Anderson-Fabry disease management: role of the cardiologist.

Anderson-Fabry disease (AFD) is a lysosomal storage disorder characterized by glycolipid accumulation in cardiac cells, associated with a peculiar form of hypertrophic cardiomyopathy (HCM). Up to 1% of patients with a diagnosis of HCM indeed have AFD. With the availability of targeted therapies for sarcomeric HCM and its genocopies, a timely differential diagnosis is essential. Specifically, the therapeutic landscape for AFD is rapidly evolving and offers increasingly effective, disease-modifying treatment options. However, diagnosing AFD may be difficult, particularly in the non-classic phenotype with prominent or isolated cardiac involvement and no systemic red flags. For many AFD patients, the clinical journey from initial clinical manifestations to diagnosis and appropriate treatment remains challenging, due to late recognition or utter neglect. Consequently, late initiation of treatment results in an exacerbation of cardiac involvement, representing the main cause of morbidity and mortality, irrespective of gender. Optimal management of AFD patients requires a dedicated multidisciplinary team, in which the cardiologist plays a decisive role, ranging from the differential diagnosis to the prevention of complications and the evaluation of timing for disease-specific therapies. The present review aims to redefine the role of cardiologists across the main decision nodes in contemporary AFD clinical care and drug discovery.

[Atrial fibrillation: 2023 novelties for the general practitioner]. / Fibrillation auriculaire : nouveautés 2023 pour la médecine de premier recours.

Atrial fibrillation (AF) is the most frequent tachyarrhythmia with a significant morbimortality. The diagnosis is based on a 12 lead ECG. New technologies such as connected watches have shown similar sensibility and specificity. The new 4S scheme (Stroke risk, Symptoms, Severity of AF burden and Substrate) allows a global evaluation. Rhythm control mainly by catheter ablation is increasingly indicated. Management of cardiovascular risk factors is an essential part of the treatment of these patients.

La fibrillation auriculaire (FA) est la tachyarythmie la plus fréquente avec une morbimortalité conséquente. Le diagnostic se fait généralement par un ECG 12 pistes. Cependant, de nouvelles technologies, comme les montres connectées, ont montré d'excellents résultats avec une sensibilité et spécificité équivalentes. L'algorithme des 4S (risque d'AVC (stroke risk), sévérité des symptômes (symptoms), temporalité de la FA (severity of AF burden) et comorbidités (substrate)) permet une évaluation globale de la FA propre à chaque patient. L'indication à la stratégie de contrôle du rythme, principalement l'ablation par cathéter, devient plus fréquente. Le contrôle des facteurs de risque cardiovasculaire fait partie intégrante du traitement.

Impact of SMART Pass filter in patients with ajmaline-induced Brugada syndrome and subcutaneous implantable cardioverter-defibrillator eligibility failure: results from a prospective multicentre study.

AIMS: Ajmaline challenge can unmask subcutaneous implantable cardioverter-defibrillator (S-ICD) screening failure in patients with Brugada syndrome (BrS) and non-diagnostic baseline electrocardiogram (ECG). The efficacy of the SMART Pass (SP) filter, a high-pass filter designed to reduce cardiac oversensing (while maintaining an appropriate sensing margin), has not yet been assessed in patients with BrS. The aim of this prospective multicentre study was to investigate the effect of the SP filter on dynamic Brugada ECG changes evoked by ajmaline and to assess its value in reducing S-ICD screening failure in patients with drug-induced Brugada ECGs. METHODS AND RESULTS: The S-ICD screening with conventional automated screening tool (AST) was performed during ajmaline challenge in subjects with suspected BrS. The S-ICD recordings were obtained before, during and after ajmaline administration and evaluated by the means of a simulation model that emulates the AST behaviour with and without SP filter. A patient was considered suitable for S-ICD if at least one sensing vector was acceptable in all tested postures. A sensing vector was considered acceptable in the presence of QRS amplitude >0.5 mV, QRS/T-wave ratio >3.5, and sense vector score >100. Of the 126 subjects (mean age: 42 ± 14 years, males: 61%, sensing vectors: 6786), 46 (36%) presented with an ajmaline-induced Brugada type 1 ECG. Up to 30% of subjects and 40% of vectors failed the screening during the appearance of Brugada type 1 ECG evoked by ajmaline. The S-ICD screening failure rate was not significantly reduced in patients with Brugada ECGs when SP filter was enabled (30% vs. 24%). Similarly, there was only a trend in reduction of vector-failure rate attributable to the SP filter (from 40% to 36%). The most frequent reason for screening failure was low QRS amplitude or low QRS/T-wave ratio. None of these patients was implanted with an S-ICD. CONCLUSION: Patients who pass the sensing screening during ajmaline can be considered good candidates for S-ICD implantation, while those who fail might be susceptible to sensing issues. Although there was a trend towards reduction of vector sensing failure rate when SP filter was enabled, the reduction in S-ICD screening failure in patients with Brugada ECGs did not reach statistical significance. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov Unique Identifier NCT04504591.

