Assessing cardiac resynchronization therapy response in heart failure patients: a comparative analysis of efficacy and outcomes between transvenous and epicardial leads.

Cardiac resynchronization therapy (CRT) is an effective treatment for selected heart failure (HF) patients. Although transvenous implantation is the standard method, it is not feasible in some patients, so the epicardial lead emerges as an alternative. We aim to compare CRT response, procedure-related complications, and the occurrence of clinical outcomes between patients with transvenous and epicardial leads. In a single-center retrospective study, we enrolled consecutive HF patients submitted to CRT implantation with a defibrillator between 2013 and 2022. Clinical response was defined as an improvement of at least one of the New York Heart Association classes with no occurrence of cardiovascular death or HF hospitalization in the first year of follow-up. Echocardiographic response was attained with an increase in left ventricular ejection fraction 10% or a reduction of left ventricular end-diastolic volume >15% at 6-12 months after CRT implantation. Major adverse cardiovascular events (MACE) (cardiovascular mortality and HF hospitalization) and all-cause mortality were evaluated. From a total of 149 patients, 38% (n=57) received an epicardial lead. Clinical (63% versus 60%, p=0.679) and echocardiographic (63% versus 60%, p=0.679) responses were similar between the transvenous and epicardial groups. Patients in the transvenous group had a shorter hospital stay (2 versus 7 days, p<0.001). Procedure-related complications were comparable between groups (24% versus 28%, p=0.572), but left ventricular lead-related complications were more frequent in the transvenous group (14% versus 2%). During a median follow-up of 4.7 years, the rate of MACE was 30% (n=44), with no differences in both groups (p=0.591), neither regarding HF hospitalization (p=0.917) nor cardiovascular mortality (p=0.060). Nevertheless, the epicardial group had a higher rate of all-cause mortality (35% versus 20%, p=0.005), the majority occurring during long-term follow-up (>12 months), with no deaths in the postoperative period. Considering the comparable rates of CRT response, procedure-related complications, and MACE between groups, we conclude that epicardial lead is a feasible alternative for CRT when transvenous lead implantation is not possible. The occurrence of a higher number of all-cause deaths in epicardial patients in the long-term follow-up was mainly due to infectious complications (unrelated to the lead) and the progression of oncological/chronic diseases.

Quality indicators in atrial fibrillation ablation (RIQAFA). A national registry from the Portuguese Association of Arrhythmology, Pacing and Electrophysiology (APAPE).

INTRODUCTION AND OBJECTIVES: Atrial fibrillation (AF) is the most common sustained arrhythmia, with significant burden for patients. Catheter ablation is safe and superior for symptom improvement. The purpose of this work was to assess how clinical practice compares with current scientific evidence and quality indicators for AF ablation. METHODS: The Portuguese Association of Arrhythmology, Pacing and Electrophysiology conducted a prospective registry among Portuguese centers to assess clinical practice regarding management of patients referred for ablation and the methodology used in the procedures and related outcomes. RESULTS: A total of 337 patients were referred for ablation, 102 (37.91%) female, age 65 (56-70.8) years. The median CHADS2-VaSC2 thromboembolic risk score was 2 (1-3), and 308 (92.49%) were on anticoagulants. AF was mainly paroxysmal (224, 66.97%) and symptomatic (mEHRA score 3; 2-3). Before ablation most patients (273, 81.49%) underwent cardiac computed tomography and only 24 (7.36%) procedures were performed with uninterrupted anticoagulation. For ablation, Carto® (194; 59.15%) and Ensite® (55; 16.77%) were mainly used, and the preferential strategy was pulmonary vein isolation (316; 94.61%). Acute complications occurred in five (1.49%) patients, while most had symptom improvement at one month (200; 86.21%), sustained at one year. There were 40 (12.6%) relapses within 30 days and 19 (26.39%) at one year. CONCLUSIONS: In a population of patients with AF referred for ablation in Portuguese centers, patient management is provided according to the best scientific evidence and there is a high standard of practice with respect to the quality of AF ablation practice.

Mitral annular disjunction: Beyond mitral valve prolapse.

