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1.
Europace ; 26(9)2024 Aug 30.
Artículo en Inglés | MEDLINE | ID: mdl-39148456

RESUMEN

AIMS: Indications and clinical impact of genetic testing for cardiac diseases have increased significantly over the past years. The aim of this physician-based European Heart Rhythm Association (EHRA) survey was to assess current clinical practice and access to genetic testing for cardiac diseases across European Society of Cardiology countries and to evaluate adherence to the 2022 EHRA/HRS/APHRS/LAHRS Expert Consensus Statement on genetic testing. METHODS AND RESULTS: An online questionnaire composed of 28 questions was submitted to the EHRA Research Network and European Reference Network GUARD-Heart healthcare partners and promoted via dedicated social media channels. There were 357 respondents from 69 countries, 40% working in a hospital setting with a cardiac genetic service and/or a dedicated clinic focusing on inherited cardiac diseases and 27% with an onsite genetic laboratory. No genetic testing or low annual rate (<10/year) was declared by 39% of respondents. The majority of respondents (78%) declared issues or limitations to genetic testing access in their clinical practice. The main reasons for not providing or limited access to genetic testing were no availability of dedicated unit or genetic laboratory (35%) or reimbursement issues (25%). The most frequently reported indication for genetic testing was diagnostic purpose (55%). Most respondents (92%) declared offering genetic testing preceded by genetic counselling and 42% regular multidisciplinary evaluations for patients with cardiac genetic diseases. The perceived value of genetic testing in the diagnostic, prognostic, and therapeutic assessment was variable (67, 39, and 29%, respectively) and primarily based on the specific inherited disease. The majority of respondents recommended cascade genetic testing for the first-degree family members in case of pathogenic/likely pathogenic variant in the proband. CONCLUSION: This survey highlights a significant heterogeneity of genetic testing access and provision and issues attributable to the availability of dedicated unit/genetic laboratory and reimbursement. However, adequate adherence to indications in the current recommendations for genetic testing in patients with cardiac diseases was observed.


Asunto(s)
Arritmias Cardíacas , Cardiomiopatías , Predisposición Genética a la Enfermedad , Pruebas Genéticas , Encuestas de Atención de la Salud , Humanos , Pruebas Genéticas/estadística & datos numéricos , Arritmias Cardíacas/genética , Arritmias Cardíacas/diagnóstico , Europa (Continente) , Cardiomiopatías/genética , Cardiomiopatías/diagnóstico , Pautas de la Práctica en Medicina/estadística & datos numéricos , Accesibilidad a los Servicios de Salud , Adhesión a Directriz/estadística & datos numéricos , Valor Predictivo de las Pruebas , Encuestas y Cuestionarios
2.
Europace ; 25(3): 896-904, 2023 03 30.
Artículo en Inglés | MEDLINE | ID: mdl-36738245

RESUMEN

AIMS: The multielectrode radiofrequency balloon catheter (RFB) has been developed to achieve safe and effective pulmonary vein isolation (PVI) for atrial fibrillation (AF) ablation. This single-centre study aimed to evaluate the midterm clinical outcome and predictors of single-shot PVI with the novel RFB. METHODS AND RESULTS: All consecutive patients with symptomatic paroxysmal or persistent AF undergoing first-time PVI with the RFB were prospectively included. Clinical and procedural parameters were systematically collected. The primary safety endpoint was defined as any major periprocedural complications. The primary efficacy endpoint consisted of freedom from any atrial tachyarrhythmias (ATas) lasting >30 s during the follow-up after a 3-month blanking period. Persistent single-shot PVI was defined as PVI achieved with a single RFB application without acute reconnection. A total of 104 consecutive patients (mean age 64.3 ± 11.4 years, 56.7% males) were included. 15 patients (14.4%) presented with persistent AF. The procedure time was 59.0 min with a dwell time of 20.0 min. One major complication occurred in one patient. At a mean follow-up of 10.1 ± 5.3 months, freedom from ATas was 82.9%. ATas occurred in 14 patients, 11/69 patients (15.9%) with paroxysmal AF and 3/13 (23.1%) with persistent AF. The best cut-offs to predict persistent single-shot PVI were impedance drop >19.2 Ω [area under the receiver operator characteristic curve (AUC) 0.74] and temperature rise >11.1° C (AUC 0.77). CONCLUSION: In a large cohort of patients undergoing PVI with the RFB, the complication rate was 1%. At a mid-term follow-up of 10.1 ± 5.3 months, freedom from ATas was 82.9%. Specific cut-offs of impedance drop and temperature rise may be useful to predict persistent single-shot isolation.