Significance of post-pacing intervals shorter than tachycardia cycle length for successful catheter ablation of atypical flutter.

AIMS: During entrainment mapping of macro-reentrant tachycardias, the time difference (dPPI) between post-pacing interval (PPI) and tachycardia cycle length (TCL) is thought to be a function of the distance of the pacing site to the re-entry circuit and dPPI < 30 ms is considered within the re-entry circuit. This study assessed the importance of PPI < TCL as a successful target for atypical flutter ablation. METHODS AND RESULTS: A total of 177 ablation procedures were investigated. Surface electrocardiograms (ECGs) were evaluated and combined activation and entrainment mapping were performed to choose ablation sites. Each entrainment sequence immediately preceding static radiofrequency delivery at the same site was analysed. A total of 545 entrainment sequences were analysed. dPPI < 0 ms was observed in 45.3% (247/545) sequences. Ablation resulted in tachycardia termination more often at sites with dPPI < 0 (27.8% vs. 14.5%, P < 0.001) and with a progressively increasingly inverse correlation between dPPI duration and ablation success [odds ratio (OR): 0.974; 95% confidence interval (CI) 0.960-0.988; P < 0.001]. Tachycardia termination or cycle length prolongation also occurred more often at sites with dPPI < 0 (50.6% vs. 33.2%, P < 0.001) and with a similar inverse correlation with dPPI duration (OR: 0.972; 95% CI 0.960-0.984; P < 0.001). Twelve-lead synchronous isoelectric intervals were observed in 64.4% (163/253) flutter ECGs and were associated with a dPPI < 0 (75.3% vs. 55.8%, P < 0.001). CONCLUSION: When combined with activation mapping, a negative dPPI is a more effective parameter for identifying a target for successful ablation compared to a dPPI = 0-30 ms. Its occurrence is associated with a critical small narrow slow-conducting isthmus at the target site.

Impact of contact force sensing technology on outcome of catheter ablation of idiopathic pre-mature ventricular contractions originating from the outflow tracts.

AIMS: Catheter ablation of frequent idiopathic pre-mature ventricular contractions (PVC) is increasingly performed. While potential benefits of contact force (CF)-sensing technology for atrial fibrillation ablation have been assessed in several studies, the impact of CF-sensing on ventricular arrhythmia ablation remains unknown. This study aimed to compare outcomes of idiopathic outflow tract PVC ablation when using standard ablation catheters as opposed to CF-sensing catheters. METHODS AND RESULTS: In a retrospective multi-centre study, unselected patients undergoing catheter ablation of idiopathic outflow tract PVCs between 2013 and 2016 were enrolled. All procedures were performed using irrigated-tip ablation catheters and a 3D electro-anatomical mapping system. Sustained ablation success was defined as a ≥80% reduction of pre-procedural PVC burden determined by 24 h Holter ECG during follow-up. Overall, 218 patients were enrolled (median age 52 years, 51% males). Baseline and procedural data were similar in the standard ablation (24%) and the CF-sensing group (76%). Overall, the median PVC burden decreased from 21% (IQR 10-30%) before ablation to 0.2% (IQR 0-3.0%) after a median follow-up of 2.3 months (IQR 1.4-3.9 months). The rates of both acute (91% vs. 91%, P = 0.94) and sustained success (79% vs. 74%, P = 0.44) were similar in the standard ablation and the CF-sensing groups. No differences were observed in subgroups according to arrhythmia origin from the RVOT (65%) or LVOT (35%). Complications were rare (1.8%) and evenly distributed between the two groups. CONCLUSION: The use of CF-sensing technology is not associated with increased success rate nor decreased complication rate in idiopathic outflow tract PVC ablation.