Mitral annular disjunction (MAD) is an easily identifiable entity on transthoracic echocardiography, but is still poorly recognized or ignored. It is often associated with mitral valve prolapse and is itself a risk marker for ventricular arrhythmias and sudden cardiac death, but the management and risk stratification of these patients is not systematized. Two clinical cases of MAD associated with mitral valve prolapse and ventricular arrhythmias are presented. The first case is of a patient with a history of surgical intervention on the mitral valve due to Barlow's disease. He presented to the emergency department with sustained monomorphic ventricular tachycardia requiring emergent electrical cardioversion. MAD with transmural fibrosis at the level of the inferolateral wall was documented. The second report is of a young woman with palpitations and frequent premature ventricular contractions on Holter with documentation of valvular prolapse and MAD, and focuses on the risk stratification approach. The present article offers a review of the literature regarding the arrhythmic risk of MAD and mitral valve prolapse, as well as a review of risk stratification in these patients.

Cardioneuroablation for severe neurocardiogenic syncope.

INTRODUCTION AND OBJECTIVES: Cardioneuroablation (CNA), a technique based on radiofrequency ablation of cardiac vagal ganglia, was developed to treat recurrent vasovagal syncope (VVS) with a predominant cardioinhibitory component, as an alternative to pacemaker implantation. The aim of our study was to evaluate the safety and success rate of CNA guided by extracardiac vagal stimulation in patients with highly symptomatic cardioinhibitory VVS. METHODS: Prospective study of patients who underwent anatomically guided CNA at two cardiology centers. All patients had a history of recurrent syncope with a predominant cardioinhibitory component and refractory to conventional measures. Acute success was determined by the absence or significant reduction of cardiac parasympathetic response to extracardiac vagal stimulation. The primary endpoint was the recurrence of syncope during follow-up. RESULTS: In total, 19 patients (13 males; mean age 37.8±12.9 years) were included. Ablation was acutely successful in all patients. One patient had a convulsive episode after the procedure, which was deemed unrelated to the ablation, requiring admission to intensive care but without sequelae. No other complications occurred. At a mean follow-up of 21.0±13.2 months (range 3-42 months), 17 patients remained free of syncope. The remaining two patients had recurrence of syncope and, despite undergoing a new ablation procedure, required pacemaker implantation during follow-up. CONCLUSION: Cardioneuroablation, confirmed by extracardiac vagal stimulation, appears to be an effective and safe treatment option for highly symptomatic patients with refractory VVS with a predominant cardioinhibitory component, providing a new potential approach as an alternative to pacemaker implantation.

Diagnostic yield of an insertable cardiac monitor in a large patient population.

Background: Insertable cardiac monitors (ICMs) are increasingly used for cardiac rhythm diagnosis with expanding indications. Little has been reported about their use and efficacy. Objective: The study sought to evaluate the clinical utility of a novel ICM (Biotronik BIOMONITOR III) including the time to diagnosis in unselected patients with different ICM indications. Methods: Patients from 2 prospective clinical studies were included to determine the diagnostic yield of the ICM. The primary endpoint was time to clinical diagnosis per implant indication or to the first change in atrial fibrillation (AF) therapy. Results: A total of 632 patients were included with a mean follow-up of 233 ± 168 days. Of 384 patients with (pre)syncope, 34.2% had a diagnosis at 1 year. The most frequent therapy was permanent pacemaker implantation. Of 133 patients with cryptogenic stroke, 16.6% had an AF diagnosis at 1 year, resulting in oral anticoagulation. Of 49 patients with an indication for AF monitoring, 41.0% had a relevant change in AF therapy based on ICM data at 1 year. Of 66 patients with other indications, 35.4% received a rhythm diagnosis at 1 year. Moreover, 6.5% of the cohort had additional diagnoses: 26 of 384 patients with syncope, 8 of 133 patients with cryptogenic stroke, and 7 of 49 patients with AF monitoring. Conclusion: In a large unselected patient population with heterogeneous ICM indications, the primary endpoint of rhythm diagnosis was achieved in ∼1 in 4, and additional clinically relevant findings was achieved in 6.5% of patients at short-term follow-up.

