Real-world assessment of multipolar and point-by-point mapping for premature ventricular contraction ablation.

AIMS: We aimed to assess the acute and midterm efficacy of premature ventricular contraction (PVC) ablation guided by multielectrode and point-by-point (PbP) mapping. METHODS AND RESULTS: This is a retrospective, international multicentre study of consecutive patients referred for PVC ablation in 10 hospital centres from January 2017 to December 2021. Based on the mapping approach, two cohorts were identified: the 'Multipolar group', where a dedicated high-density mapping catheter was employed, and the 'PbP group', where mapping was performed with the ablation catheter. Procedural endpoints, safety, and acute (procedural) and midterm efficacies were assessed. Of the 698 patients included in this study, 592 received activation mapping [46% males, median age of 55 (41-65) years]-248 patients in the Multipolar group and 344 patients in the PbP group. A higher number of activation points [432 (217-843) vs. 95 (42-185), P < 0.001], reduced mapping time (40 ± 38 vs. 61 ± 50 min, P < 0.001), and shorter procedure time (124 ± 60 vs. 143 ± 63 min, P < 0.001) were reported in the Multipolar group. Both groups had high acute success rates (84.7% with Multipolar mapping vs. 81.3% with PbP mapping, P = 0.63), as well as midterm efficacy (83.4% vs. 77.4%, P = 0.08), with no significant differences in the risk of adverse events (6.0% vs. 3.5%, P = 0.24). However, for left-sided PVC ablation specifically, there was a higher midterm efficacy in the Multipolar group (80.7% vs. 69.5%, P = 0.04), with multipolar mapping being an independent predictor of success [adjusted OR = 2.231 (95% CI, 1.476-5.108), P = 0.02]. CONCLUSION: The acute and midterm efficacies of PVC ablation are high with both multipolar and PbP mapping, although the former allows for quicker procedures and may potentially improve the outcomes of left-sided PVC ablation.

Ostial vs. wide area circumferential ablation guided by the Ablation Index in paroxysmal atrial fibrillation.

AIMS: Pulmonary vein isolation (PVI) guided by the Ablation Index (AI) has shown high acute and mid-term efficacy in the treatment of paroxysmal atrial fibrillation (AF). Previous data before the AI-era had suggested that wide-area circumferential ablation (WACA) was preferable to ostial ablation. However, with the use of AI, we hypothesize that ostial circumferential ablation is non-inferior to WACA and can improve outcomes in paroxysmal AF. METHODS AND RESULTS: Prospective, multicentre, non-randomized, non-inferiority study of consecutive patients were referred for paroxysmal AF ablation from January 2020 to September 2021. All procedures were performed using the AI software, and patients were separated into two different groups: WACA vs. ostial circumferential ablation. Acute reconnection, procedural data, and 1-year arrhythmia recurrence were assessed. During the enrolment period, 162 patients (64% males, mean age of 60 ± 11 years) fulfilled the study inclusion criteria-81 patients [304 pulmonary vein (PV)] in the WACA group and 81 patients (301 PV) in the ostial group. Acute PV reconnection was identified in 7.9% [95% confidence interval (CI), 4.9-11.1%] of PVs in the WACA group compared with 3.3% (95% CI, 1.8-6.1%) of PVs in the ostial group [P < 0.001 for non-inferiority; adjusted odds ratio 0.51 (95% CI, 0.23-0.83), P = 0.05]. Patients in the WACA group had longer ablation (35 vs. 29 min, P = 0.001) and procedure (121 vs. 102 min, P < 0.001) times. No significant difference in arrhythmia recurrence was seen at 1-year of follow-up [11.1% in WACA vs. 9.9% in ostial, hazard ratio 1.13 (95% CI, 0.44-1.94), P = 0.80 for superiority]. CONCLUSION: In paroxysmal AF patients treated with tailored AI-guided PVI, ostial circumferential ablation is not inferior to WACA with regard to acute PV reconnection, while allowing quicker procedures with less ablation time.

Assessment of the need of a waiting period after pulmonary vein isolation with the ablation index software.