Sustained and self-terminating atrial fibrillation induced immediately after pulmonary vein isolation exhibit differences in coronary sinus electrical activity from onset.

INTRODUCTION: Little data exists on the electrophysiological differences between sustained atrial fibrillation (sAF; >5 minutes) vs self-terminating nonsustained AF (nsAF; <5 minutes). We sought to investigate the electrophysiological characteristics of coronary sinus (CS) activity during postpulmonary vein isolation (PVI) sAF vs nsAF. METHODS AND RESULTS: We studied 142 patients post-PVI for paroxysmal AF (PAF). In a 50-patient subset, CS electrograms in the first 30 seconds of induced AF were analyzed manually. A custom-made algorithm for automated electrogram annotation was derived for validation on the whole patient set. In patients with sAF post-PVI, CS fractionated potentials were ablated. Manual analysis showed that patients with sAF exhibited higher activation pattern variability (2.1 vs 0.5 changes/sec; P < .001); fewer proximal-to-distal wavefronts (25 vs 61%; P < .001); fewer unidirectional wavefronts (60 vs 86%; P < .001); more pivot locations (4.3 vs 2.1; P < .001); shorter cycle lengths (190 vs 220 ms; P < .001); and shorter cumulative isoelectric segments (35 vs 44%; P = .045) compared to nsAF. These observations were confirmed on the whole study population by automated electrogram annotation and sample entropy computation (SampEn: 0.29 ± 0.15 in sAF vs 0.15 ± 0.05 in nsAF; P < .0001). The derived model predicted sAF with 78% sensitivity, 88% specificity; agreement with manual model: 88% (Cohen's kappa= 0.76). CS defragmentation resulted in AF termination or noninducibility in 49% of sAF. CONCLUSION: In PAF patients post-PVI, induced sAF shows greater activation sequence variability, shorter cycle length, and higher SampEn in the CS compared to nsAF. Automated electrogram annotation confirmed these results and accurately distinguished self-terminating nsAF episodes from sAF based on 30-second recordings at AF onset.

[New onset atrial fibrillation in patients hospitalized with sepsis : is there an indication for anticoagulation ?] / Fibrillation auriculaire inaugurale lors d'un sepsis : y a-t-il une indication à une anticoagulation ?

Atrial fibrillation (AF) is the most common cardiac arrhythmia in the general population and in patients with sepsis hospitalized in intensive care. The indication for long-term anticoagulation is based on expert recommendations that take into account data from the general population and thus recommend therapeutic anticoagulation for AF longer than 48 hours. However, a majority of new onset AF in intensive care seem to last less than 48 hours and additional risk factors such as the type of sepsis, the drugs administered as well as the presence of a central venous catheters, are involved. Moreover, the increased of minor and major hemorrhage renders it difficult to apply the usual recommendations. In this literature review, we will focus on the various risk factors, prognosis, and indication of long-term anticoagulation in the new onset AF in this population.