Insertable cardiac monitor with a long sensing vector: Impact of obesity on sensing quality and safety.

Background: Fat layers in obese patients can impair R-wave detection and diagnostic performance of a subcutaneous insertable cardiac monitor (ICM). We compared safety and ICM sensing quality between obese patients [body mass index (BMI) ≥ 30 kg/m2] and normal-weight controls (BMI <30 kg/m2) in terms of R-wave amplitude and time in noise mode (noise burden) detected by a long-sensing-vector ICM. Materials and methods: Patients from two multicentre, non-randomized clinical registries are included in the present analysis on January 31, 2022 (data freeze), if the follow-up period was at least 90 days after ICM insertion, including daily remote monitoring. The R-wave amplitudes and daily noise burden averaged intraindividually for days 61-90 and days 1-90, respectively, were compared between obese patients (n = 104) and unmatched (n = 268) and a nearest-neighbour propensity score (PS) matched (n = 69) normal-weight controls. Results: The average R-wave amplitude was significantly lower in obese (median 0.46 mV) than in normal-weight unmatched (0.70 mV, P < 0.0001) or PS-matched (0.60 mV, P = 0.003) patients. The median noise burden was 1.0% in obese patients, which was not significantly higher than in unmatched (0.7%; P = 0.056) or PS-matched (0.8%; P = 0.133) controls. The rate of adverse device effects during the first 90 days did not differ significantly between groups. Conclusion: Although increased BMI was associated with reduced signal amplitude, also in obese patients the median R-wave amplitude was >0.3 mV, a value which is generally accepted as the minimum level for adequate R-wave detection. The noise burden and adverse event rates did not differ significantly between obese and normal-weight patients.Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT04075084 and NCT04198220.

Remote versus in-office monitoring for implantable cardioverter defibrillators: Results from a randomized pragmatic controlled study in Portugal.

INTRODUCTION: Remote monitoring (RM) is a safe and effective alternative to in-office conventional follow-up. OBJECTIVE: We aimed to evaluate patient satisfaction with RM and its impact on healthcare resources in a population with cardiac implantable electronic devices. METHODS: Randomized, pragmatic, open-label controlled trial, with adult wearers of implantable cardioverter-defibrillator (ICD) or cardiac resynchronization therapy with ICD (CRT-D), eligible for the CareLink® system. Patients newly implanted or with previous conventional follow-up were randomized to RM or conventional follow-up (control), and followed for 12 months, according to the centers' practice. The number of in-office visits and adverse events were compared between groups. Patient and healthcare professionals' satisfaction with RM were described. RESULTS: Of the 134 randomized patients (69 RM; 65 control, aged 60±13 years), 80% were male, 23% employed, 72% ICD wearers and 54% newly implanted. Most patients (70%) reported travel costs less than 15€/visit, and 46% daily routine interference with in-office visits. Median physician/technician time with patient was 15 min/15 min, per in-office visit. Excluding baseline and final visits, control patients had more in-office visits in total: median 1 vs. 0, p<0.001. In 81% of the in-office visits, no clinical measures were taken. There were 10 adverse events, with no differences between groups. At the final visit, 95% of RM patients considered RM easy/very easy to use, and would all prefer to maintain RM and recommend it to others. All professionals found the CareLink website easy/very easy to use and were satisfied with transmission data. CONCLUSIONS: In a Portuguese population with ICD and CRT-D, RM safely reduced the burden of in-office visits, with high levels of satisfaction among patients and healthcare professionals.

Left ventricular noncompaction associated with a pathogenic mutation in the MYH7 gene: Known mutation, different phenotype.