Asunto(s)
Fibrilación Atrial , Ablación por Catéter , Venas Pulmonares , Masculino , Humanos , Persona de Mediana Edad , Anciano , Femenino , Resultado del Tratamiento , Estudios de Seguimiento , Estudios Prospectivos , Venas Pulmonares/cirugía , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/cirugía , Taquicardia/cirugía , Catéteres , Ablación por Catéter/efectos adversos , Ablación por Catéter/métodos , Recurrencia
3.
Europace ; 25(5)2023 05 19.
Artículo en Inglés | MEDLINE | ID: mdl-37246904

RESUMEN

AIMS: Hybrid atrial fibrillation (AF) ablation is a promising approach in non-paroxysmal AF. The aim of this study is to assess the long-term outcomes of hybrid ablation in a large cohort of patients after both an initial and as a redo procedure. METHODS AND RESULTS: All consecutive patients undergoing hybrid AF ablation at UZ Brussel from 2010 to 2020 were retrospectively evaluated. Hybrid AF ablation was performed in a one-step procedure: (i) thoracoscopic ablation followed by (ii) endocardial mapping and eventual ablation. All patients received PVI and posterior wall isolation. Additional lesions were performed based on clinical indication and physician judgement. Primary endpoint was freedom from atrial tachyarrhythmias (ATas). A total of 120 consecutive patients were included, 85 patients (70.8%) underwent hybrid AF ablation as first procedure (non-paroxysmal AF 100%), 20 patients (16.7%) as second procedure (non-paroxysmal AF 30%), and 15 patients (12.5%) as third procedure (non-paroxysmal AF 33.3%). After a mean follow-up of 62.3 months ± 20.3, a total of 63 patients (52.5%) experienced ATas recurrence. Complications occurred in 12.5% of patients. There was no difference in ATas between patients undergoing hybrid as first vs. redo procedure (P = 0.53). Left atrial volume index and recurrence during blanking period were independent predictors of ATas recurrence. CONCLUSION: In a large cohort of patients undergoing hybrid AF ablation, the survival from ATas recurrence was 47.5% at ≈5 years follow-up. There was no difference in clinical outcomes between patients undergoing hybrid AF ablation as first procedure or as a redo.


Asunto(s)
Técnicas de Ablación , Fibrilación Atrial , Humanos , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/cirugía , Estudios de Seguimiento , Atrios Cardíacos , Estudios Retrospectivos , Taquicardia , Resultado del Tratamiento
4.
Europace ; 25(5)2023 05 19.
Artículo en Inglés | MEDLINE | ID: mdl-37061847

RESUMEN

AIMS: A pathogenic/likely pathogenic (P/LP) variant in SCN5A is found in 20-25% of patients with Brugada syndrome (BrS). However, the diagnostic yield and prognosis of gene panel testing in paediatric BrS is unclear. The aim of this study is to define the diagnostic yield and outcomes of SCN5A gene testing with ACMG variant classification in paediatric BrS patients compared with adults. METHODS AND RESULTS: All consecutive patients diagnosed with BrS, between 1992 and 2022, were prospectively enrolled in the UZ Brussel BrS registry. Inclusion criteria were: (i) BrS diagnosis; (ii) genetic analysis performed with a large gene panel; and (iii) classification of gene variants following ACMG guidelines. Paediatric patients were defined as ≤16 years of age. The primary endpoint was ventricular arrhythmias (VAs). A total of 500 BrS patients were included, with 63 paediatric patients and 437 adult patients. Among children with BrS, 29 patients (46%) had a P/LP variant (P+) in SCN5A and no variants were found in 34 (54%) patients (P-). After a mean follow-up of 125.9 months, 8 children (12.7%) experienced a VA, treated with implanted cardioverter defibrillator shock. At survival analysis, P- paediatric patients had higher VA-free survival during the follow-up, compared with P+ paediatric patients. P+ status was an independent predictor of VA. There was no difference in VA-free survival between paediatric and adult BrS patients for both P- and P+. CONCLUSION: In a large BrS cohort, the diagnostic yield for P/LP variants in the paediatric population is 46%. P+ children with BrS have a worse arrhythmic prognosis.


Asunto(s)
Síndrome de Brugada , Adulto , Humanos , Niño , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/genética , Síndrome de Brugada/terapia , Pruebas Genéticas , Arritmias Cardíacas/genética , Cardioversión Eléctrica , Pronóstico , Electrocardiografía/métodos , Canal de Sodio Activado por Voltaje NAV1.5/genética
5.
Clin Auton Res ; 33(1): 51-62, 2023 02.
Artículo en Inglés | MEDLINE | ID: mdl-36645559

RESUMEN

PURPOSE: Drug-induced type I Brugada syndrome (BrS) is associated with a ventricular arrhythmia (VA) rate of 1 case per 100 person-years. This study aims to evaluate changes in electrocardiographic (ECG) parameters such as microvolt T wave alternans (mTWA) and heart rate variability (HRV) at baseline and during ajmaline testing for BrS diagnosis. METHODS: Consecutive patients diagnosed with BrS during ajmaline testing with 5-year follow-up were included in this study. For comparison, a negative ajmaline control group and an isoproterenol control group were also included. ECG recordings during ajmaline or isoproterenol test were divided in two timeframes from which ECG parameters were calculated: a 5-min baseline timeframe and a 5-min drug timeframe. RESULTS: A total of 308 patients with BrS were included, 22 (0.7%) of which suffered VAs during follow-up. One hundred patients were included in both isoproterenol and negative ajmaline control groups. At baseline, there was no difference in ECG parameters between control groups and patients with BrS, nor between BrS with and without VAs. During ajmaline testing, BrS with VAs presented longer QRS duration [159 ± 34 ms versus 138 (122-155) ms, p = 0.006], higher maximum mTWA [33.8 (14.0-114) µV versus 8.00 (3.67-28.2) µV, p = 0.001], and lower power in low frequency band [25.6 (5.8-53.8) ms2 versus 129.5 (52.7-286) ms2, p < 0.0001] when compared to BrS without VAs. CONCLUSIONS: Ajmaline induced important HRV changes similar to those observed during isoproterenol. Increased mTWA was observed only in patients with BrS. BrS with VAs during follow-up presented worse changes during ajmaline test, including lower LF power and higher maximum mTWA which were independent predictors of events.