La fibrillation auriculaire (FA) est l'arythmie cardiaque la plus fréquente chez les patients en sepsis admis aux soins intensifs. L'indication à une anticoagulation au long cours se fonde sur des recommandations d'experts qui proposent une anticoagulation thérapeutique pour les FA de plus de 48 heures, compte tenu de données populationnelles. Or, la majorité de ces FA inaugurales semblent durer moins longtemps. La sévérité du sepsis, les médicaments administrés, la présence d'une voie veineuse centrale sont autant de facteurs de risque de survenue. S'y ajoute un risque hémorragique accru, rendant difficile l'application des recommandations usuelles. Nous allons, dans cette revue de littérature, nous intéresser aux facteurs de risque, au pronostic et à l'indication d'une anticoagulation au long cours de la FA inaugurale dans cette population.

[Isolated left ventricular hypertrophy : is it a Fabry disease?] / Hypertrophie ventriculaire gauche isolée : et si c'était une maladie de Fabry ?

Fabry disease, an X-linked disease, results from a deficiency of the lysosomal enzyme alpha-galactosidase A, which causes glycosphingolipids accumulation in the body. On the basis of the residual enzymatic activity level, a classical, severe multisystemic form and an attenuated cardiac variant form are distinguished. In all cases, patients can develop hypertrophic cardiomyopathy in adulthood, the severity of which is the leading cause of morbidity and mortality of the disease. The cardiomyopathy is usually isolated in the cardiac variant form, the most common form of the disease, and should be suspected in the presence of relatively specific ECG, echocardiographic and MRI characteristics.

La maladie de Fabry est liée au chromosome X et résulte d'un déficit de l'enzyme lysosomale alpha-galactosidase A, responsable de l'accumulation de glycosphingolipides dans l'organisme. On distingue une forme classique, multisystémique, sévère, et une forme atténuée ou variant cardiaque. Dans tous les cas, les adultes peuvent développer une cardiomyopathie hypertrophique (CMH), principale cause de morbi-mortalité de la maladie. Dans la forme variant cardiaque, la plus fréquente de la maladie, la CMH est généralement isolée. Elle peut être suspectée en présence de certaines anomalies ECG, échocardiographiques et/ou IRM, et amener à un dépistage.

European expert consensus statement on therapeutic goals in Fabry disease.

BACKGROUND: Fabry disease, an inherited lysosomal storage disorder, causes multi-organ pathology resulting in substantial morbidity and a reduced life expectancy. Although Fabry disease is an X-linked disorder, both genders may be affected, but generally to a lesser extent in females. The disease spectrum ranges from classic early-onset disease to non-classic later-onset phenotypes, with complications occurring in multiple organs or being confined to a single organ system depending on the stage of the disease. The impact of therapy depends upon patient- and disease-specific factors and timing of initiation. METHODS: A European panel of experts collaborated to develop a set of organ-specific therapeutic goals for Fabry disease, based on evidence identified in a recent systematic literature review and consensus opinion. RESULTS: A series of organ-specific treatment goals were developed. For each organ system, optimal treatment strategies accounted for inter-patient differences in disease severity, natural history, and treatment responses as well as the negative burden of therapy and the importance of multidisciplinary care. The consensus therapeutic goals and proposed patient management algorithm take into account the need for early disease-specific therapy to delay or slow the progression of disease as well as non-specific adjunctive therapies that prevent or treat the effects of organ damage on quality of life and long-term prognosis. CONCLUSIONS: These consensus recommendations help advance Fabry disease management by considering the balance between anticipated clinical benefits and potential therapy-related challenges in order to facilitate individualized treatment, optimize patient care and improve quality of life.

[ECG : interpretation and clinical significance of P-wave abnormalities]. / ECG : interprétation et signification clinique des anomalies de l'onde P.

