ablation as primary management of well-tolerated sustained monomorphic ventricular tachycardia in patients with structural heart disease and left ventricular ejection fraction over 30%.

AIMS: Patients with well-tolerated sustained monomorphic ventricular tachycardia (SMVT) and left ventricular ejection fraction (LVEF) over 30% may benefit from a primary strategy of VT ablation without immediate need for a 'back-up' implantable cardioverter-defibrillator (ICD). METHODS AND RESULTS: One hundred and sixty-six patients with structural heart disease (SHD), LVEF over 30%, and well-tolerated SMVT (no syncope) underwent primary radiofrequency ablation without ICD implantation at eight European centres. There were 139 men (84%) with mean age 62 ± 15 years and mean LVEF of 50 ± 10%. Fifty-five percent had ischaemic heart disease, 19% non-ischaemic cardiomyopathy, and 12% arrhythmogenic right ventricular cardiomyopathy. Three hundred seventy-eight similar patients were implanted with an ICD during the same period and serve as a control group. All-cause mortality was 12% (20 patients) over a mean follow-up of 32 ± 27 months. Eight patients (40%) died from non-cardiovascular causes, 8 (40%) died from non-arrhythmic cardiovascular causes, and 4 (20%) died suddenly (SD) (2.4% of the population). All-cause mortality in the control group was 12%. Twenty-seven patients (16%) had a non-fatal recurrence at a median time of 5 months, while 20 patients (12%) required an ICD, of whom 4 died (20%). CONCLUSION: Patients with well-tolerated SMVT, SHD, and LVEF > 30% undergoing primary VT ablation without a back-up ICD had a very low rate of arrhythmic death and recurrences were generally non-fatal. These data would support a randomized clinical trial comparing this approach with others incorporating implantation of an ICD as a primary strategy.

Dronedarone in high-risk permanent atrial fibrillation.

BACKGROUND: Dronedarone restores sinus rhythm and reduces hospitalization or death in intermittent atrial fibrillation. It also lowers heart rate and blood pressure and has antiadrenergic and potential ventricular antiarrhythmic effects. We hypothesized that dronedarone would reduce major vascular events in high-risk permanent atrial fibrillation. METHODS: We assigned patients who were at least 65 years of age with at least a 6-month history of permanent atrial fibrillation and risk factors for major vascular events to receive dronedarone or placebo. The first coprimary outcome was stroke, myocardial infarction, systemic embolism, or death from cardiovascular causes. The second coprimary outcome was unplanned hospitalization for a cardiovascular cause or death. RESULTS: After the enrollment of 3236 patients, the study was stopped for safety reasons. The first coprimary outcome occurred in 43 patients receiving dronedarone and 19 receiving placebo (hazard ratio, 2.29; 95% confidence interval [CI], 1.34 to 3.94; P=0.002). There were 21 deaths from cardiovascular causes in the dronedarone group and 10 in the placebo group (hazard ratio, 2.11; 95% CI, 1.00 to 4.49; P=0.046), including death from arrhythmia in 13 patients and 4 patients, respectively (hazard ratio, 3.26; 95% CI, 1.06 to 10.00; P=0.03). Stroke occurred in 23 patients in the dronedarone group and 10 in the placebo group (hazard ratio, 2.32; 95% CI, 1.11 to 4.88; P=0.02). Hospitalization for heart failure occurred in 43 patients in the dronedarone group and 24 in the placebo group (hazard ratio, 1.81; 95% CI, 1.10 to 2.99; P=0.02). CONCLUSIONS: Dronedarone increased rates of heart failure, stroke, and death from cardiovascular causes in patients with permanent atrial fibrillation who were at risk for major vascular events. Our data show that this drug should not be used in such patients. (Funded by Sanofi-Aventis; PALLAS ClinicalTrials.gov number, NCT01151137.).

Catheter ablation of stable ventricular tachycardia before defibrillator implantation in patients with coronary heart disease (VTACH): an on-treatment analysis.