PURPOSE: Since the widespread availability of contact-force sensing catheters, the need for a waiting period after pulmonary vein isolation (PVI) has not been reassessed. We aim to evaluate whether a waiting period is still necessary after PVI guided by the ablation Index (AI). METHODS: Prospective, multicenter, randomized study of consecutive patients referred for paroxysmal atrial fibrillation (AF) ablation from May 2019 to February 2020. Patients were randomized in a 1:1 ratio to PVI with versus without a waiting period of 20 min. Acute pulmonary vein (PV) reconnection after adenosine challenge was the primary endpoint. A per-protocol analysis was designed to determine whether a strategy of dismissing the waiting period after PVI was noninferior to waiting for 20 min for identifying acute PV reconnection. PVI was guided by tailored AI values and an interlesion distance ≤6 mm. RESULTS: During the enrollment period, 167 patients (56% males, mean age of 57 ± 14 years) fulfilled the study inclusion criteria - 84 patients (308 PV) in the waiting period group (Group A) and 83 patients (314 PV) in the group without a waiting period (Group B). Acute PV reconnection was identified in 3.8% (95% confidence interval [CI], 1.7%-5.9%) of PVs in the study group B compared to 2.9% (95% CI, 1.0%-4.8%) of PVs in the Group A (p = .002 for non-inferiority). At 1-year follow-up, there was no significant difference in arrhythmia recurrence between groups (9.5% in Group A vs. 9.6% in Group B, hazard ratio: 1.03 [95% CI, 0.39-2.73], p = .98). CONCLUSION: In paroxysmal AF patients submitted to ablation, a tailored PVI guided by the AI rendered a 20-min waiting period unnecessary.

Can Pre-Ablation Biomarkers Be Used to Predict Arrhythmia Recurrence after Ablation Index-Guided Atrial Fibrillation Ablation? / Os Biomarcadores podem ser Utilizados para Prever Recorrência de Arritmia após a Ablação de Fibrilação Atrial Guiada pelo Ablation-Index?

BACKGROUND: Ablation Index (AI) software has allowed better atrial fibrillation (AF) ablation results, but recurrence rates remain significant. Specific serum biomarkers have been associated with this recurrence. OBJECTIVES: To evaluate whether certain biomarkers could be used (either individually or combined) to predict arrhythmia recurrence after AI-guided AF ablation. METHODS: Prospective multicenter observational study of consecutive patients referred for AF ablation from January 2018 to March 2021. Hemoglobin, brain natriuretic peptide (BNP), C-reactive protein, high sensitivity cardiac troponin I, creatinine clearance, thyroid-stimulating hormone (TSH) and free thyroxine (FT4) were assessed for their ability to predict arrhythmia recurrence during follow-up. Statistical significance was accepted for p values of<0.05. RESULTS: A total of 593 patients were included - 412 patients with paroxysmal AF and 181 with persistent AF. After a mean follow-up of 24±6 months, overall single-procedure freedom from atrial arrhythmia was 76.4%. Individually, all biomarkers had no or only modest predictive power for recurrence. However, a TSH value >1.8 µUI/mL (HR=1.82 [95% CI, 1.89-2.80], p=0.006) was an independent predictor of arrhythmia recurrence. When assessing TSH, FT4 and BNP values in combination, each additional "abnormal" biomarker value was associated with a lower freedom from arrhythmia recurrence (87.1 % for no biomarker vs. 83.5% for one vs. 75.1% for two vs. 43.3% for three biomarkers, p<0.001). Patients with three "abnormal" biomarkers had a threefold higher risk of AF recurrence compared with no "abnormal" biomarker (HR=2.88 [95% CI, 1.39-5.17], p=0.003). CONCLUSIONS: When used in combination, abnormal TSH, FT4 and BNP values can be a useful tool for predicting arrhythmia recurrence after AI-guided AF ablation.