The ECG provides information about heart rhythm and myocardial integrity, including the atria. The sinus P wave exhibits a 0­90° axis and a generally biphasic morphology in lead V1. An amplitude >2 mm in lead II and >1 mm in lead V1 is a specific sign of right atrial enlargement, often related to pulmonary disease or pulmonary hypertension. Interatrial block (IAB) is defined as a P-wave ≥120 ms with, in the advanced form, a biphasic morphology in inferior leads. It is most commonly seen in the context of advanced age, cardiovascular risk factors, coronary artery disease or valvulopathies. IAB is a risk factor for supraventricular tachyarrhythmias, stroke, left ventricular dysfunction and mortality. The identification of a P-wave abnormality has prognostic implications and should trigger the search for associated conditions.

L'ECG (électrocardiogramme) renseigne sur le rythme et l'intégrité du myocarde, y compris atrial. L'onde P sinusale présente un axe de 0­90° et une morphologie généralement biphasique en V1. Une amplitude > 2 mm en II et > 1 mm en V1 est un signe spécifique de dilatation de l'oreillette droite, marqueur de pneumopathie ou d'hypertension pulmonaire. Le bloc interatrial (BIA) est défini par une onde P ≥ 120 ms avec, dans la forme avancée, une morphologie biphasique en dérivations inférieures. Il est souvent observé dans un contexte d'âge avancé, de facteurs de risque cardiovasculaire, de maladie coronarienne ou de valvulopathie. Un BIA augmente le risque d'arythmie, d'AVC, de dysfonction ventriculaire et de mortalité. Identifier une altération de l'onde P a une importance pronostique et doit susciter la recherche de pathologies associées.

[Assessment of QT interval duration in patients with wide QRS]. / Evaluation de l'intervalle QT en présence d'un élargissement du QRS.

The QT interval is the most widely used ECG parameter for the assessement of myocardial repolarization and the risk of torsades de pointes. Measured from the beginning of the QRS complex, it is also influenced by the duration of the depolarization phase. The presence of ventricular conduction abnormalities or a widening of the QRS during ventricular pacing prolongs the QT interval, even if the repolarization phase is normal. Consequently, it is difficult to assess the QT interval in this population and to estimate the risk of torsades de pointes. In this article, we would like to give an overview of the current literature as guidance to the measurement of the QT interval in the presence of a QRS widening.

Second-generation cryoballoon ablation for paroxysmal atrial fibrillation: 1-year follow-up.

BACKGROUND: The novel cryoballoon Advance (CB-A) has proven to achieve significantly lower temperatures and faster pulmonary vein isolation (PVI) times in comparison with the first-generation device. Although acutely very effective, to the best of our knowledge, data on mid-term clinical follow-up is lacking. AIMS: The aim of the study was to analyse the freedom from recurrence of atrial fibrillation (AF) on a 1-year follow-up period, in a series of consecutive patients having undergone PVI with the CB-A for paroxysmal AF (PAF). METHODS AND RESULTS: Forty-two patients [30 male (71%); mean age: 57.9 ± 21.1 years] were included. All patients underwent a procedure with the large 28 mm CB-A. A total 168 PVs were depicted on the pre-procedural computed tomography scan. All PVs (100%) could be isolated with the CB only. The freedom from AF off-antiarrhythmic drug treatment after a single procedure was 78% of patients at a mean 11.6 ± 2.0 months follow-up. If considering a blanking period (BP) of 3 months, success rate was 83%. Phrenic nerve palsy (PNP) was the most frequent complication occurring in 19% of individuals. CONCLUSION: The CB-A is very effective in producing PVI and affords freedom from AF at 12 months follow-up in 83% of patients affected by drug-resistant PAF following a 3-month BP. The most frequent complication observed was PNP which occurred in 19% of patients. All PNP reverted during follow-up.

Anatomical and Electrophysiological Characteristics of Dual-Loop Re-Entry in Atypical Atrial Flutter: Implications for Mapping and Catheter Ablation.