BACKGROUND: In the Ventricular Tachycardia Ablation in Coronary Heart Disease (VTACH) study, an intention-to-treat approach was used and may have diminished the observed degree of treatment effect. We present a subanalysis of the VTACH study by treatment actually received. METHODS AND RESULTS: The VTACH study was a prospective, open, randomized controlled trial, undertaken in 16 European centers, comparing defibrillator implantation with and without ventricular tachycardia (VT) ablation in patients with stable VT, previous myocardial infarction, and reduced left-ventricular ejection fraction. Of the 52 patients in the ablation group, 7 (13%) did not receive VT ablation and 19% of patients assigned to implantable cardioverter defibrillator (ICD) only treatment group crossed over and had an ablation. The primary endpoint (first recurrence of any documented VT or ventricular fibrillation [VF]) was reached after a median of 19.5 months in the ablation group and 5.9 months in the ICD only group (P = 0.01). Overall, 685 VT/VF events occurred per year of follow-up in 22 patients of the ablation group and 4,986 events in 43 patients of the control group (P = 0.024). In the ICD only group, median numbers of VT/VF episodes were 25 (IQR 5.8-45.3) and 1.5 (IQR 0-24.8) per patient and year before and after crossover (n = 12), respectively. CONCLUSION: On-treatment analysis of the VTACH study emphasizes the effectiveness of VT ablation in patients receiving ICD treatment because of monomorphic VT post myocardial infarction. VT ablation clearly prolonged time to recurrence of VT/VF episodes and markedly decreased VT/VF burden.

Atrioventricular conduction after transcatheter aortic valve implantation and surgical aortic valve replacement.

INTRODUCTION: Atrioventricular conduction abnormalities (AVCA) may complicate transcatheter aortic valve implantation (TAVI) and surgical aortic valve replacement (SAVR). The aim of this study was to prospectively evaluate AVCA after TAVI and SAVR. METHODS AND RESULTS: Among 50 patients undergoing TAVI and 25 patients undergoing SAVR a continuous 7-day Holter electrocardiogram (ECG) was recorded after the procedure. ECGs during TAVI and 12-lead ECGs before and 1 and 7 days after TAVI and SAVR were analyzed. At baseline, TAVI patients were older (mean 82.1 vs 75.4, P < 0.001), had a longer PR interval (median 200 milliseconds vs 175 milliseconds, P = 0.004) and broader QRS width (median 100 milliseconds vs 80 milliseconds, P = 0.007) than SAVR patients. New AVCA were observed among 29 TAVI patients (58%), mostly new left bundle branch block (76%). Predilatation induced new AVCA in 14 TAVI patients (28%). New AVCA resolved within 24 hours in 15 TAVI patients (30%), and persisted in 14 TAVI (28%) and 3 SAVR patients (12%, P = 0.12). Among patients with persistent QRS width <120 milliseconds during the first 24 hours after TAVI, QRS width remained stable during the remainder of the observation period. During Holter monitoring complete AV block was observed in 4 TAVI patients (8%) and 3 SAVR patients (12%; P = 0.68). CONCLUSIONS: Almost half of AVCA during TAVI are induced by predilatation, but half of them resolve within 24 hours. Persistent AVCA are more frequently observed after TAVI than SAVR. If QRS width is below 120 milliseconds the first day after TAVI, the risk of late AVCA seems low.

Long-term outcome of ablative therapy of post-operative atrial tachyarrhythmias in patients with tetralogy of Fallot: a European multi-centre study.