Asunto(s)
Ajmalina , Síndrome de Brugada , Humanos , Ajmalina/farmacología , Síndrome de Brugada/diagnóstico , Frecuencia Cardíaca , Isoproterenol , Arritmias Cardíacas , Electrocardiografía , Pronóstico
6.
Pacing Clin Electrophysiol ; 45(5): 605-611, 2022 05.
Artículo en Inglés | MEDLINE | ID: mdl-35352363

RESUMEN

BACKGROUND: Left atrial posterior wall isolation (LAPWI) is often performed in addition to pulmonary vein isolation (PVI) in the setting of persistent atrial fibrillation (AF) ablation. The aim of this study was to evaluate the feasibility and safety of a new cryoballoon ablation system in achieving PVI + LAPWI isolation. METHODS: The study was a prospective, non-randomized, single center study. Forty consecutive patients, undergoing PVI + LAPWI with the novel POLARx™, were compared to 40 consecutive patients who underwent the same procedure with the established Arctic Front Advance PRO™. RESULTS: Acute isolation was achieved in all PVs in both groups and left posterior wall isolation (LAPWI) was achieved in 38 patients (95%) in the POLARx group and in 36 patients (90%) in Arctic Front group. Procedural outcomes were similar between both groups, except for lower temperatures during cryoenergy in the POLARx group, for both pulmonary vein isolation (PVI) and LAPWI. CONCLUSION: LAPWI + PVI with the novel POLARx™ Cryoballoon is feasible and safe; the results are comparable with the Arctic Front Advance PRO™ system.


Asunto(s)
Fibrilación Atrial , Ablación por Catéter , Criocirugía , Venas Pulmonares , Fibrilación Atrial/cirugía , Criocirugía/métodos , Estudios de Factibilidad , Humanos , Estudios Prospectivos , Venas Pulmonares/cirugía , Recurrencia , Tecnología , Resultado del Tratamiento
7.
Am J Physiol Regul Integr Comp Physiol ; 319(4): R497-R506, 2020 10 01.
Artículo en Inglés | MEDLINE | ID: mdl-32877240

RESUMEN

Ballistocardiography (BCG) and seismocardiography (SCG) assess vibrations produced by cardiac contraction and blood flow, respectively, through micro-accelerometers and micro-gyroscopes. BCG and SCG kinetic energies (KE) and their temporal integrals (iK) during a single heartbeat are computed in linear and rotational dimensions. Our aim was to test the hypothesis that iK from BCG and SCG are related to sympathetic activation during maximal voluntary end-expiratory apnea. Multiunit muscle sympathetic nerve traffic [burst frequency (BF), total muscular sympathetic nerve activity (tMSNA)] was measured by microneurography during normal breathing and apnea (n = 28, healthy men). iK of BCG and SCG were simultaneously recorded in the linear and rotational dimension, along with oxygen saturation ([Formula: see text]) and systolic blood pressure (SBP). The mean duration of apneas was 25.4 ± 9.4 s. SBP, BF, and tMSNA increased during the apnea compared with baseline (P = 0.01, P = 0.002,and P = 0.001, respectively), whereas [Formula: see text] decreased (P = 0.02). At the end of the apnea compared with normal breathing, changes in iK computed from BCG were related to changes of tMSNA and BF only in the linear dimension (r = 0.85, P < 0.0001; and r = 0.72, P = 0.002, respectively), whereas changes in linear iK of SCG were related only to changes of tMSNA (r = 0.62, P = 0.01). We conclude that maximal end expiratory apnea increases cardiac kinetic energy computed from BCG and SCG, along with sympathetic activity. The novelty of the present investigation is that linear iK of BCG is directly and more strongly related to the rise in sympathetic activity than the SCG, mainly at the end of a sustained apnea, likely because the BCG is more affected by the sympathetic and hemodynamic effects of breathing cessation. BCG and SCG may prove useful to assess sympathetic nerve changes in patients with sleep disturbances.NEW & NOTEWORTHY Ballistocardiography (BCG) and seismocardiography (SCG) assess vibrations produced by cardiac contraction and blood flow, respectively, through micro-accelerometers and micro-gyroscopes. Kinetic energies (KE) and their temporal integrals (iK) during a single heartbeat are computed from the BCG and SCG waveforms in a linear and a rotational dimension. When compared with normal breathing, during an end-expiratory voluntary apnea, iK increased and was positively related to sympathetic nerve traffic rise assessed by microneurography. Further studies are needed to determine whether BCG and SCG can probe sympathetic nerve changes in patients with sleep disturbances.