Background: Atypical atrial flutter (AFL) can be challenging to ablate, especially when involving dual-loop re-entry. We sought to assess the electroanatomical characteristics of single- and dual-loop AFLs in patients undergoing catheter ablation. Methods: We analyzed 25 non-cavotricuspid isthmus-dependent macro-re-entrant AFL in 19 consecutive patients. Three-dimensional high-density activation mapping was performed, and active re-entry loops were confirmed by entrainment mapping. Results: Of 25 AFLs (24 left, 1 right atrial), 13 (52%) exhibited dual-loop re-entry. The most common circuits included, in 6/13 (46% of dual loops), a perimitral re-entry with a second loop around the right/left pulmonary veins (PV) and, in 6/13 (46%), involved a right PV ostium with a second loop around either a functional conduction block or another PV. Ablation at the common isthmus of dual-loop AFLs and at the critical isthmus of single-loop AFLs terminated the arrhythmia more frequently than ablation at a secondary isthmus of dual-loop AFLs (5/6 (83%) and 8/11 (73%) versus 1/8 (13%), respectively, p = 0.013). Conclusions: More than half of AFLs exhibited a dual-loop re-entrant mechanism. Most critical isthmuses were found at the mitral isthmus, the left atrial roof or right PV ostia. Ablation targeting the common isthmus resulted in a higher termination rate.

Fabry disease: development and progression of left ventricular hypertrophy despite long-term enzyme replacement therapy.

BACKGROUND: Enzyme replacement therapy (ERT) may halt or attenuate disease progression in patients with Anderson-Fabry disease (AFD). However, whether left ventricular hypertrophy (LVH) can be prevented by early therapy or may still progress despite ERT over a long-term follow-up is still unclear. METHODS: Consecutive patients with AFD from the Independent Swiss-Fabry Cohort receiving ERT who were at least followed up for 5 years were included. Cardiac progression was defined as an increase of >10 g/m2 in left ventricular mass index (LVMI) between the first and the last available follow-up transthoracic echocardiography. RESULTS: 60 patients (35 (23-48) years, 39 (65%) men) were followed up for 10.5 (7.2-12.2) years. 22 had LVH at ERT start (LVMI of 150±38 g/m2). During follow-up, 22 (36%, 34±15 years) had LVMI progression of 12.1 (7-17.6) g/m2 per 100 patient-years, of these 7 (11%, 29±13 years) with no LVH at baseline. Three of them progressed to LVH. LVMI progression occurred mostly in men (17 of 39 (43%) vs 5 of 21 (24%), p<0.01) and after the age of 30 years (17 of 22 (77%)). LVH at ERT start was associated with LVMI progression (OR 1.3, 95% CI 1.1 to 2.6; p=0.02). A total of 19 (31%) patients experienced a major AFD-related event. They were predominantly men (17 of 19, 89%), older (45±11 vs 32±9 years) with baseline LVH (12 of 19, 63%), and 10 of 19 (52%) presented with LVMI progression. CONCLUSIONS: Over a median follow-up of >10 years under ERT, 36% of the patients still had LVMI cardiac progression, and 32%, predominantly older men, experienced major AFD-related events. LVH at treatment initiation was a strong predictor of LVMI progression and adverse events on ERT.

Cross-sectional baseline analysis of electrocardiography in a large cohort of patients with untreated Fabry disease.

BACKGROUND: Morphology and function of Fabry cardiomyopathy has been previously studied by echocardiography and cardiac magnetic resonance (CMR). However, the value of electrocardiography (ECG) in relation to these two techniques remains largely unknown. METHODS: One hundred fifty genetically confirmed Fabry patients were investigated using a comprehensive clinical workup comprising 12-lead ECG, echocardiography, and CMR. RESULTS: ECG parameters at rest [PR, P wave, QT, QTc, QT dispersion and time interval from the peak to the end of the T wave (Tpeak to Tend)] were normal in the entire cohort and did not distinguish between males and females or stages of cardiomyopathy. A significant positive correlation was found between left ventricular (LV) mass on CMR and both the QRS duration and the LV Sokolow index, with the highest values in male patients with an advanced cardiomyopathy stage. No prediction of late enhancement on CMR (a sign for replacement fibrosis) was possible by a single ECG parameter. However, the absence of ST or T alterations (in 37 of 38 patients) specifically excluded late enhancement on CMR. CONCLUSION: Our data in a large cohort of Fabry patients, including all cardiomyopathy stages, show, in contrast to former assumptions, that ECG parameters are not suitable to stage Fabry cardiomoypathy. Most ECG parameters were normal in the complete cohort. However, the absence of ST or T alterations seems to almost exclude late enhancement on CMR in these patients.