AIM: Post-operative atrial tachyarrhythmias (AT) in patients with tetralogy of Fallot (ToF) are associated with congestive heart failure, stroke, and cardiac death. Effective treatment is therefore essential. The aim of the study is to evaluate long-term outcome of ablative therapy of AT in ToF patients and to study characteristics of AT recurrences. METHODS AND RESULTS: Tetralogy of Fallot patients (N = 38, age 43 ± 12 years) referred for ablation of post-operative AT, appearing 26 ± 10 years after complete repair, were studied. Electro-anatomical/entrainment mapping was performed prior to ablation. Successful ablation was defined as (i) achievement of bi-directional conduction block for isthmus-dependent atrial flutter (IDAF), (ii) termination during ablation for intra-atrial reentrant tachycardia (IART) and focal atrial tachycardia (FAT). Fifty-two AT were ablated, including 37 IDAF [cycle length (CL) 294 ± 70 ms], 11 IART (CL 295 ± 46 ms), and 4 FAT (CL 371 ± 93 ms). Ablation was successful in 98%. Fifty-one of 52 AT involved the cavo-tricuspid isthmus and/or the area between scar tissue related to prior atriotomy incisions and the inferior caval vein. Multiple AT developed in 11 patients, with different mechanisms in 9. After 45 ± 24 months, 32 patients were in sinus rhythm; 5 used anti-arrhythmic drugs. CONCLUSION: Ablative therapy of AT in ToF patients is an effective curative treatment modality with a high procedural success rate. Sinus rhythm during long-term follow-up was obtained in the majority of patients. Fifty-one of 52 AT originated from sites related to surgical incisions created at complete repair, suggesting that extending the atriotomy incision towards the inferior caval vein during cardiac surgery combined with surgical ablation of the cavo-tricuspid isthmus will be effective in preventing development of AT.

Catheter ablation of stable ventricular tachycardia before defibrillator implantation in patients with coronary heart disease (VTACH): a multicentre randomised controlled trial.

BACKGROUND: In patients with ventricular tachycardia (VT) and a history of myocardial infarction, intervention with an implantable cardioverter defibrillator (ICD) can prevent sudden cardiac death and thereby reduce total mortality. However, ICD shocks are painful and do not provide complete protection against sudden cardiac death. We assessed the potential benefit of catheter ablation before implantation of a cardioverter defibrillator. METHODS: The Ventricular Tachycardia Ablation in Coronary Heart Disease (VTACH) study was a prospective, open, randomised controlled trial, undertaken in 16 centres in four European countries. Patients aged 18-80 years were eligible for enrolment if they had stable VT, previous myocardial infarction, and reduced left-ventricular ejection fraction (LVEF; 30%). Patients were followed up for at least 1 year. The primary endpoint was the time to first recurrence of VT or ventricular fibrillation (VF). Analysis was by intention to treat (ITT). This study is registered with ClinicalTrials.gov, number NCT00919373. FINDINGS: 107 patients were included in the ITT population (ablation group, n=52; control group, n=55). Two patients (one in each group) withdrew consent immediately after randomisation without any follow-up data and one patient (ablation group) was excluded because of a protocol violaton. Mean follow-up was 22.5 months (SD 9.0). Time to recurrence of VT or VF was longer in the ablation group (median 18.6 months [lower quartile 2.4, upper quartile not determinable]) than in the control group (5.9 months [IQR 0.8-26.7]). At 2 years, estimates for survival free from VT or VF were 47% in the ablation group and 29% in the control group (hazard ratio 0.61; 95% CI 0.37-0.99; p=0.045). Complications related to the ablation procedure occurred in two patients; no deaths occurred within 30 days after ablation. 15 device-related complications requiring surgical intervention occurred in 13 patients (ablation group, four; control group, nine). Nine patients died during the study (ablation group, five; control group, four). INTERPRETATION: Prophylactic VT ablation before defibrillator implantation seemed to prolong time to recurrence of VT in patients with stable VT, previous myocardial infarction, and reduced LVEF. Prophylactic catheter ablation should therefore be considered before implantation of a cardioverter defibrillator in such patients. FUNDING: St Jude Medical.

Catheter ablation of arrhythmias in ebstein's anomaly: a multicenter study.