Asunto(s)
Apnea/fisiopatología , Contracción Miocárdica/fisiología , Sistema Nervioso Simpático/fisiología , Adulto , Balistocardiografía , Presión Sanguínea/fisiología , Frecuencia Cardíaca/fisiología , Humanos , Masculino
8.
J Cardiovasc Electrophysiol ; 31(7): 1793-1800, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32412155

RESUMEN

INTRODUCTION: Management of subcutaneous implantable cardioverter defibrillator (S-ICD) patients with newly acquired pacing needs remains problematic. His bundle pacing (HBP) allows for cardiac pacing without significant changes in the QRS morphology. We hypothesized that HBP does not alter S-ICD sensing and functions. METHODS: Twenty consecutive patients were implanted with a HB pacemaker. Among them, 17 demonstrated successful His recruitment and were prospectively screened with the automated screening tool (AST). Results of screenings performed immediately after implant and during follow-up, during intrinsic rhythm and while pacing from all available pacing configurations, were compared using the AST score. Positive-screening tests were defined by greater than or equal to 1 positive vector. RESULTS: Among the 17 patients successfully implanted (male: 41%; mean age: 73), 13 presented an indication of ventricular pacing and four of cardiac resynchronization. Absolute AST scores during both HBP (all configurations) and intrinsic rhythm were similar (p: NS). Due to left bundle branch block correction, HBP resulted in higher number of positive vectors (AST ≥ 100). AST scores were higher during HBP when compared with right ventricular pacing (RVP) (primary vector: 272 [16; 648] vs 4.6 [0.8; 16.2]; P = .003; secondary vector: 569 [183; 1186] vs 1.5 [0.7; 8.3]; P < .0001; alternate vector: 44 [2;125] vs 4.8 [0.9; 9.3]; P = .02) and resulted in a much higher number of positive vectors. Up to 90% of the patients had a positive-screening test during HBP. This passing rate was higher when compared RVP (17%; P < .0001). CONCLUSION: HBP restores normal intrinsic conduction and minimally modifies the surface electrocardiograph and subcutaneous electrograms. When ventricular pacing is needed, HBP might represent an ideal pacing option for patients implanted with a S-ICD.


Asunto(s)
Terapia de Resincronización Cardíaca , Desfibriladores Implantables , Anciano , Fascículo Atrioventricular , Estimulación Cardíaca Artificial/efectos adversos , Electrocardiografía , Estudios de Factibilidad , Humanos , Masculino , Resultado del Tratamiento
9.
J Cardiovasc Electrophysiol ; 31(2): 494-502, 2020 02.
Artículo en Inglés | MEDLINE | ID: mdl-31908084

RESUMEN

INTRODUCTION: We investigated whether pacing-induced electrical dyssynchrony at the time of cardiac resynchronization therapy (CRT) device implantation was associated with chronic CRT response. METHODS AND RESULTS: We included a total of 69 consecutive heart failure patients who received a CRT device. Left (LVp-RVs) and right (RVp-LVs) pacing-induced interlead delays were measured intraoperatively and used to determine if there was paced left ventricular (LV) dyssynchrony, defined as present when LVp-RVs is larger than RVp-LVs. CRT response was defined as a reduction in LV end-systolic volume ≥15%, 6 months after implantation. Paced left ventricular dyssynchrony (PLVD) was associated with ischemic cardiomyopathy (ICM) (χ2 : 8; P = .005) but not with QRS morphology nor with pacing lead positions. In a univariate analysis, PLVD (odds ratio [OR], 6.53; 95% confidence interval [CI], 2.2-18.9; P = .001), atypical left bundle branch block (LBBB) (OR, 3.3; 95% CI, 1.2-9.4; P = .022), and ICM (OR, 5.2; 95% CI, 1.6-17; P = .006) were associated with nonresponse. In a multivariate analysis, both PLVD (OR, 9.74; 95% CI, 2.8-33.9; P < .0001) and atypical LBBB (OR, 5.6; 95% CI, 1.5-20.3; P = .009) were independently associated with nonresponse. Adding PLVD to a model based on QRS morphology provided a significant and meaningful incremental value to predict LV reverse remodeling after CRT (χ2 to enter: 8; P < .005). Computer simulations corroborate these findings by showing that, while intrinsic electrical dyssynchrony is a prerequisite, the level of pacing-induced dyssynchrony modulates acute CRT response. CONCLUSION: In addition to the intrinsic electrical substrate, PLVD is strongly associated with less LV reverse remodeling, demonstrating that measuring the electrical substrate during pacing has additional value for prediction of CRT response in an already well-selected patient population.