Left ventricular noncompaction (LVNC) is a genetically heterogeneous cardiomyopathy, with familial and sporadic forms, but genetic testing only identifies a pathogenic mutation in a minority of cases. The main complications are heart failure, embolism and dysrhythmias. Herein we report a familial case of LVNC associated with a mutation in the MYH7 gene and review the literature regarding controversies in LVNC. A 50-year-old woman was referred to the cardiology clinic for palpitations. She underwent echocardiography and cardiac magnetic resonance imaging that revealed mild left ventricular systolic dysfunction and LVNC criteria. She had several episodes of non-sustained ventricular tachycardia and received an implantable cardioverter-defibrillator (ICD). Genetic testing revealed the c.1003G>C (p.Ala335Pro) mutation in the MYH7 gene. Familial screening showed clear genotype-phenotype cosegregation, which provided strong evidence for the pathogenic role of this mutation. To the best of our knowledge, this is the first report of LVNC associated with the p.Ala335Pro mutation in the MYH7 gene. This mutation has been described in hypertrophic cardiomyopathy, suggesting that the same pathogenic sarcomere mutation may be associated with different cardiomyopathies. This case also highlights the current difficulties regarding decisions on ICD implantation for primary prevention of sudden cardiac death in LVNC.

New-generation miniaturized insertable cardiac monitor with a long sensing vector: Insertion procedure, sensing performance, and home monitoring transmission success in a real-world population.

Background: Insertable cardiac monitors (ICMs) require an invasive procedure and are used for purely diagnostic purposes. Therefore, simplicity of the insertion procedure, low complication rate, long-term patient acceptance, sensing quality, and reliable remote monitoring are of great importance. Objective: To evaluate a novel ICM (BIOMONITOR III) regarding all these aspects. Methods: BIOMONITOR III has a miniaturized profile, long sensing vector (≈70 mm), a fast insertion tool for pocket formation and ICM placement in 1 step, and daily automatic Home Monitoring (HM) function. We evaluated the insertion procedure, complication rate, patient acceptance, sensing quality, and HM performance in 653 patients with BIOMONITOR III inserted for any ICM indication within 2 ongoing studies involving 51 sites in 11 countries. Results: The median time from skin incision to wound closure was 4.0 minutes (interquartile range, 2.3-6.2 minutes). Median follow-up period was 274 days (interquartile range, 175-342 days). Serious adverse device-related events occurred in 6 patients (0.9%). No deep infections were reported in 334 patients without antibiotic prophylaxis. The wearing comfort was good or excellent in ≈95%. The mean R-wave amplitude (0.73 mV) and HM transmission rate (≈94% of days) were stable over 1.5 years. R-wave amplitudes were larger (mean 0.80 vs 0.62 mV, P < .001) and noise burden was lower (median 3.7 vs 14.5 minutes/day, P < .001) for ICM insertions parallel to the heart's long axis (54.2%) vs parasternal (41.3%). A gross visibility of P waves was 95.1%. Conclusion: The study demonstrated fast insertion times, low complication rate, high patient acceptance, and favorable long-term sensing and HM performance of the ICM.

Brugada syndrome: Eligibility for subcutaneous implantable cardioverter-defibrillator after exercise stress test.

INTRODUCTION: Brugada syndrome (BrS) is a channelopathy associated with ventricular arrhythmias and sudden cardiac death. In patients at high risk of sudden death, an implantable cardioverter-defibrillator is indicated. Subcutaneous implantable cardioverter-defibrillators (S-ICDs) are an alternative to transvenous systems, with reduced risk of infection and complications associated with system extraction or explantation. OBJECTIVE: To test electrocardiographic eligibility for S-ICD placement after exercise stress testing (EST) in patients with BrS. METHODS: The sample included 35 consecutive patients with BrS. Electrocardiographic eligibility was assessed using the Boston Scientific model 2889 EMBLEM™ S-ICD automated screening tool, in four phases: decubitus and orthostatism, and before and after EST. Those who had at least one acceptable vector in the four measurements were considered eligible. RESULTS: In this study, 71.4% of patients were male and mean age was 53.86±12 years. In screening prior to EST, 14.3% of patients (n=5) were not eligible for an S-ICD. There was a statistically significant association between ineligibility and presence of complete right bundle branch block and history of syncope. After EST, 16.7% of initially eligible patients no longer had eligible vectors (n=5). CONCLUSION: In this study, 16.7% of patients previously eligible for an S-ICD were no longer eligible after EST. This result demonstrates the importance of screening after EST in all patients with BrS and with indication for an S-ICD, and may influence decisions concerning which ICD to implant or whether to institute pharmacological measures that avoid inappropriate therapies.