FUNDAMENTO: O software ablation index (AI) permitiu melhorar os resultados da ablação de fibrilação atrial (FA), mas as taxas de recorrência permanecem significativas. Biomarcadores séricos específicos têm sido associados a essa recorrência. OBJETIVOS: Avaliar se certos biomarcadores podem ser utilizados (individualmente ou combinados) para predizer a recorrência de FA pós ablação guiada pelo AI. MÉTODOS: Estudo multicêntrico, observacional, prospectivo de pacientes consecutivos, encaminhados para ablação de FA de janeiro de 2018 a março de 2021. Hemoglobina, peptídeo natriurético cerebral (BNP), proteína C reativa, troponina I ultrassensível, clearance de creatinina, Hormônio Tireoestimulante (TSH), e Tiroxina livre (T4) foram avaliados quanto à capacidade de prever a recorrência de arritmias durante o acompanhamento. Valores de p <0,05 foram aceitos como estatisticamente significativos. RESULTADOS: Um total de 593 pacientes foram incluídos ­ 412 com FA paroxística e 181 com FA persistente. Durante o seguimento médio de 24±6 meses, 76,4% não apresentaram recidiva após ablação. Individualmente, os biomarcadores demonstraram um valor preditivo baixo ou nulo para recorrência. No entanto, TSH >1,8 µUI/mL [HR=1,82 (IC95%, 1,89-2,80), p=0,006] foi um preditor independente de recorrência. Avaliando-se a combinação de TSH, FT4 e BNP, a adição de cada valor "anormal" foi associada a uma menor sobrevida livre de recorrência (87,1% se nenhum vs. 83,5% se um vs. 75,1% se dois vs. 43,3% se três biomarcadores, p<0,001). Doentes com três biomarcadores "anormais" apresentaram três vezes maior probabilidade de recorrência de FA, comparativamente aos que não apresentaram nenhum biomarcador "anormal" (HR=2,88 [IC95%, 1,39-5,17], p=0,003). CONCLUSÕES: Quando combinados, valores anormais de TSH, FT4 e BNP podem ser uma ferramenta útil para prever a recorrência de FA pós ablação guiada pelo AI.

Long-term prognosis of idiopathic ventricular fibrillation: An eighteen-year experience from a tertiary center.

INTRODUCTION AND OBJECTIVES: Idiopathic ventricular fibrillation (IVF) is diagnosed in patients who survive sudden cardiac arrest (SCA), preferably with documented ventricular fibrillation (VF), without any identifiable structural or electrical abnormality. Current evidence provides limited guidance on the diagnosis and follow-up of these patients. Our aim was to assess the clinical outcomes of survivors of an aborted SCA attributed to IVF. METHODS: We retrospectively collected clinical data from all patients who survived SCA and implanted a cardiac defibrillator (ICD) between 2005 and 2023. RESULTS: A total of 38 patients, 36.8% female, with a mean age of 44±14 years old were included. Median follow-up time was 8.7 years (interquartile range (IQR) 4.7-14.7 years). All patients underwent a comprehensive diagnostic evaluation that excluded structural and coronary disease. During follow-up, underlying diagnoses were established in 34.2% of the whole cohort. Genetic testing, performed in 37.2%, revealed underlying diagnoses in 57.1% of those tested, compared to only 26.3% of patients who did not undergo genetic testing [p=0.035, OR=5.1 (95% confidence interval (CI) 1.2-21.5)]. Mortality was 10.5% (due to non-arrhythmic causes) and 36.8% patients received appropriate therapies with a median time to first ICD therapy of 39 [5.4-47.3] months. CONCLUSION(S): Etiological diagnosis and recurrence prediction in patients with IVF remains challenging, even with extensive diagnostic evaluation and long-term follow-up. In our study, genetic testing enhanced diagnostic yield. Consistent with previous findings, our cohort experienced a notable arrhythmic recurrence, with no cardiac deaths, underlining the pivotal role of ICD implantation in these patients.

ECMO for drug-refractory electrical storm without a reversible trigger: a retrospective multicentric observational study.

AIMS: Drug-refractory electrical storm (ES) is a life-threatening medical emergency. We describe the use of venoarterial extracorporeal membrane oxygenation (VA-ECMO) in drug-refractory ES without a reversible trigger, for which specific guideline recommendations are still lacking. METHODS AND RESULTS: Retrospective observational study in four Iberian centres on the indications, treatment, complications, and outcome of drug-refractory ES not associated with acute coronary syndromes, decompensated heart failure, drug toxicity, electrolyte disturbances, endocrine emergencies, concomitant acute illness with fever, or poor compliance with anti-arrhythmic drugs, requiring VA-ECMO for circulatory support. Thirty-four (6%) out of 552 patients with VA-ECMO for cardiogenic shock were included [71% men; 57 (44-62) years], 65% underwent cardiopulmonary resuscitation before VA-ECMO implantation, and 26% during cannulation. Left ventricular unloading during VA-ECMO was used in 8 (24%) patients: 3 (9%) with intraaortic balloon pump, 3 (9%) with LV vent, and 2 (6%) with Impella. Thirty (88%) had structural heart disease and 8 (24%) had an implantable cardioverter-defibrillator. The drug-refractory ES was mostly due to monomorphic ventricular tachycardia (VT) and ventricular fibrillation (VF) (59%), isolated monomorphic VT (26%), polymorphic VT (9%), or VF (6%). Thirty-one (91%) required deep sedation, 44% overdrive pacing, 36% catheter ablation, and 26% acute autonomic modulation. The main complications were nosocomial infection (47%), bleeding (24%), and limb ischaemia (21%). Eighteen (53%) were weaned from VA-ECMO, and 29% had heart transplantation. Twenty-seven (79%) survived to hospital discharge (48 (33-82) days). Non-survivors were older [62 (58-67) vs. 54 (43-58); P < 0.01] and had a higher first rhythm disorder-to-ECMO interval [0 (0-2) vs. 2 (1-11) days; P = 0.02]. Seven (20%) had rehospitalization during follow-up [29 (12-48) months], with ES recurrence in 6%. CONCLUSIONS: VA-ECMO bridged drug-refractory ES without a reversible trigger with a high success rate. This required prolonged hospital stays and coordination between the ECMO centre, the electrophysiology laboratory, and the heart transplant programme.

