Genetic Testing in Brugada Syndrome: A 30-Year Experience.

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.

Genetic testing in children with Brugada syndrome: results from a large prospective registry.

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.

First experience with a transseptal puncture using a novel transseptal crossing device with integrated dilator and needle.

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.

Hybrid-Approach Ablation in Drug-Refractory Arrhythmogenic Right Ventricular Cardiomyopathy.

Management of ventricular arrhythmias (VAs) beyond implantable cardioverter-defibrillator positioning in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) is challenging. Catheter ablation of the ventricular substrate often requires a combination of endocardial and epicardial approaches, with disappointing outcomes due to the progressive nature of the disease. We report the Universitair Ziekenhuis Brussel experience through a case series of 16 patients with drug-refractory ARVC, who have undergone endocardial and/or epicardial catheter ablation of VAs with a thoracoscopic hybrid-approach. After a mean follow-up time of 5.16 years (SD 2.9 years) from the first hybrid-approach ablation, VA recurrence was observed in 5 patients (31.25%): among these, patients 4 patients (80%) received a previous ablation and 1 of 11 patients (9.09%) who had a hybrid ablation as first approach had a VA recurrence (80% vs 9.09%; log-rank p = 0.04). Despite the recurrence rate of arrhythmic events, all patients had a significant reduction in the arrhythmic burden after ablation, with a mean of 4.65 years (SD 2.9 years) of freedom from clinically significant arrhythmias, defined as symptomatic VAs or implantable cardioverter-defibrillator-delivered therapies. In conclusion, our case series confirms that management of VAs in patients with ARVC is difficult because patients do not always respond to antiarrhythmic medications and can require multiple invasive procedures. A multidisciplinary approach involving cardiologists, cardiac surgeons, and cardiac electrophysiologists, together with recent cardiac mapping techniques and ablation tools, might mitigate these difficulties and improve outcomes.

Incidence and Predictors of Cardiac Arrhythmias in Patients With COVID-19.

Background: Coronavirus disease 2019 (COVID-19) is a systemic disease caused by severe acute respiratory syndrome coronavirus 2. Arrhythmias are frequently associated with COVID-19 and could be the result of inflammation or hypoxia. This study aimed to define the incidence of arrhythmias in patients with COVID-19 and to correlate arrhythmias with pulmonary damage assessed by computed tomography (CT). Methods: All consecutive patients with a COVID-19 diagnosis hospitalized at Universitair Ziekenhuis Brussel, Belgium, between March 2020 and May 2020, were screened. All included patients underwent a thorax CT scan and a CT severity score, a semiquantitative scoring system of pulmonary damage, was calculated. The primary endpoint was the arrhythmia occurrence during follow-up. Results: In this study, 100 patients were prospectively included. At a mean follow-up of 19.6 months, 25 patients with COVID-19 (25%) experienced 26 arrhythmic episodes, including atrial fibrillation in 17 patients, inappropriate sinus tachycardia in 7 patients, atrial flutter in 1 patient, and third-degree atrioventricular block in 1 patient. No ventricular arrhythmias were documented. Patients with COVID-19 with arrhythmias showed more often need for oxygen, higher oxygen maximum flow, longer QTc at admission, and worse damage at CT severity score. In univariate logistic regression analysis, significant predictors of the primary endpoint were: the need for oxygen therapy (odds ratio [OR] 4.59, 95% CI 1.44-14.67, p = 0.01) and CT severity score of pulmonary damage (OR per 1 point increase 1.25, 95% CI 1.11-1.4, p < 0.001). Conclusions: In a consecutive cohort of patients with COVID-19 the incidence of cardiac arrhythmias was 25%. The need for oxygen therapy and CT severity score were predictors of arrhythmia occurrence during follow-up.

Comparison between the novel diamond temp and the classical 8-mm tip ablation catheters in the setting of typical atrial flutter.