Role of home monitoring in children with implantable cardioverter defibrillators for Brugada syndrome.

AIMS: Implementation of remote home monitoring systems (HM) in clinical practice has become undoubtedly an added value for all patients with implantable cardiac devices. The aim of this study was to investigate the impact of HM in a population of children with Brugada syndrome (BS) who received an implantable cardioverter defibrillator (ICD). METHODS AND RESULTS: Eleven children (age between 6 months and 18 years) implanted with an ICD were followed either by means of HM and with conventional in-hospital visits in our centre. Alerts and/or device-related clinical events were recorded, analysed, and subsequent clinical decisions were made if needed. During an average observation time of 26 months a total of 16 relevant alerts (13 pre-emptive alerts) were recorded in seven patients of our population. One patient experienced appropriate therapies for life-threatening ventricular arrhythmias. Three patients experienced inappropriate therapies due to supraventricular tachycardia and lead dislodgement. By means of HM two patients were discovered to have lead problems because of dislodgement or lead fracture. Mean anticipation of treatment based on the alerts was 76 ± 59 days. CONCLUSION: Remote monitoring systems substantially improve the proper management of children with BS.

ICD function and dysfunction in patients with arrhythmogenic cardiac diseases: the role of home monitoring.

BACKGROUND: Since their implementation in clinical practice, remote home monitoring systems (HM) have undoubtedly become an added value in patients with implantable devices for cardiac rhythm management. The aim of this study was to investigate the impact of HM on clinical management and outcome in patients with channelopathies and other arrhythmogenic diseases who received an implantable cardioverter defibrillator (ICD). METHODS: Fifty-four patients (age 6 months--74 years) were followed by means of HM in our ICD clinic. Alerts and/or device-related clinical events were analysed in all patients and subsequent clinical decisions were made if indicated. RESULTS: During an average observation time of 27 months, 46 alerts were received from 32 different patients. Five patients (9%) received appropriate therapies for life-threatening arrhythmias and four patients (8%) inappropriate therapies because ofT wave oversensing or supraventricular tachycardias. Three patients had alerts due to electrical noise (two on the atrial, one on the ventricular channel). Overall, 18 alerts (39%) required a modification of the pharmacological therapy or the programming of the device. Mean anticipation of clinical visits based on the alerts was 92.6 +/- 56 days (median 97, interquartile range 50-150). CONCLUSION: HM substantially improves the clinical management of patients with cardiac arrhythmogenic disease by early recognition of device-related inappropriate therapies and subsequent anticipation of treatment adaptation.

Isolating the pulmonary veins as first-line therapy in patients with lone paroxysmal atrial fibrillation using the cryoballoon.

AIMS: Atrial fibrillation (AF) is the most common arrhythmia encountered in clinical practice. Nowadays, catheter-based ablative approaches are mainly reserved for drug-refractory patients. However, the value of an ablative therapy as a first-line alternative remains elusive. The aim of our study was to analyse the acute procedural success and clinical outcome of patients with lone paroxysmal AF undergoing cryoballoon ablation (CBA) as first-line treatment. METHODS AND RESULTS: Eighteen individuals (mean age 44 ± 9 years, range 23-61 years, 15 males) with lone paroxysmal AF preferring a catheter-based treatment to drug treatment as first-line therapy were consecutively enrolled in our study. Mean left atrial size was 39 ± 4 mm and mean left ventricular ejection fraction 58 ± 3%. After a mean of 2.4 CBA (range 2-4) applications pulmonary vein (PV) isolation could be demonstrated in 70 (97%) PVs. Additional lesions with a focal ablation catheter were needed to isolate one right inferior pulmonary vein and one left superior pulmonary vein in two different patients. At the end of the procedure, all (100%) PVs were isolated. After a 2-month blanking period, 16 patients (89%) were free of symptomatic AF recurrence at a mean follow-up of 14 ± 9 months and without antiarrhythmic drugs (AADs). CONCLUSION: Cryoballoon ablation in patients with lone paroxysmal AF yields a high acute efficacy rate with a great chance of being free of symptomatic AF recurrence without antiarrhythmic drugs on a mid-term follow-up period, when offered as a first-line treatment.