Asunto(s)
Terapia de Resincronización Cardíaca , Insuficiencia Cardíaca/terapia , Disfunción Ventricular Izquierda/terapia , Función Ventricular Izquierda , Remodelación Ventricular , Adulto , Anciano , Anciano de 80 o más Años , Simulación por Computador , Femenino , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/fisiopatología , Hemodinámica , Humanos , Masculino , Persona de Mediana Edad , Modelos Cardiovasculares , Estudios Prospectivos , Recuperación de la Función , Factores de Tiempo , Resultado del Tratamiento , Disfunción Ventricular Izquierda/diagnóstico , Disfunción Ventricular Izquierda/fisiopatología
10.
J Cardiovasc Electrophysiol ; 31(4): 813-821, 2020 04.
Artículo en Inglés | MEDLINE | ID: mdl-31990128

RESUMEN

BACKGROUND: Conduction disorders requiring permanent pacemaker implantation occur frequently after transcatheter aortic valve replacement (TAVR). This multicenter study explored the feasibility and safety of His bundle pacing (HBP) in TAVR patients with a pacemaker indication to correct a TAVR-induced left bundle branch block (LBBB). METHODS: Patients qualifying for a permanent pacemaker implant after TAVR were planned for HBP implant. HBP was performed using the Select Secure (3830; Medtronic) pacing lead, delivered through a fixed curve or deflectable sheath (C315HIS or C304; Medtronic). Successful HBP was defined as selective or nonselective HBP, irrespective of LBB recruitment. Successful LBBB correction was defined as selective or nonselective HBP resulting in paced QRS morphology similar to pre-TAVR QRS and paced QRS duration (QRSd) less than 120 milliseconds with thresholds less than 3.0 V at 1.0-millisecond pulse width. RESULTS: The study enrolled 16 patients requiring a permanent pacemaker after TAVR (age 85 ± 4 years, 31% female, all LBBB; QRSd: 161 ± 14 milliseconds). Capture of the His bundle was achieved in 13 of 16 (81%) patients. HBP with LBBB correction was achieved in 11 of 16 (69%) and QRSd narrowed from 162 ± 14 to 99 ± 13 milliseconds and 134 ± 7 milliseconds during S-HBP and NS-HBP, respectively (P = .005). At implantation, mean threshold for LBBB correction was 1.9 ± 1.1 V at 1.0 millisecond. Thresholds remained stable at 11 ± 4 months follow-up (1.8 ± 0.9 V at 1.0 millisecond, P = .231 for comparison with implant thresholds). During HBP implant, one temporary complete atrioventricular block occurred. CONCLUSION: Permanent HBP is feasible in the majority of patients with TAVR requiring a permanent pacemaker with the potential to correct a TAVR-induced LBBB with acceptable pacing thresholds.


Asunto(s)
Potenciales de Acción , Fascículo Atrioventricular/fisiopatología , Bloqueo de Rama/terapia , Estimulación Cardíaca Artificial , Frecuencia Cardíaca , Marcapaso Artificial , Reemplazo de la Válvula Aórtica Transcatéter/efectos adversos , Anciano de 80 o más Años , Bélgica , Bloqueo de Rama/diagnóstico , Bloqueo de Rama/etiología , Bloqueo de Rama/fisiopatología , Estimulación Cardíaca Artificial/efectos adversos , Estudios de Factibilidad , Femenino , Humanos , Masculino , Factores de Tiempo , Resultado del Tratamiento
11.
Circ Arrhythm Electrophysiol ; 17(4): e012374, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38426305

RESUMEN

BACKGROUND: A pathogenic/likely pathogenic variant can be found in 20% to 25% of patients with Brugada syndrome (BrS) and a pathogenic/likely pathogenic variant in SCN5A is associated with a worse prognosis. The aim of this study is to define the diagnostic yield of a large gene panel with American College of Medical Genetics and Genomics variant classification and to assess prognosis of SCN5A and non-SCN5A variants. METHODS: All patients with BrS, were prospectively enrolled in the Universitair Ziekenhuis Brussel registry between 1992 and 2022. Inclusion criteria for the study were (1) BrS diagnosis; (2) genetic analysis performed with a large gene panel; (3) classification of variants following American College of Medical Genetics and Genomics guidelines. Patients with a pathogenic/likely pathogenic variant in SCN5A were defined as SCN5A+. Patients with a reported variant in a non-SCN5A gene or with no reported variants were defined as patients with SCN5A-. All variants were classified as missense or predicted loss of function. RESULTS: A total of 500 BrS patients were analyzed. A total of 104 patients (20.8%) were SCN5A+ and 396 patients (79.2%) were SCN5A-. A non-SCN5A gene variant was found in 75 patients (15.0%), of whom, 58 patients (77.3%) had a missense variant and 17 patients (22.7%) had a predicted loss of function variant. At a follow-up of 84.0 months, 48 patients (9.6%) experienced a ventricular arrhythmia (VA). Patients without any variant had higher VA-free survival, compared with carriers of a predicted loss of function variant in SCN5A+ or non-SCN5A genes. There was no difference in VA-free survival between patients without any variant and missense variant carriers in SCN5A+ or non-SCN5A genes. At Cox analysis, SCN5A+ or non-SCN5A predicted loss of function variant was an independent predictor of VA. CONCLUSIONS: In a large BrS cohort, the yield for SCN5A+ is 20.8%. A predicted loss of function variant carrier is an independent predictor of VA.