PURPOSE: Radiofrequency (RF) catheter ablation is widely accepted as a first-line therapy for cavotricuspid isthmus (CTI)-dependent atrial flutter (AFL). The novel DiamondTemp (DT) catheter with temperature feedback during RF ablation has been released recently on the market. The purpose of this study was to evaluate the impact of DiamondTemp (DT) technology on ablation efficiency during AFL. METHODS: In this single-center study, 30 consecutive patients with typical AFL indicated to ablation of CTI were included. The first 15 patients underwent CTI ablation using 8-mm tip catheter, and the following 15 patients underwent temperature-controlled RF ablation using DT catheter. The endpoints were number and mean total duration of RF applications, mean temperature reached in the setting of CTI, procedural times, and fluoroscopy times. RESULTS: There were no significant differences between the two groups concerning baseline characteristics. Mean duration of the each application (71.5 s ± 30.6 vs 12.4 s ± 13.2, p value < 0.001), mean total duration of RF applications (517,73 s ± 377,96 vs 112,8 s ± 43,58; p value < 0.001), procedural times (51.6 min ± 24.2 vs 38.6 ± 8.2; p = 0.03), and fluoroscopy times (16.2 min ± 10.2 vs 8 min ± 4.24; p = 0.005) were longer in the 8-mm ablation catheter group. Mean temperature measurements (51.9 °C ± 3.59 vs 56.7 °C ± 3.34, p value < 0.003) were as well lower in the 8-mm ablation catheter group. CONCLUSIONS: Catheter ablation of CTI-dependent AFL by means of DT resulted in a significant reduction of total and single application RF delivery time, procedure, and fluoroscopy times.

Ajmaline-Induced Abnormalities in Brugada Syndrome: Evaluation With ECG Imaging.

Background The rate of sudden cardiac death (SCD) in Brugada syndrome (BrS) is ≈1%/y. Noninvasive electrocardiographic imaging is a noninvasive mapping system that has a role in assessing BrS depolarization and repolarization abnormalities. This study aimed to analyze electrocardiographic imaging parameters during ajmaline test (AJT). Methods and Results All consecutive epicardial maps of the right ventricle outflow tract (RVOT-EPI) in BrS with CardioInsight were retrospectively analyzed. (1) RVOT-EPI activation time (RVOT-AT); (2) RVOT-EPI recovery time, and (3) RVOT-EPI activation-recovery interval (RVOT-ARI) were calculated. ∆RVOT-AT, ∆RVOT-EPI recovery time, and ∆RVOT-ARI were defined as the difference in parameters before and after AJT. SCD-BrS patients were defined as individuals presenting a history of aborted SCD. Thirty-nine patients with BrS were retrospectively analyzed and 12 patients (30.8%) were SCD-BrS. After AJT, an increase in both RVOT-AT [105.9 milliseconds versus 65.8 milliseconds, P<0.001] and RVOT-EPI recovery time [403.4 milliseconds versus 365.7 milliseconds, P<0.001] was observed. No changes occurred in RVOT-ARI [297.5 milliseconds versus 299.9 milliseconds, P=0.7]. Before AJT no differences were observed between SCD-BrS and non SCD-BrS in RVOT-AT, RVOT-EPI recovery time, and RVOT-ARI (P=0.9, P=0.91, P=0.86, respectively). Following AJT, SCD-BrS patients showed higher RVOT-AT, higher ∆RVOT-AT, lower RVOT-ARI, and lower ∆RVOT-ARI (P<0.001, P<0.001, P=0.007, P=0.002, respectively). At the univariate logistic regression, predictors of SCD-BrS were the following: RVOT-AT after AJT (specificity: 0.74, sensitivity 1.00, area under the curve 0.92); ∆RVOT-AT (specificity: 0.74, sensitivity 0.92, area under the curve 0.86); RVOT-ARI after AJT (specificity 0.96, sensitivity 0.58, area under the curve 0.79), and ∆RVOT-ARI (specificity 0.85, sensitivity 0.67, area under the curve 0.76). Conclusions Noninvasive electrocardiographic imaging can be useful in evaluating the results of AJT in BrS.

High-density epicardial mapping in Brugada syndrome: Depolarization and repolarization abnormalities.