High-power short-duration versus low-power long-duration ablation guided by the ablation index.

AIMS: To compare the two different ablation strategies, both guided by the Ablation Index (AI), in the setting of atrial fibrillation (AF) ablation: high-power short-duration (HPSD) ablation using 40 W on the posterior wall and 50 W elsewhere versus low-power long-duration (LPLD) using 25 W posteriorly and 35 W elsewhere. METHODS: Prospective, multicenter nonrandomized, noninferiority study of consecutive patients referred for paroxysmal AF ablation from January 2018 to July 2019. Ablation was guided by the AI (≥500 for anterior segments, ≥450 for the roof and inferior segments and 400 posteriorly) and an interlesion distance (ILD) ≤ 6 mm. Patients were separated into two groups: HPSD vs LPLD. Acute reconnection (after adenosine trial) and 2-year outcomes were assessed. RESULTS: 160 patients (61% males, median age of 62 [IQR 51-69] years), fulfilled the study inclusion criteria - 80 patients (316 pulmonary veins [PV]) in the HPSD group and 80 patients (314 PV) in the LPLD. The probability of acute PV reconnection was similar between both groups: 2.2% in HPSD, 95%CI 0.6% to 3.8% vs. 3.4% in LPLD, 95%CI 1.4% to 5.4%; p < 0.001 for noninferiority. Median PV ablation time (20 min vs 30 min, p < 0.01) and procedure duration (80 min vs 100 min, p < 0.001) were shorter in the HPSD group. After a median follow-up of 26 months, arrhythmia recurrence was similar between groups (17.5% in HPSD group vs. 18.8% in LPLD group, p = 0.79). CONCLUSIONS: In paroxysmal AF patients treated with the Ablation Index, a HPSD strategy is noninferior to the more standard LPLD ablation, while allowing for quicker procedures with shorter ablation times.

Transcatheter closure of an iatrogenic aorto-right ventricular fistula.

Aortocardiac fistulas are rare, especially if they develop after an aortic valve replacement surgery. We report the case of a 54-year-old male submitted to aortic valve replacement and implantation of an ascending aortic prosthetic graft, complaining of exertional dyspnea, who was found to have significant shunt between the aortic root and right ventricle (RV), and de novo moderate pulmonary hypertension. At the catheterization laboratory, the left-to-right shunt was confirmed (Qp:Qs = 1.9:1). Contrast angiography of the ascending aorta showed a significant flow into the right ventricular cavity, and the fistulous tract was then measured, inflating a Tyshak II balloon of 10 × 20 mm (NuMED, Hopkinton, New York), until achieving a complete interruption of flow. Minimal diameter of the defect was 4.9 mm. Percutaneous closure of the aorto-RV shunt was performed under general anesthesia and transesophageal echocardiogram and fluoroscopic guidance. Using a venous and an arterial femoral access, a 0.035″ hydrophilic guide-wire crossed the defect between the aorta and RV, creating an arteriovenous loop. Then, using a 7F Delivery System 45° (AGA medical corporation, Golden Valley, MN) an Amplatzer Duct Occluder(®) (AGA Medical Corporation) 8/6 mm was advanced and released within the defect, achieving an almost complete closure of the fistulous tract.

Long-term follow-up of patients with postural tachycardia syndrome.