INTRODUCTION: In patients with Ebstein's anomaly (EA) arrhythmias are frequently encountered. Although most arrhythmias can be targeted with catheter ablation, specific issues render the procedure more challenging in EA. This study examines the mechanisms of the different arrhythmias related to EA and the outcome after catheter ablation. METHODS AND RESULTS: Clinical and procedural data of catheter ablation in patients with EA in 4 European centers were analyzed. In 32 patients (mean age 24 ± 15 years), 34 accessory pathways (APs), 8 intra-atrial reentry tachycardias (IART), 5 cavotricuspid isthmus-dependent atrial flutter (CTI-AFL), 2 focal atrial tachycardias, and 1 atrioventricular nodal reentry tachycardia were ablated. In 11 patients (34%), multiple ablation targets were present. Eighteen patients (56%) required multiple procedures either for repeat ablation of the same arrhythmia (n = 12), ablation of a different arrhythmia (n = 4), or both re-ablation of the same and of a different arrhythmia (n = 2). Procedural success rate after first ablation was 80% for APs and CTI-AFL ablation, and 100% for IART ablation. Redo procedures were necessary in 40% of the patients after ablation of an APs, and in 60% after CTI-AFL ablation, but in none of the patient with IART ablation. CONCLUSION: Most arrhythmias related to EA are amenable to catheter ablation. However, ablation procedures are challenging and the need for repeat procedure is particularly high, because some patients have multiple ablation targets and because of technical issues in relation with the dysplastic tricuspid annulus. In addition, several patients develop other arrhythmia mechanisms following ablation.

Catheter ablation of atrial fibrillation as first-line therapy--a single-centre experience.

AIMS: The aims of the study were (i) to assess the characteristics of patients selected for atrial fibrillation (AF) ablation as first-line therapy, (ii) to identify current clinical criteria for such a strategy, and (iii) to analyse the outcome compared with patients who had failure of antiarrhythmic drug (AAD) therapy prior to ablation. METHODS AND RESULTS: Consecutive patients undergoing ablation of AF were included in a prospective registry. Serial long-term electrocardiogram monitoring and clinical follow-up were performed after 3, 6, and 12 months. Out of 434 patients, 17% underwent AF catheter ablation as first-line therapy (AAD-), and 83% had undergone at least one AAD trial (AAD+). In AAD- patients, the reasons for this strategy were: (i) patient preference, n= 51 (71%); (ii) contra-indication to AAD, n= 21 (29%). Atrial fibrillation duration prior to ablation was shorter (52 ± 54 vs. 78 ± 81 months, P= 0.005), and the percentage of patients hospitalized for AF (32% vs. 48%, P= 0.01) was lower in AAD- patients. Long-term multiple procedure success rate (78% vs. 64%, P= 0.03) was higher in the AAD- group, and there were less repeat ablations in this group (21% vs. 38%, P= 0.01). CONCLUSION: Catheter ablation was first-line therapy of AF in a significant number of patients, according either to patient preference or to medical factors, and this had important implications. Ablative therapy was performed at an earlier stage of the disease, and was associated with a significantly higher success rate and with a decreased need for repeat procedures.

An indicator of sudden cardiac death during brief coronary occlusion: electrocardiogram QT time and the role of collaterals.

AIMS: The coronary collateral circulation has a beneficial role regarding all-cause and cardiac mortality. Hitherto, the underlying mechanism has not been clarified. The aim of this prospective study was to assess the effect of the coronary collateral circulation on electrocardiogram (ECG) QTc time change during short-term myocardial ischaemia. METHODS AND RESULTS: A total of 150 patients (mean age 63 +/- 11 years, 38 women) were prospectively included in this study. An ECG was recorded at baseline and during a standardized 1 min coronary balloon occlusion. QT interval was measured before, during, and after balloon occlusion and was corrected for heart rate (QTc). Simultaneously obtained collateral flow index (CFI), expressing collateral flow relative to normal anterograde flow, was determined based on intracoronary pressure measurements. During occlusion of the left anterior descending coronary artery mean QTc interval increased from 422 +/- 33 to 439 +/- 36 ms (P < 0.001), left circumflex occlusion led to an increase from 414 +/- 32 to 427 +/- 27 ms (P < 0.001). QTc was not influenced by occlusion of the right coronary artery (RCA) (417 +/- 35 and 415 +/- 34 ms, respectively; P = 0.863). QTc change during occlusion of the left coronary artery was inversely correlated with CFI (R(2) = 0.122, P = 0.0002). CONCLUSION: Myocardial ischaemia leads to QT prolongation during a controlled 1 min occlusion of the left, but not the RCA. QT prolongation is inversely related to collateral function indicating a protective mechanism of human coronary collaterals against cardiac death.