Pulmonary vein isolation during cryoballoon ablation using the novel Achieve inner lumen mapping catheter: a feasibility study.

AIMS: Cryoballoon (CB) ablation has proven very effective in achieving pulmonary vein isolation (PVI). The Achieve catheter (AC) is a novel inner lumen catheter designed to be used in conjunction with the CB, which serves the double purpose of a guidewire and a mapping catheter. We aimed to evaluate the feasibility of CB ablation in conjunction with the novel AC, in terms of PVI and safety in a series of patients affected by drug resistant paroxysmal atrial fibrillation (AF). METHODS AND RESULTS: Seventy patients (49 male) affected by paroxysmal AF were assigned to CB PVI using the AC as a mapping catheter. Patients underwent loop-Holter monitoring 1, 3 and 6 months after ablation. Isolation occurred in 98% of PVs with the CB-AC association without having switching to a regular guidewire. Pulmonary vein isolation could be documented by real-time (RT) recordings in 47% (132) of veins. Time to isolation was significantly longer in PVs exhibiting early left atrium-PV reconnection if compared with veins with sustained isolation (117 ± 25 s vs. 59 ± 25 s; P< 0,005). No serious complications occurred; four transient phrenic nerve palsies occurred all resolving completely before the end of the procedure. CONCLUSION: Cryoballoon ablation in conjunction with the novel AC is feasible, safe, and most importantly affords PVI in nearly all veins without having to switch to a regular guidewire. However, RT recordings could be documented in only 47% of pulmonary veins.

Added value of transoesophageal echocardiography during transseptal puncture performed by inexperienced operators.

AIMS: Transseptal puncture (TP) appears to be safe in experienced hands; however, it can be associated with life-threatening complications. The aim of our study was to demonstrate the added value of routine use of transoesophageal echocardiography (TEE) for the correct positioning of the transseptal system in the fossa ovalis, thus potentially preventing complications during fluoroscopy-guided TP performed by inexperienced operators. METHODS AND RESULTS: Two hundred and five patients undergoing pulmonary vein isolation procedure (PVI) for drug-resistant paroxysmal or persistent atrial fibrillation were prospectively included. When the operator (initially blinded to TEE) assumed that the transseptal system was in a correct position according to fluoroscopical landmarks, the latter was then checked with TEE unblinding the physician. If necessary, further refinement of the catheter position was performed. Refinement >10 mm, or in case of catheter pointing directly at the aortic root or posterior wall were considered as major repositioning. Thirty-four patients required major repositioning. Regression analysis revealed age (P: 0.0001, Wald: 12.9, 95% confidence interval: 1.04-1.16), left atrial diameter (P: 0.01, Wald: 6.6, 95% confidence interval: 1.04-1.34), previous PVI (P: 0.01, Wald: 6.3, 95% confidence interval: 1.31-8.76), and atrial septal thickness (P: 0.03, Wald: 4.5, 95% confidence interval: 1.05-3.4) as independent predictors of major revision with TEE. CONCLUSION: Routine 2D TEE in addition to traditional fluoroscopic TP appears to be very useful to guide the TP assembly in a correct puncture position and thus, to avoid TP-related complications. However, further randomized prospective comparative studies are necessary to support these suggestions.