BACKGROUND: The pathogenesis of Brugada syndrome (BrS) and consequently of abnormal electrograms (aEGMs) found in the epicardium of the right ventricular outflow tract (RVOT-EPI) is controversial. OBJECTIVE: The purpose of this study was to analyze aEGM from high-density RVOT-EPI electroanatomic mapping (EAM). METHODS: All patients undergoing RVOT-EPI EAM with the HD-Grid catheter for BrS were retrospectively included. Maps were acquired before and after ajmaline, and all patients had concomitant noninvasive electrocardiographic imaging with annotation of RVOT-EPI latest activation time (RVOTat). High-frequency potentials (HFPs) were defined as ventricular potentials occurring during or after the far-field ventricular EGM showing a local activation time (HFPat). Low-frequency potentials (LFPs) were defined as aEGMs occurring after near-field ventricular activation showing fractionation or delayed components. Their activation time from surface ECG was defined as LFPat. RESULTS: Fifteen consecutive patients were included in the study. At EAM before ajmaline, 7 patients (46.7%) showed LFPs. All patients showed HFPs before and after ajmaline and LFPs after ajmaline. Mean HFPat (134.4 vs 65.3 ms, P <.001), mean LFPat (224.6 vs 113.6 ms, P <.001), and mean RVOTat (124.8 vs 55.9 ms, P <.001) increased after ajmaline. RVOTat correlated with HFPat before (ρ = 0.76) and after ajmaline (ρ = 0.82), while RVOTat was shorter than LFPat before (P <.001) and after ajmaline (P <.001). BrS patients with history of aborted sudden cardiac death had longer aEGMs after ajmaline. CONCLUSION: Two different types of aEGMs are described from BrS high-density epicardial mapping. This might correlate with depolarization and repolarization abnormalities.

Procedural Safety and Efficacy for Pulmonary Vein Isolation with the Novel Polarx™ Cryoablation System: A Propensity Score Matched Comparison with the Arctic Front™ Cryoballoon in the Setting of Paroxysmal Atrial Fibrillation.

BACKGROUND: The novel Polarx™ cryoablation system is currently being studied for atrial fibrillation (AF) ablation. To the best of our knowledge, no study comparing the novel cryoablation system with the standard Arctic Front™ cryoballoon is available in today's literature. This study aims to compare Polarx™ and Arctic Front™ cryoballoon in terms of safety and efficacy. METHODS: From a total cohort of 202 patients who underwent pulmonary vein (PV) isolation for paroxysmal AF through cryoablation, a population of 30 patients who used Polarx™ were compared with 30 propensity-score matched patients who used Arctic Front™. RESULTS: Pulmonary vein occlusion and electrical isolation were achieved in all (100%) veins with a mean number of 1.09 ± 0.3 occlusion per vein using Polarx™ and 1.19 ± 0.5 occlusion per vein using Arctic Front™ (p = 0.6). Shorter procedure and fluoroscopy time were observed with Polarx™ group (60.5 ± 14.23 vs 73.43 ± 13.26 mins, p = 0.001; 12.83 ± 6.03 vs 17.23 ± 7.17 mins, p = 0.01, respectively). Lower cumulative freeze duration per vein was also observed with Polarx™ (203.38 ± 72.03 vs 224.9 ± 79.35 mins, p = 0.02). There was no significant difference in isolation time between the two groups (34.47 ± 21.23 vs 34.18 ± 26.79 secs, p = 0.9). CONCLUSIONS: The novel Polarx™ cryoablation system showed similar efficacy in vein occlusion and isolation and safety profile when compared to Arctic Front™ cryoablation system. Procedure time, fluoroscopy time, and cumulative freeze duration were significantly lower with Polarx™ cryoablation system.

High vagal tone predicts pulmonary vein reconnection after cryoballoon ablation for paroxysmal atrial fibrillation.