Postural tachycardia syndrome (POTS) refers to the presence of orthostatic intolerance symptoms with a heart rate increment ≥ 30 bpm, usually up to ≥ 120 bpm. Pathophysiology and POTS's clinical presentation are heterogeneous and its prognosis is uncertain. We reviewed the major clinical characteristics of POTS patients and assessed their long-term follow-up. Our series results, one with the longest follow-up, illustrate POTS as a clinical entity with variable, but usually benign outcome, in which most patients can reassume their daily activities without great limitations, after proper diagnosis and treatment are made.

Diagnostic challenges of Marfan syndrome in an XYY young man.

Tall stature is a common feature of both Marfan syndrome and XYY syndrome. Differential diagnosis between these entities has important prognostic implications. We report the case of a 21-year-old young man with a previously known diagnosis of XYY syndrome, in whom the identification of a fibrilin-1 mutation was determinant to establish an appropriate diagnosis, medical follow-up, and genetic counselling.

An atypical presentation of infective endocarditis.

Infective endocarditis is a well-known clinical entity. However, despite improved diagnostic techniques and advances in treatment options, left-sided native valve infective endocarditis remains a serious disease with high morbidity and mortality, especially in cases caused by Staphylococcus aureus. The clinical heterogeneity of infective endocarditis sometimes prevents rapid recognition, correct diagnosis and timely treatment, which are essential to reduce the morbidity and mortality associated with this disease. We report the case of a 62-year-old man, admitted for atrial fibrillation with complete atrioventricular block, which was found to be the result of methicillin-resistant S. aureus mitral valve endocarditis, complicated by local extension of the infection, heart failure, systemic embolism and multiple organ failure.

Acute coronary syndrome, right ventricular dysfunction and cardiogenic shock: a diagnostic challenge.

Right ventricular infarction is uncommon in isolation but can be observed in 50% of cases of inferior wall myocardial infarction. Diagnosis is difficult and suspicion of this condition should always be borne in mind. Progression to cardiogenic shock is not uncommon, when the outcome is similar to left ventricular infarction; mortality can reach 60%. We present the case of a 64-year-old woman with known coronary disease who was admitted to our coronary care unit after an anterior myocardial infarction. Cardiac catheterization showed diffuse stenosis of the left descending and 70% stenosis of the posterior descending arteries. She was surgically revascularized with a favorable evolution, but was later readmitted for acute decompensated heart failure with cardiogenic shock. She was refractory to medical therapy, with biventricular dysfunction on echocardiographic examination. Cardiac magnetic resonance imaging confirmed the diagnosis of right ventricular infarction.

Iatrogenic ventricular fibrillation in Wolff-Parkinson-White syndrome.

Wolff-Parkinson-White (WPW) syndrome is the most common manifestation of ventricular pre-excitation syndrome and is mostly found in individuals with no structural heart disease. Although the risk of malignant arrhythmias is low, sudden cardiac death (SCD) as the first clinical manifestation of WPW syndrome is well documented, and atrial fibrillation (AF) with a rapid ventricular response is the main mechanism involved. Unfortunately, the signs of pre-excitation and arrhythmias are sometimes under-diagnosed and under-treated. We describe the case of a 31-year-old man who was admitted with an irregular wide complex tachycardia consistent with pre-excited AF, which was not promptly diagnosed, and who developed ventricular fibrillation (VF) after administration of atrioventricular (AV) nodal blockers, as a primary manifestation of WPW syndrome. Blocking the AV node in patients with pre-excited AF may increase the ventricular rate and potentially result in hemodynamic instability. Among patients with WPW syndrome who survive an episode of SCD, catheter ablation of the accessory pathway is the treatment of choice.

Long-Term Ventricular Pacing Dependency and Pacemaker Implantation Predictors after Transcatheter Aortic Valve Replacement - A 1-Year Follow-Up. / Dependência de Pacing a Longo-Prazo e Preditores de Implante de Pacemaker após Implante Percutâneo de Prótese Valvular Aórtica ­ 1 Ano de Seguimento.