Cardiac arrhythmias in patients presenting with COVID-19 treated in Portuguese hospitals: A national registry from the Portuguese Association of Arrhythmology, Pacing and Electrophysiology.

INTRODUCTION AND OBJECTIVES: In December 2019, SARS-CoV-2, was discovered as the agent of COVID-19 disease. Cardiac arrhythmias have been reported as frequent but their incidence is unknown. The aim of this research was to assess the real incidence of cardiac arrhythmias among COVID-19 patients admitted to Portuguese hospitals and to understand the underlying prognostic implications. METHODS: The Portuguese Association of Arrhythmology, Pacing and Electrophysiology (APAPE) conducted a survey in Portuguese hospitals to assess the occurrence of arrhythmias in COVID-19 patients, their clinical characteristics, the use of experimental therapies and the impact on QT interval. RESULTS: Twenty hospitals participated, reporting 692 hospitalized patients. An arrhythmic episode occurred in 81 (11.7%) and 64 (79%) had detailed information on these episodes. New onset arrhythmias occurred in 41 (64%) patients, 45 (70.3%) male, median age 73.5 (61-80.3) years. There were 51 (79.7%) with associated comorbidities, mainly arterial hypertension (41, 64.1%). Of 53 patients (82.3%) on experimental therapy, 7 (10.9%) had an increased QTc interval. Regarding arrhythmias, two patients (3.1%) had ventricular tachycardia, 5 (7.8%) sinus bradycardia, 17 (26.6%) paroxysmal supraventricular tachycardia and 40 (62.5%) atrial fibrillation or flutter. At the time of reporting, there had been no deaths due to arrhythmic syndrome or related complications. CONCLUSIONS: In a population of COVID-19 patients. The incidence of cardiac arrhythmias is high but not associated with increased cardiac mortality although it does though occur frequently in extremely ill patients and with multiple organ failure. Regardless of the use of experimental drugs, the incidence of ventricular arrhythmias is low and atrial fibrillation and other supraventricular arrhythmias are the most prevalent arrythmias.

INTRODUÇÃO E OBJETIVOS: Em dezembro de 2019, o SARS-CoV-2 foi descoberto como agente da doença Covid-19. As arritmias cardíacas são reportadas como frequentes, mas a sua incidência é desconhecida. O objetivo deste trabalho foi entender a incidência de arritmias em doentes Covid-19 tratados em hospitais portugueses e entender as suas implicações prognósticas. MÉTODOS: A Associação Portuguesa de Arritmologia, Pacing e Electrofisiologia (APAPE) conduziu um inquérito em hospitais portugueses, documentando a ocorrência de arritmias em doentes com Covid-19, as suas caraterísticas clínicas, o uso de terapêutica experimental e o seu impacto no intervalo QT. RESULTADOS: Participaram 20 hospitais, reportando 692 doentes hospitalizados. Ocorreram episódios arrítmicos em 81 (11,7%), 64 (79%) com informação adicional. Documentaram-se arritmias de novo em 41 (64%) doentes, 45 (79%) do sexo masculino, idade mediana 73,5 (61-80,3) anos. Destes, 51 (79,7%) tinham comorbilidades associadas, maioritariamente hipertensão arterial (41, 64,1%). Dos 53 (82,3%) doentes sob terapêutica experimental, 7 (10,9%) tiveram aumento do intervalo QTc. Tiveram taquicardia ventricular 2 (3,1%) doentes, 5 (7,8%) bradicardia sinusal, 17 (26,6%) taquicardia paroxística supraventricular e 40 (7,8%) fibrilhação ou flutter auricular. Nenhum doente teve morte por causa arrítmica ou complicações associadas, à data do registo. CONCLUSÕES: Numa população de doentes com Covid-19, a incidência de arritmias é elevada, mas não associada a aumento de mortalidade cardíaca, apesar da ocorrência mais frequente em doentes graves e com falência multiorgânica. Independentemente do uso de terapêuticas experimentais, a incidência de arritmias ventriculares é baixa e a fibrilhação auricular e outras arritmias supraventriculares são as arritmias mais prevalentes.