BACKGROUND: Pulmonary vein (PV) isolation is an established treatment for paroxysmal drug-refractory atrial fibrillation (AF). High parasympathetic tone and reconnection of PVs have demonstrated to be possible culprits of AF recurrence after ablation. Our aim was to investigate the association between parasympathetic tone and reconnected PVs in patients with paroxysmal AF. METHODS: Consecutive patients who underwent a redo catheter ablation procedure for atrial tachyarrhythmia recurrence by means of 3D electroanatomic mapping with documentation of presence or absence of PVs reconnection following an initial procedure of cryoballoon (CB) ablation for symptomatic drug-refractory paroxysmal AF were screened for the study. RESULTS: A total of 92 patients were included, of whom 50 (54.35%) were males. Reconnected PVs were found in 64 (69%) patients. PVs reconnection could be predicted by DC (C-statistic = .770), by SDNNI (C-statistic = .714) and by absolute VLF power (C-statistic = .722), while right-sided PVs reconnection could be better predicted by DC (C-statistic = .848) and by SDNNI (C-statistic = .761). In multivariate binary logistic regression analysis, a DC value ≥6.45 ms and an absolute VLF power value ≥160 ms2 were associated with three times and five times higher odds of PVs reconnection, respectively. On a vein-per-vein analysis, absolute VLF power ≥160 ms2 was associated with three times higher odds, while reaching of -40°C within 60 s was associated with three times lower odds of PVs reconnection. CONCLUSION: High parasympathetic tonus accurately predicts PVs reconnection. On a vein-per-vein analysis, parasympathetic markers along with biophysical parameters predicted PVs reconnection. On a case-by-case analysis, parasympathetic markers were the only predictors of PVs reconnection, thus being a robust PVs reconnection prediction tool.

Repeat procedures for recurrent persistent atrial fibrillation: A propensity-matched score comparison between left atrial linear ablation with radiofrequency and posterior wall isolation with the cryoballoon.

AIMS: To evaluate the clinical outcome in patients undergoing repeat procedures for recurrent persistent atrial fibrillation following an index cryoballoon (CB-A) pulmonary vein isolation ablation on a mid-term follow-up of 12 months. METHODS: In this propensity score-matched comparison, 50 patients undergoing left atrial posterior wall isolation (LAPWI) with the CB-A were matched to 50 patients treated with additional linear ablation using radiofrequency catheter ablation (RFCA). RESULTS: Meantime to repeat the procedure was 9.74 ± 4.36 months. At 12 months follow-up freedom from atrial tachyarrhythmias (ATas) was achieved in 82% of patients in the LAPWI group and in 62% of patients in the linear ablation group (P = .03). Regression analysis demonstrated that relapses during the blanking period and LA dimensions were independent predictors of ATas recurrences following the repeat procedure. CONCLUSION: LAPWI using CB-A is associated with a significantly higher freedom from atrial arrhythmias when compared with the RFCA mediated left atrial linear lesions on a mid-term follow-up of 12 months in patients with persAF undergoing a redo procedure.

Comparison between superior vena cava ablation in addition to pulmonary vein isolation and standard pulmonary vein isolation in patients with paroxysmal atrial fibrillation with the cryoballoon technique.

BACKGROUND: Paroxysmal atrial fibrillation (PAF) can be triggered by non-pulmonary vein foci, like the superior vena cava (SVC). The latter is correlated with improved result in terms of freedom from atrial tachycardias (ATs), when electrical isolation of this vessel utilizing radiofrequency energy (RF) is achieved. OBJECTIVES: Evaluate the clinical impact, in patients with PAF, of the SVC isolation (SVCi) in addition to ordinary pulmonary vein isolation (PVI) by means of the second-generation cryoballoon (CB) METHODS: A total of 100 consecutive patients that underwent CB ablation for PAF were retrospectively selected. Fifty consecutive patients received PVI followed by SVCi by CB application, and the following 50 consecutive patients received standard PVI. All patients were followed 12 months. RESULTS: The mean time to SVCi was 36.7 ± 29.0 s and temperature at SVC isolation was - 35 (- 18 to - 40) °C. Real-time recording (RTR) during SVCi was observed in 42 (84.0%) patients. At the end of 12 months of follow-up, freedom from ATs was achieved in 36 (72%) patients in the PVI only group and in 45 (90%) patients of the SVC and PV isolation group (Fisher's exact test p = 0.039, binary logistic regression: p = 0.027, OR = 0.28, 95%CI = 0.09-0.86). In survival analysis, SVC and PV isolation group was also associated with improved freedom from ATs (log-rank test: p = 0.017, Cox regression: p = 0.026, HR = 0.31, 95%CI = 0.11-0.87). CONCLUSION: Superior vena cava isolation with the CB in addition to PVI might improve freedom from ATs if compared to PVI alone at 1-year follow-up.