BACKGROUND: Conduction disturbances (CD) are the most frequent complication after transcatheter aortic valve replacement (TAVR), and there continues to be a lack of consensus on their management. OBJECTIVE: To assess new CD and permanent pacemaker (PPM) implantation after TAVR and to evaluate the ventricular pacing percentage (VP) up to 1 year of follow-up. METHODS: Patients who underwent TAVR from October 2014 to November 2019 were enrolled; patients with previous PPM were excluded. Clinical, procedure, ECG, and PPM data were collected up to 1 year after implantation. The significance level adopted in the statistical analysis was 0.05. RESULTS: A total of 340 patients underwent TAVR. The most frequent CD was the new left bundle branch block (LBBB; 32.2%), which 56% resolved after 6 months. Right bundle branch block (RBBB) was the biggest risk factor for advanced atrioventricular block (AVB) [OR=8.46; p<0.001] and PPM implantation [OR=5.18, p<0.001], followed by previous low-grade AVB [OR=2.25; p=0.016 for PPM implantation]. Regarding procedure characteristics, newer generation valves and valve-in-valve procedures were associated with fewer CDs. Overall, 18.5% of patients had a PPM implanted post-TAVR. At first PPM evaluation, patients with advanced AVB had a median percentage of VP of 80% and 83% at one year. Regarding patients with LBBB plus low-grade AVB, median VP was lower (6% at first assessment, p=0.036; 2% at one year, p = 0.065). CONCLUSION: LBBB was the most frequent CD after TAVR, with more than half being resolved in the first six months. RBBB was the major risk factor for advanced AVB and PPM implantation. Advanced AVB was associated with a higher percentage of VP at 1 year of follow-up.

FUNDAMENTO: Os distúrbios de condução (DC) são a complicação mais frequente após a substituição da válvula aórtica transcateter (TAVR) e ainda não há consenso sobre seu tratamento. OBJETIVO: Avaliar novos DC e implante de marca-passo definitivo (MPD) após a TAVR e avaliar a porcentagem de estimulação ventricular (EV) até 1 ano de acompanhamento. MÉTODOS: Pacientes submetidos a TAVR de outubro de 2014 a novembro de 2019 foram cadastrados; pacientes com MPD anterior foram excluídos. Dados clínicos, do procedimento, do ECG e do MPD foram coletados até 1 ano após o implante. O nível de significância adotado para a análise estatística foi 0,05%. RESULTADOS: Um total de 340 indivíduos foram submetidos a TAVR. O DC mais comum foi bloqueio de ramo esquerdo novo (BRE; 32,2%), sendo que 56% destes foram resolvidos após 6 meses. O bloqueio do ramo direito (BRD) foi o maior fator de risco para bloqueio atrioventricular avançado (BAV) [RC=8,46; p<0,001] e implante de MPD [RC=5,18; p<0,001], seguido de BAV de baixo grau prévio [RC=2,25; p=0,016 para implante de MPD]. Em relação às características do procedimento, válvulas de gerações mais recentes e procedimentos de válvula-em-válvula foram associados a menos DC. No total, 18,5% dos pacientes tiveram MPD implantado após a TAVR. Na primeira avaliação do MPD, pacientes com BAV avançado tinham uma porcentagem mediana de EV de 80%, e, após um ano, de 83%. Em relação aos pacientes com BRE e BAV de baixo grau, a EV mediana foi mais baixa (6% na primeira avaliação, p=0,036; 2% após um ano, p = 0,065). CONCLUSÃO: O BRE foi o DC mais frequente após a TAVR, com mais da metade dos casos se resolvendo nos primeiros 6 meses. O BRD foi o principal fator de risco para BAV avançado e implante de MPD. O BAV avançado foi associado a uma porcentagem mais alta de EV no acompanhamento de 1 ano.

Two years after pulmonary vein isolation guided by ablation index-a multicenter study.

Background: The use of the Ablation Index (AI) software for paroxysmal atrial fibrillation (AF) has been associated with higher acute effectiveness and higher 1-year arrhythmia freedom. There is, however, a lack of data concerning longer follow-up. We aim to evaluate the 2-year outcomes after a standardized AI-guided pulmonary vein isolation (PVI). Methods: Prospective, multicenter study of consecutive patients referred for paroxysmal AF ablation from January 2018 to July 2019. PVI was guided by a tailored AI value (≥500 for anterior segment, ≥450 for the roof segments and inferior segments, and 400 for the posterior wall) and an ILD ≤6 mm. The primary endpoints were acute and long-term effectiveness. Results: The study included 218 (842 PV) patients (61% males, median age of 60 [IQR 49-68] years) with paroxysmal AF. First-pass isolation was obtained in 93% of the patients, with an acute reconnection occurring in 10.6% of the patients (3.2% of the PV) following adenosine trial. After a median follow-up of 26 (IQR 20-30) months, freedom from any documented atrial arrhythmia was 83.4%, off-AAD. The rate of adverse events was 1.4%. Although procedural parameters differ across centers (p < 0.001), the acute (p = 0.56) and long-term effectiveness (p = 0.83) were consistent between centers. Conclusions: Patients with paroxysmal AF submitted to an AI-guided PVI workflow presented high arrhythmia freedom at 2-years of follow-up.