Cardiac arrhythmias in patients presenting with COVID-19 treated in Portuguese hospitals: A national registry from the Portuguese Association of Arrhythmology, Pacing and Electrophysiology.

INTRODUCTION AND OBJECTIVES: In December 2019, SARS-CoV-2, was discovered as the agent of COVID-19 disease. Cardiac arrhythmias have been reported as frequent but their incidence is unknown. The aim of this research was to assess the real incidence of cardiac arrhythmias among COVID-19 patients admitted to Portuguese hospitals and to understand the underlying prognostic implications. METHODS: The Portuguese Association of Arrhythmology, Pacing and Electrophysiology (APAPE) conducted a survey in Portuguese hospitals to assess the occurrence of arrhythmias in COVID-19 patients, their clinical characteristics, the use of experimental therapies and the impact on QT interval. RESULTS: Twenty hospitals participated, reporting 692 hospitalized patients. An arrhythmic episode occurred in 81 (11.7%) and 64 (79%) had detailed information on these episodes. New onset arrhythmias occurred in 41 (64%) patients, 45 (70.3%) male, median age 73.5 (61-80.3) years. There were 51 (79.7%) with associated comorbidities, mainly arterial hypertension (41, 64.1%). Of 53 patients (82.3%) on experimental therapy, 7 (10.9%) had an increased QTc interval. Regarding arrhythmias, two patients (3.1%) had ventricular tachycardia, 5 (7.8%) sinus bradycardia, 17 (26.6%) paroxysmal supraventricular tachycardia and 40 (62.5%) atrial fibrillation or flutter. At the time of reporting, there had been no deaths due to arrhythmic syndrome or related complications. CONCLUSIONS: In a population of COVID-19 patients. The incidence of cardiac arrhythmias is high but not associated with increased cardiac mortality although it does though occur frequently in extremely ill patients and with multiple organ failure. Regardless of the use of experimental drugs, the incidence of ventricular arrhythmias is low and atrial fibrillation and other supraventricular arrhythmias are the most prevalent arrythmias.

RIVER: Portuguese registry to monitor unnecessary right ventricular pacing.

The aim of this prospective registry is to evaluate a new algorithm designed to reduce the percentage of unnecessary ventricular pacing (%VP) in patients implanted with a dual-chamber pacemaker, through a dedicated pacing mode (called AAISafeR2) operating in AAI mode with back-up ventricular pacing in DDD mode, and to describe the incidence and distribution of atrioventricular (AV) block in this population. Investigators were free to assign patients to AAISafeR2 mode or to standard DDD (if AAISafeR was contraindicated, mainly due to permanent high-degree AV block). Patients underwent routine follow-up visits at 3, 6, 12, 18 and 24 months after implantation. At each follow-up visit, data were retrieved from pacemaker memories and analyzed to extract %VP and incidence of AV block. Up to December 2006, 158 patients (94 men, mean age 69 +/- 14 years) from nine Portuguese centers had been consecutively included. We also determined the distribution of AV block (according to the criteria used by the pacemaker to classify AV block and switch to DDD mode). AAISafeR was shown to be effective in reducing unnecessary VP in our patient population. The analysis also reveals a high incidence of paroxysmal AV block, often unknown at the time of implantation. There were no complications associated with AAISafeR programming.

Post mortem genetic test, the clinical diagnosis is not fade with the death of the patient. / Teste genético post mortem, o diagnóstico clínico não se esgota com a morte do doente.