Substrate mapping of the left atrium in persistent atrial fibrillation: spatial correlation of localized complex conduction patterns in global charge-density maps to low-voltage areas in 3D contact bipolar voltage maps.

PURPOSE: This study aimed to investigate the spatial relationship between low-voltage areas (LVAs) in bipolar voltage mapping (BVM) and localized complex conduction (LCC)-cores in a global, non-contact, charge-density-based imaging, and mapping system (AcM). METHODS: Patients with history of index PVI for PsAF and scheduled for a repeat ablation procedure for recurrence of the same arrhythmia were enrolled between August 2018 and February 2020. All patients underwent both substrate mappings of the left atrium (LA) with the CARTO 3D map-ping system and with AcM. RESULTS: Ten patients where included in our analysis. All presented with persistency of PVI in all veins at the moment of repeat procedure. There was no linear relationship in BVM maps between SR and CSd (correlation coefficient 0.31 ± 0.15), SR and CSp (0.36 ± 0.12) and CSd and CSp (0.43 ± 0.10). The % overlap of localized irregular activation (LIA), localized rotational activation (LRA) and Focal (F) regions with LVA was lower at 0.2 mV compared to 0.5 mV (4.97 ± 7.39%, 3.27 ± 5.25%, 1.09 ± 1.92% and 12.59 ± 11.81%, 7.8 ± 9.20%, 4.62 ± 5.27%). Sensitivity and specificity are not significantly different when comparing composite maps with different LVA cut-offs. AURC was 0.46, 0.48, and 0.39 for LIA, LRA, and Focal, respectively. CONCLUSION: Due to wave front direction dependency, LVAs mapped with BVM in sinus rhythm and during coronary sinus pacing only partially overlap in patients with PsAF. LCC-cores mapped during PsAF partially co-localize with LVAs.

Novel noncontact charge density map in the setting of post-atrial fibrillation atrial tachycardias: first experience with the Acutus SuperMap Algorithm.

PURPOSE: The purpose of this study was to evaluate the safety and feasibility of the new high-resolution mapping algorithm SuperMap (Acutus Medical, CA, USA) in identifying and guiding ablation in the setting of regular atrial tachycardias following index atrial fibrillation (AF) ablation. METHODS: Seven consecutive patients who underwent a radiofrequency catheter ablation guided by the novel noncontact charge density (CD) SuperMap for atrial tachycardia were prospectively enrolled in our study. RESULTS: Arrhythmogenic substrate was identified in all seven patients. Mean number of EGM per map was 5859.7 ± 4348.5 points. Three patients (43%) exhibited focal tachycardia mechanisms in the left atrium, alternating from anteroseptal right superior pulmonary vein (RSPV), posterior in proximity of left inferior pulmonary vein (LIPV), and interarial septum in proximity of fossa ovalis, respectively. Four patients exhibited macroreentrant mechanism. In 3 of these patients, SuperMap detected mitral isthmus-dependent flutters with tachycardia cycle lengths of 240, 270 and 420 ms, respectively. In one patient, the mechanism was a macroreentrant tachycardia with the critical isthmus located between the crista terminalis and atriotomy. The mean ablation time (min) was 18.2 ± 12.5 and the mean procedural duration time was 56.4 ± 12.1 min. No minor or major complications occurred. CONCLUSION: The novel high-resolution mapping algorithm SuperMap proved to be safe, fast, and feasible in identifying and guiding ablation in the setting of regular atrial tachycardias following index AF ablation.

Systolic Anterior Motion of Mitral Valve Subchordal Apparatus: A Rare Echocardiographic Pattern in Non-Obstructive Hypertrophic Cardiomyopathy.