Atypical ST segment elevation and ventricular fibrillation without structural heart disease: a new electrocardiographic presentation of a channelopathy?

Primary electrical syndromes are a group of rare inherited diseases that predispose to arrhythmias in the absence of structural abnormalities of the heart, and are associated with several ion channel mutations. Extrinsic factors, such as fever, may contribute to the development of electrical instability in these patients. We report the case of a 52-year-old patient who was admitted for syncope and had an in-hospital episode of ventricular fibrillation, who presented with an admission ECG showing marked precordial ST segment elevation (maximum 5 mm in V2). The patient did not have structural heart disease and during the hospital stay there was progressive ST segment normalization, with features suggestive of Brugada pattern. An automated defibrillator was implanted for secondary prevention of sudden cardiac arrest. We believe that these findings may represent a new form of presentation of a genetic electrical syndrome.

[Percutaneous closure of a giant coronary artery fistula draining into superior vena cava]. / Encerramento percutâneo de uma fístula coronária gigante com drenagem na veia cava superior.

Coronary artery fistulas are rare coronary anomalies; coronary angiography remains the gold standard for the diagnosis, and is essential to plan either surgical or percutaneous intervention. The use of an Amplatzer Vascular Plug device in percutaneous closure of coronary fistulas has been recently described in a few cases of paediatric age patients, with positive results, but has never been reported in adult patients. We report the case of a 36-year-old female, with an unusually large right coronary artery fistula draining into superior vena cava that was successfully closed percutaneously, using a 14 mm Amplatzer device (Vascular Plug II, AGA). We also discuss our therapeutic option, since management of large, asymptomatic fistulas, is still a matter of debate, although most authors tend to recommend its closure.

Marfan syndrome: clinical manifestations, pathophysiology and new outlook on drug therapy.

Marfan Syndrome (MFS) is a genetic disorder of the connective tissue with multisystemic manifestations, which typically involves the skeletal, cardiovascular and ocular systems. It is usually associated with fibrillin-1 (FBN1) gene mutations, an extracellular matrix protein, and its diagnosi requires the presence of several clinical criteria, called the Ghent criteria. Studies with animal models have helped understand some of the pathophysiologic mechanisms of the syndrome, and the core role of transforming growth factor, (TGF-beta) signalling pathways in these mutations. These findings created new therapeutic opportunities, like the use of losartan, known to have an antagonistic effect on TGF-beta. With the aging of this population, new clinical manifestations are expected, requiring close and continued MFS patient monitoring.

[Prothrombotic events after percutaneous closure of an atrial septal defect]. / Complicações pró-trombóticas após o encerramento percutâneo de comunicação interauricular.

We describe the case of a 66 years-old patient, submitted to percutaneous closure of an ostium secundum atrial septal defect ( ASD ), with an " Amplatzer ASO 20 mm " ( Medical AGA Corp., MN (. There were no immediate complications and no residual leak. Eleven days after the procedure, atrial fibrillation ( AF ) ensued, followed by an acute myocardial infarction ( distal occlusion of the circumflex artery ). Three days later, this patient had also a transitory ischemic accident. Percutaneous closure of ASD is a procedure that can be associated with a few complications, namely AF and thromboembolic events. Although common, the use of dual antiplatelet therapy doesn't have an established efficacy to prevent these cases.

Recommendations for genetic testing in cardiology: Review of major international guidelines. / Recomendações para a realização de testes genéticos em cardiologia ­ revisão das principais diretrizes internacionais.

In recent years, the importance of genetic causes of cardiovascular diseases has been increasingly recognized, as the result of significant advances in molecular diagnosis techniques. This growing knowledge has enabled the identification of new phenotypes and the subclassification of clinical syndromes, impacting the therapeutic approach and genetic counseling offered to affected families. This paper describes the state of the art of genetic testing in the main cardiovascular diseases, aiming to provide a useful tool to help cardiologists and other health professionals involved in the care of individuals with hereditary heart diseases and their families.