Asunto(s)
Síndrome de Brugada , Humanos , Síndrome de Brugada/diagnóstico , Síndrome de Brugada/genética , Pruebas Genéticas , Arritmias Cardíacas/genética , Mutación Missense , Canal de Sodio Activado por Voltaje NAV1.5/genética , Mutación
12.
JACC Clin Electrophysiol ; 9(10): 2096-2105, 2023 10.
Artículo en Inglés | MEDLINE | ID: mdl-37565952

RESUMEN

BACKGROUND: Patients with Brugada syndrome (BrS) have an increased risk of arrhythmias, including atrial tachyarrhythmias (ATas). OBJECTIVES: The purpose of this study was to assess underlying atrial cardiomyopathy in BrS and the effect of ajmaline (AJM) test on the atrium of BrS patients using electrocardiogram imaging (ECGI). METHODS: All consecutive patients diagnosed with BrS in a monocentric registry were screened and included if they met the following criteria: 1) BrS diagnosed following current recommendations; and 2) ECGI map performed before and after AJM with a standard protocol. Consecutive patients with no structural heart disease or BrS who had undergone ECGI were included as a control group. Genetic analysis for SCN5A was performed in all BrS patients. Total atrial conduction time (TACT) and local atrial conduction time (LACT) were calculated from atrial ECGI. The primary endpoint was ATas during follow-up. RESULTS: Forty-three consecutive BrS patients and 40 control patients were included. Both TACT and LACT were significantly prolonged in BrS patients compared with control patients. Furthermore, TACT and LACT were significantly higher after AJM administration and in BrS patients who were carriers of a pathogenic/likely pathogenic SCN5A variant. After a mean follow-up of 40.9 months, 6 patients experienced a first ATa occurrence (all in the BrS group, 13.9%). TACT was the only independent predictor of ATas with a cutoff of >138.5 ms (sensitivity 0.92 [95% CI: 0.83-0.98], specificity 0.70 [95% CI: 0.59-0.81]). CONCLUSIONS: ECGI-calculated TACT and LACT are significantly prolonged in BrS patients compared with control patients, and in BrS patients after AJM. This may be consistent with a concealed atrial cardiomyopathy in BrS.


Asunto(s)
Fibrilación Atrial , Síndrome de Brugada , Cardiomiopatías , Humanos , Síndrome de Brugada/diagnóstico por imagen , Síndrome de Brugada/genética , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico por imagen , Electrocardiografía/métodos , Ajmalina
13.
JACC Clin Electrophysiol ; 9(8 Pt 1): 1296-1306, 2023 08.
Artículo en Inglés | MEDLINE | ID: mdl-37227348

RESUMEN

BACKGROUND: Different genes have been associated with idiopathic ventricular fibrillation (IVF); however, there are no studies correlating genotype with phenotype. OBJECTIVES: The aim of this study was to define the genetic background of probands with IVF using large gene panel analysis and to correlate genetics with long-term clinical outcomes. METHODS: All consecutive probands with a diagnosis of IVF were included in a multicenter retrospective study. All patients had: 1) IVF diagnosis throughout the follow-up; and 2) genetic analysis with a broad gene panel. All genetic variants were classified as pathogenic/likely pathogenic (P+), variants of unknown significance (VUS) or no variants (NO-V), following current guidelines of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology. The primary endpoint was occurrence of ventricular arrhythmias (VA). RESULTS: Forty-five consecutive patients were included. A variant was found in 12 patients, 3 P+ and 9 VUS carriers. After a mean follow-up time of 105.0 months, there were no deaths and 16 patients (35.6%) experienced a VA. NO-V patients had higher VA free survival during the follow-up, compared with both VUS (72.7% vs 55.6%, log-rank P < 0.001) and P+ (72.7% vs 0%, log-rank P = 0.013). At Cox analysis, P+ or VUS carrier status was a predictor of VA occurrence. CONCLUSIONS: In probands with IVF, undergoing genetic analysis with a broad panel, the diagnostic yield for P+ is 6.7%. P+ or VUS carrier status is a predictor of VA occurrence.


Asunto(s)
Pruebas Genéticas , Fibrilación Ventricular , Humanos , Estudios Retrospectivos , Arritmias Cardíacas/complicaciones
14.
Int J Cardiol Heart Vasc ; 40: 101040, 2022 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-35514875

RESUMEN

Introduction: The second-generation cryoballoon (CB) has emerged in the last decade as an effective treatment for atrial fibrillation (AF). This study sought to analyze the rate of PV reconnection following CB ablation, evaluate the most frequent PV sites of conduction recovery and finally to assess procedural and biophysical indicators of reconnection in a large cohort of patients undergoing repeat ablation for recurrence of atrial arrhythmias. Methods and Results: A total of 300 consecutive patients (189 males, 63%; mean age 63.0 ± 11.1 years) underwent a repeat ablation after 18.2 ± 10.8 months from the index CB ablation. All repeat ablations were performed using a 3-dimensional electro-anatomical mapping system. Among all 1178 PVs, 209 (17.7%) showed a late PV reconnection in 177 patients (1.18 per patient), at the time of repeat ablation procedure. Overall, persistent PV isolation could be documented in 969 of 1178 PVs (82.3%). In 123 of 300 patients (41%), persistent isolation could be demonstrated in all PVs, whereas PV reconnection could be documented in 177 patients (59%). In the multivariable analysis, nadir temperature (p = 0.03), time to PV isolation (p = 0.01) and failure to achieve - 40 °C within 60 s (p = 0.05) were independently associated with late PV reconnection. Conclusions: The rate of late PV reconnection after CB ablation was low (1.18 PVs/patient). The most frequent sites of reconnections were the superior-anterior portions for the upper PVs and the inferior-posterior portions for the lower PVs. Faster time to isolation, colder nadir temperatures and achievement of - 40 °C within 60 s were associated with durable PV isolation.