Systolic anterior motion (SAM) of the mitral valve or chordate is one characteristic seen in hypertrophic cardiomyopathy (HCM) either in obstructive or non-obstructive phenotypes. More often than not, the obstruction is caused by valvular rather than chordal SAM. We describe the role of echocardiography in identifying the actual anatomical location of the mitral valve apparatus involved in SAM and in assessing consequent left ventricular outflow tract (LVOT) obstruction in an otherwise asymptomatic patient. We report a case of a 29-year-old male admitted for an elective non-cardiac surgery, presenting with a cardiac murmur and left axis deviation with biventricular hypertrophy on electrocardiogram. On 2D transthoracic echocardiography (TTE), an asymmetrically hypertrophied left ventricle with systolic motion of anterior mitral valve was incidentally seen. Continuous wave Doppler assessment across the LVOT showed some gradient of obstruction (peak gradient: 9 mm Hg). Transesophageal echocardiography (TEE) demonstrated a redundant anterior mitral valve with the subchordal apparatus mainly causing SAM and confirmed the gradient obtained on TTE, with a mild degree, yet non-significant, degree of LVOT obstruction (mean gradient: 10 mm Hg) documented. Because of this finding, patient was cleared for surgery. Management was deemed conservative with emphasis on close surveillance for signs and symptoms attributable to development of significant LVOT obstruction in patients with HCM. To our knowledge, this is the first reported case in our country of an echocardiographic pattern of systolic anterior motion primarily of the subchordal mitral valve apparatus causing some, though non-significant, degree of LVOT obstruction in HCM. Echocardiographic features such as asymmetric left ventricular hypertrophy and presence of some LVOT obstruction caused primarily by subchordal apparatus could impact management in asymptomatic patients.

Weathering an adenosine insensitive right ventricular outflow tract ventricular tachycardia (Ado-insensitive RVOT VT) storm in an adolescent female: A case report

INTRODUCTION: Ventricular tachycardias (VT) are commonly associated with structural heart disease. However, 10% of VTs have no identifiable cause. Right ventricular outflow tract ventricular tachycardia (RVOT VT), a small subgroup of idiopathic VTs localized in the right ventricular outflow tract is highly sensitive to adenosine (ADO). Only 11% of RVOT VT is ADO-insensitive, posing a diagnostic challenge. We present a peculiar case of an ADO-insensitive RVOT-VT storm and the challenges of recognizing and managing it in a resource-limited setting. CASE SUMMARY: A 15-year-old female, asthmatic, complained of palpitations, lightheadedness, chest pain and dyspnea a few hours prior to admission. She had a similar episode a month ago, which necessitated ER admission, electrical cardioversion and amiodarone. On admission, she was tachycardic but normotensive. She had diffuse wheezes. Cardiac exam was normal. ECG revealed a wide complex tachycardia (WCT). Work-up revealed a normal chest x-ray, thyroid function tests and electrolytes. Echocardiogram showed a structurally normal heart. She was managed as a case of viral myocarditis and SVT with aberrancy. Vagal maneuvers and adenosine was given which slowed down the tachycardia. She was then started on IV anti-arrhythmics however, sustained symptomatic VT recurred on the same day. ECG analysis showed a WCT, LBBB, AV dissociation with positive QRS complexes in inferior leads suggestive of VT originating from the RVOT. RVOT VT storm was considered and adenosine (maximum dose) was given. The patient did not revert to sinus, hence, ADO-insensitive RVOT VT was considered. Cardioversion terminated the VT storm. On electrophysiology study, the VT was induced/ localized at the RVOT via 3D mapping. Ablation of the RVOT focus was performed, immediately terminating the VT. Post ablation, the patient was asymptomatic and was discharged improved with excellent prognosis. DISCUSSION: This case report highlights two things. The ECG remains a reliable tool in recognizing and localizing VTs clinically. Secondly, it highlights the importance of prompt recognition of ADO-insensitive RVOT VT because its management and prognosis is very different from the common causes of VT.