In up to one-third of cases of sudden death, the medico-legal autopsy finding is inconclusive, and the option to perform a molecular autopsy is covered in international guidelines. The importance of postmortem genetic testing lies in its ability to identify hereditary diseases, often those with an autosomal dominant transmission pattern, and, through consultations and screening of relatives, to identify family members with a pathogenic mutation, who are often asymptomatic, providing an opportunity to change the course of their lives. The authors present three clinical cases that highlight the importance of postmortem genetic studies and family studies, as well as the integration of the data obtained in a cardiology consultation, which may be for arrhythmology, coronary disease or cardiomyopathy, depending on the specific condition. This could modify the course of the disease in many relatives.

Importance of cardiac implantable eletronic devices in the diagnosis of Sleep Apnea Syndrome. / Importância dos dispositivos eletrónicos cardíacos implantáveis no diagnóstico da Síndrome da Apneia do Sono.

INTRODUCTION: Sleep Apnea Syndrome (SAS) is a prevalent respiratory disease with marked expression in the population with cardiovascular disease. The diagnosis is based on polysomnography. In patients with cardiac implantable electronic devices (CIED), the prevalence of SAS may reach 60%. The objective of this study was to evaluate the value of DEC in the SAS screening. METHODS: Prospective study that included patients with CIED with sleep apnea algorithm. The frequency response function was activated and simplified polygraphy was performed. The data of the device were collected on the day of the polygraph. RESULTS: The sample included 29 patients, with a mean age of 76.1 years, 71.4% of the male gender. The prevalence of SAS was 77%. For SAS, the agreement between polysomnography and the Pacemaker was Kappa = 0.54 (p = 0.001), 95% CI (0.28, 0.81) (moderate agreement); for moderate to severe SAS, the agreement was Kappa = 0.73 (p <0.001), 95% CI (0.49, 0.976) (substantial agreement). Severe SAS was obtained: sensitivity 60%, specificity 100%, positive predictive value 100%, negative predictive value 60% and diagnostic accuracy 75%; for moderate to severe SAS: sensitivity of 90%, specificity of 83%, positive predictive values of 90% and negative of 87.5%, with a diagnostic accuracy of 87.5%. CONCLUSION: SAS is highly prevalent in patients with CIED. The values obtained through these devices have a strong positive correlation with the Apnea-Hypopnea Índex, which makes them a good tool for the screening of severe SAS.

Long QT syndrome with mutations in three genes: A rare case.

Congenital long QT syndrome (LQTS) is a rare hereditary disease, with an incidence of 1 in 2000, characterized by prolonged ventricular repolarization and malignant ventricular tachyarrhythmias. We report the case of a 30-year-old woman, previously diagnosed with neurocardiogenic syncope, in whom LQTS was identified. The patient received an implantable cardioverter-defibrillator due to polymorphic ventricular tachycardia under beta-blocker therapy. Molecular genetic testing identified three mutations in heterozygosity in the KCNH2, KCNQ1 and SCN5A genes, which is a rare finding and is associated with worse prognosis.

Use of an implantable loop recorder: initial experience.

OBJECTIVE: Retrospective analysis of the initial experience at our department with an implantable loop recorder (ILR) for the evaluation of patients with recurrent syncope, palpitations or dizziness. METHODS: We studied ten patients aged 31-65 (52.3) years referred to our center after an inconclusive evaluation that included ECG, Holter monitoring, events recorder, stress test, echocardiogram and electrophysiological study. Seven patients had structural heart disease. We used a Reveal Plus (Medtronic, USA) ILR, which allows up to 41 minutes of electrocardiographic recording, over a period of 24 months, patient or device activated, according to the programmed parameters. Follow-up was scheduled every three months or after every event activated by the patient. Symptoms and recorded events were analyzed. RESULTS: Eight patients activated events, out of a total of 45 episodes, and five patients reported two or more symptoms. Dizziness was reported by five patients (24 episodes), palpitations by three (seven episodes), syncope by three (seven episodes) and nonspecific symptoms by five patients (seven episodes). The first symptom occurred between the twelfth day and the sixth month after implantation. Four episodes (three syncope and one dizziness) occurred with significant AV conduction disturbance (complete or high grade AV block) and in 41 episodes no rhythm or AV disturbance was recorded. Thirty events were automatically activated. There were no device-related complications. None of the patients died during follow-up.