15.
J Clin Med ; 11(10)2022 May 23.
Artículo en Inglés | MEDLINE | ID: mdl-35629074

RESUMEN

BACKGROUND: Non-contact charge density (CD) mapping allows a global visualization of left atrium (LA) activation and of activation patterns during atrial fibrillation (AF). The aim of this study was to analyze, with CD mapping, the changes in persistent AF induced by pulmonary vein isolation (PVI) and LA posterior wall isolation (LAPWI). METHODS: Patients undergoing PVI + LAPWI using the Arctic Front Advance PROTM cryoballoon system were included in the study. CD maps were created during AF at baseline, after PVI and after LAPWI. Three distinct activation patterns were identified in the CD maps: localized irregular activation (LIA), localized rotational activation (LRA) and focal centrifugal activation (FCA). LA maps were divided into the following regions: anterior, septal, lateral, roof, posterior, inferior. RESULTS: Eleven patients were included, with a total of 33 maps and 198 AF regions analyzed. Global and regional AF cycle lengths significantly increased after PVI and LAPWI. Baseline analysis demonstrated higher LIA, LRA and FCA numbers in the posterior and anterior regions. After PVI, there was no change in LIA, LRA and FCA occurrence. After PVI + LAPWI, a significant decrease in LRA was observed with no difference in LIA and FCA occurrence. In the regional analysis, there was a significant reduction in the LIA number in the inferior region, in the LRA number in the roof and posterior regions and in the FCA number in the lateral region. CONCLUSIONS: A global reduction in the LRA number was observed only after PVI + LAPWI; it was driven by a reduction in rotational activity in the roof and posterior regions.

16.
Front Cardiovasc Med ; 9: 917218, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35837608

RESUMEN

Background: New technologies for ablation procedures are often produced by different companies with no cross-compatibility out of the box. This is not a negligible clinical problem since those separately developed devices are often used together. The aim of this study was to develop a bench-testing method to assess compatibility between the DiamondTemp ablation system (DTA) and the Rhythmia electroanatomic mapping system (EAM). Methods: Different setups were tested. DTA was connected to the Rhythmia EAM using the following configurations: 3.1. An Ensite EPT GenConnect box (GCB) and Rhythmia Maestro GCB (Maestro GCB, native Rhythmia setup); 3.2. The Medtronic GCB-E and Maestro GCB; 3.3. The Medtronic GCB-E out via the Medtronic GCB-E directly to the Rhythmia at box 1 (pin A61 to A64). Results: The DTA location was represented in real-time on the Rhythmia EAM. A proper tracking of the DTA was observed in all setups tested by visual comparison of physical catheter movements and its representation on EAM. In configuration 3.1, a significant shift was observed after the first radio frequency (RF) application; however, further applications caused no further shift. In setup 3.2, no significant shift was observed. The setup 3.3 showed a massive shift in the catheter position before ablation compared to baseline points acquired using the Orion catheter as a reference. Conclusions: A universal and reproducible solution for compatibility testing between the various mapping systems and the ablation catheters has been described. DTA has been demonstrated as compatible with Rhythmia EAM with satisfactory results if a specific setup is used.

17.
J Interv Card Electrophysiol ; 65(3): 717-724, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35930128

RESUMEN

BACKGROUND: The second-generation cryoballoon (CB) has proven to be a highly effective ablative strategy in patients with symptomatic atrial fibrillation (AF). This study sought to investigate the anatomical characteristics of pulmonary veins (PVs) and the relationship between their size, ovality, and late reconnections in a large cohort of patients undergoing repeat ablation for recurrence of atrial arrhythmias. METHODS AND RESULTS: A total of 152 consecutive patients (98 males, 64.5%; mean age 64.9 ± 9.6 years) underwent a repeat ablation for recurrent atrial tachyarrhythmias after a median time of 6.5 months [IQR 11] from the index CB ablation. All repeat ablations were performed using a 3-dimensional electro-anatomical mapping system. Among all 593 PVs, 134 (22.6%) showed a late PV reconnection in 95 patients (0.88 per patient), at the time of repeat ablation procedure. There was a significant difference in ovality between left- and right-sided PVs (p < 0.001). Greater diameters of left superior PV, left inferior PV, and right inferior PV ostia (both maximum and minimum) and higher index ovality were significantly associated with late PV reconnection. CONCLUSIONS: The rate of late PV reconnection after CB ablation was low (0.88 PVs/patient). Left-sided PVs were more oval than septal PVs. Larger PV dimensions and higher ovality index were significantly associated with reconnections in all PVs except for RSPV.