Swiss National Registry on Catheter Ablation Procedures: Changing Trends over the Last 20 Years.

The Swiss Ablation Registry provides a national database for electrophysiologic studies and catheter ablations. We analyzed the database to provide an in-depth look at changing trends over the last 20 years. During the study period a total of 78622 catheter ablations (age 61.0 ± 1.2 years; 63.7% male) were performed in 29 centers. The number of ablations increased by approximately ten-fold in 20 years. Ablation for atrial fibrillation (AF) was the main driver behind this increase, with more than hundred-fold (39.7% of all ablations in 2019). Atrioventricular-nodal-reentrant-tachycardia (AVNRT) and accessory pathways, being the main indications for ablation in 2000 (44.1%/25.1%, respectively), made up of only a small proportion (15.2%/3.5%,) respectively in 2019. Fluoroscopy, ablation, and procedure durations were reduced for all ablations over time. The highest repeat ablations were performed for ventricular tachycardia and AF (24.4%/24.3%). The majority of ablations (63.0%) are currently performed in private hospitals and non-university public hospitals whereas university hospitals had dominated (82.4%) at the turn of the century. A pronounced increase in the number of catheter ablations in Switzerland was accompanied by a marked decrease in fluoroscopy, ablation, and procedure durations. We observed a shift toward more complex procedures in older patients with comorbidities.

Three-dimensional electroanatomic entrainment map in atypical atrial flutter late after heart transplantation.

Atrial flutter in the donor part of orthotopic heart transplants has been reported and successfully treated by radiofrequency ablation of the cavotricuspid isthmus, but mapping and ablation of atypical flutter circuits may be challenging.(1) Entrainment mapping has been used in combination with activation mapping to define the mechanism of atypical atrial flutter. Here, we report a case where colour-coded three-dimensional (3D) entrainment mapping allowed us to accurately determine and visualize the 3D location of the reentrant circuit and to plan the ablation of a left atrial flutter without the need for activation mapping.

Desmosomal gene analysis in arrhythmogenic right ventricular dysplasia/cardiomyopathy: spectrum of mutations and clinical impact in practice.

AIMS: Five desmosomal genes have been recently implicated in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) but the clinical impact of genetics remains poorly understood. We wanted to address the potential impact of genotyping. METHODS AND RESULTS: Direct sequencing of the five genes (JUP, DSP, PKP2, DSG2, and DSC2) was performed in 135 unrelated patients with ARVD/C. We identified 41 different disease-causing mutations, including 28 novel ones, in 62 patients (46%). In addition, a genetic variant of unknown significance was identified in nine additional patients (7%). Distribution of genes was 31% (PKP2), 10% (DSG2), 4.5% (DSP), 1.5% (DSC2), and 0% (JUP). The presence of desmosomal mutations was not associated with familial context but was associated with young age, symptoms, electrical substrate, and extensive structural damage. When compared with other genes, DSG2 mutations were associated with more frequent left ventricular involvement (P = 0.006). Finally, complex genetic status with multiple mutations was identified in 4% of patients and was associated with more frequent sudden death (P = 0.047). CONCLUSION: This study supports the use of genetic testing as a new diagnostic tool in ARVC/D and also suggests a prognostic impact, as the severity of the disease appears different according to the underlying gene or the presence of multiple mutations.

Palliative care in heart failure: a position statement from the palliative care workshop of the Heart Failure Association of the European Society of Cardiology.