Asunto(s)
Fibrilación Atrial , Venas Pulmonares , Humanos , Persona de Mediana Edad , Anciano , Venas Pulmonares/cirugía , Fibrilación Atrial/diagnóstico por imagen , Fibrilación Atrial/cirugía
18.
J Interv Card Electrophysiol ; 65(3): 731-737, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35945310

RESUMEN

BACKGROUND: This study aimed to evaluate the feasibility and safety of an innovative "all in one" integrated transseptal crossing device to achieve transseptal puncture (TSP). METHODS: Twenty patients (10 males, mean age 65.65 ± 9.25 years), indicated to supraventricular left side tachyarrhythmia ablation, underwent TSP using a new-generation integrated crossing device, and a control cohort of twenty patients (10 males, mean age 65.5 ± 10.12 years) underwent TSP using the traditional TSP system. RESULTS: In all the study patients, the novel TSP device led to a successful and safe access to the left atrium (LA). The mean transseptal time, defined as the time occurring between the groin puncture and the advancing of the guidewire into the left superior pulmonary vein (PV), was 3 min 33 s ± 44 s, 7 min 5 s ± 36 s in the control cohort. Additionally, we compared the cost of the two systems. No acute complications related to the TSP were noted in both cohorts. CONCLUSIONS: TSP performed with the new integrated transseptal system is feasible and safe.


Asunto(s)
Humanos , Persona de Mediana Edad , Anciano
19.
J Am Heart Assoc ; 11(15): e026290, 2022 08 02.
Artículo en Inglés | MEDLINE | ID: mdl-35862178

RESUMEN

Background Pharmacological treatment of atrial fibrillation (AF) in the setting of Brugada syndrome (BrS) is challenging. In addition, patients with BrS with an implantable cardioverter-defibrillator (ICD) might experience inappropriate shocks for fast AF. Long-term outcome of pulmonary vein isolation in BrS has not been well established yet, and it is still unclear whether pulmonary vein triggers are the only pathophysiological mechanism of AF in BrS. The aim of the study is to assess the long-term outcomes in patients with BrS undergoing pulmonary vein isolation for paroxysmal AF compared with a matched cohort of patients without BrS. Methods and Results Sixty patients with BrS undergoing pulmonary vein isolation with cryoballoon catheter ablation for paroxysmal AF were matched with 60 patients without BrS, who underwent the same procedure. After a mean follow-up of 58.2±31.7 months, freedom from atrial tachyarrhythmias was achieved in 61.7% in the BrS group and in 78.3% in the non-BrS group (log-rank P=0.047). In particular, freedom from AF was 76.7% in the first group and in 83.3% in the second (P=0.27), while freedom from atrial tachycardia/atrial flutter was 85% and 95% (P=0.057). In the BrS group, 29 patients (48.3%) had an ICD and 8 (27.6%) had a previous ICD-inappropriate shock for fast AF. In the BrS cohort, ICD-inappropriate interventions for AF were significantly reduced after ablation (3.4% versus 27.6%; P=0.01). Conclusions Pulmonary vein isolation in patients with BrS was associated with higher rate of arrhythmic recurrence. Despite this, catheter ablation significantly reduced inappropriate ICD interventions in BrS patients and can be considered a therapeutic strategy to prevent inappropriate device therapies.


Asunto(s)
Fibrilación Atrial , Aleteo Atrial , Síndrome de Brugada , Ablación por Catéter , Criocirugía , Venas Pulmonares , Fibrilación Atrial/complicaciones , Fibrilación Atrial/diagnóstico , Fibrilación Atrial/cirugía , Síndrome de Brugada/complicaciones , Síndrome de Brugada/cirugía , Ablación por Catéter/efectos adversos , Ablación por Catéter/métodos , Criocirugía/efectos adversos , Humanos , Venas Pulmonares/cirugía , Recurrencia , Resultado del Tratamiento
20.
J Cardiovasc Dev Dis ; 9(9)2022 Sep 08.
Artículo en Inglés | MEDLINE | ID: mdl-36135447

RESUMEN

Electrical isolation of pulmonary veins (PVI) is the cornerstone of invasive treatment of atrial fibrillation (AF). However, arrhythmia-free survival of a PVI only approach is suboptimal in patients with persistent and long-term persistent AF. Hybrid AF ablation has been developed with the aim of combining the advantages of a thoracoscopic surgical ablation (direct visualization of anatomical structures to be spared and the possibility to perform epicardial lesions) and endocardial ablation (possibility to check line block, confirm PVI, and possibility to perform cavotricuspid isthmus ablation). Patient selection is of utmost importance. In persistent and long-term persistent AF, hybrid AF ablation demonstrated promising results in terms of AF free survival. It has been associated with a relatively low complication rate if performed in centers with expertise in hybrid procedures and experience with both surgical and endocardial ablation. Different techniques have been described, with different approaches and lesion sets. The aim of this review is to provide a state-of-the-art overview of hybrid AF ablation.

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