Heart failure is a serious condition and equivalent to malignant disease in terms of symptom burden and mortality. At this moment only a comparatively small number of heart failure patients receive specialist palliative care. Heart failure patients may have generic palliative care needs, such as refractory multifaceted symptoms, communication and decision making issues and the requirement for family support. The Advanced Heart Failure Study Group of the Heart Failure Association of the European Society of Cardiology organized a workshop to address the issue of palliative care in heart failure to increase awareness of the need for palliative care. Additional objectives included improving the accessibility and quality of palliative care for heart failure patients and promoting the development of heart failure-orientated palliative care services across Europe. This document represents a synthesis of the presentations and discussion during the workshop and describes recommendations in the area of delivery of quality care to patients and families, education, treatment coordination, research and policy.

Gender differences in patients referred for atrial fibrillation management to a tertiary center.

BACKGROUND: Atrial fibrillation (AF) ablation is less frequently performed in women than in men. Although the prevalence of AF is slightly higher in men, this does not fully account for the lower number of AF ablations performed in women. This study sought to examine the effect of gender on referral for AF and subsequent AF management. METHODS: Consecutive patients referred to our tertiary arrhythmia outpatient clinic for AF management were retrospectively analyzed. RESULTS: Of 264 patients referred, only 27% were women. Women were older than men (63 +/- 9 vs 58 +/- 11 years, P = 0.002), more often had paroxysmal AF (78% vs 63% in men, P = 0.022), and women more frequently complained about palpitations (71% vs 49%, P = 0.002). In addition, they had more often experienced amiodarone side effects than men (56% vs 36%, P = 0.046). In this referred population, there was no difference in the proportion of women and men undergoing AF ablation immediately following the initial evaluation (21% vs 25%, P = ns), at any time during the follow-up (38% vs 44%, P = ns), and there was no difference in the proportion of patients undergoing atrioventricular node ablation in both sexes (6% of women vs 3% of men, P = ns). CONCLUSIONS: There is an important difference in the proportion of men and women referred for management of AF in a specialized outpatient arrhythmia clinic, with women being referred three times less often than men. However, there is no gender-related difference in the subsequent treatment decisions. These findings emphasize the importance of focusing on management of symptomatic AF in women.

High risk of heart failure associated with desmoglein-2 mutations compared to plakophilin-2 mutations in arrhythmogenic right ventricular cardiomyopathy/dysplasia.

BACKGROUND: Previous studies suggested that genetic status affects the clinical course of arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) patients. The aim of this study was to compare the outcome of desmoglein-2 (DSG2) mutation carriers to those who carry the plakophilin-2 (PKP2) mutation, the most common ARVC/D-associated gene. METHODS AND RESULTS: Consecutive ARVC/D patients carrying a pathogenic mutation in PKP2 or DSG2 were selected from a national ARVC/D registry. The cumulative freedom from sustained ventricular arrhythmia and cardiac transplantation/death from heart failure (HF) during follow-up was assessed, compared between PKP2 and DSG2, and predictors for ventricular arrhythmia and HF events determined. Overall, 118 patients from 78 families were included: 27 (23%) carried a DSG2 mutation and 91 (77%) a PKP2 mutation. There were no significant differences between DSG2 and PKP2 mutation carriers concerning gender, proband status, age at diagnosis, T-wave inversion, or right ventricular dysfunction at baseline. DSG2 patients displayed more frequent epsilon wave (37% vs. 17%, P = 0.048) and left ventricular dysfunction at diagnosis (54% vs. 10%, P < 0.001). During a median follow-up of 5.6 years (2.5-16), DSG2 and PKP2 mutation carriers displayed a similar risk of sustained ventricular arrhythmia (log-rank P = 0.20), but DSG2 mutation carriers were at higher risk of transplantation/HF-related death (log-rank P < 0.001). The presence of a DSG2 mutation vs. PKP2 mutation was a predictor of transplantation/HF-related death in univariate Cox analysis (P = 0.0005). CONCLUSIONS: In this multicentre cohort, DSG2 mutation carriers were found to be at high risk of end-stage HF compared to PKP2 mutation carriers, supporting careful haemodynamic monitoring of these patients. The benefit of early HF treatment needs to be assessed in DSG2 carriers.