scholarly journals Successful ablation of bifocal PVCs from both left bundle branches triggering polymorphic VT in LQTS 2—A case report

Author(s):  
M Martinek ◽  
G Kollias ◽  
M Derndorfer ◽  
H Pürerfellner

Abstract Background A 19-year-old woman with an established diagnosis of long QT syndrome (LQTS) 2 and underlying KCNH2-mutation was referred to our centre for recurrent polymorphic ventricular tachycardia (VT) and ventricular fibrillation (VF) refractory to medical therapy and bilateral thoracic sympathectomy. Case summary Holter monitoring revealed a relevant PVC burden of two different morphologies. One PVC was originating from the left anterior fascicle, the other from the left posterior fascicle. Radiofrequency ablation resulted in complete suppression of both spontaneous PVC morphologies with a favourable clinical course over the next 2 years. Discussion This case presents two interesting insights: Firstly, the consistent bigeminal pattern of the torsade de pointes triggering PVC. These were retrieved from the device interrogation and correlated with the pattern that was seen at the time of the procedure. Secondly, PVC morphologies suggested an origin from both the left ventricular (posterior and anterior) fascicles, which has not been described so far. This was confirmed by the preceding Purkinje potentials seen at the successful ablation sites in sinus rhythm and during PVC. Ablation of triggering PVCs causing recurrent VT/VF in LQT 2 syndrome is feasible and effective over a mid-term Follow-up.

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
S Briongos Figuero ◽  
A Estevez ◽  
M.L Perez ◽  
J.B Martinez-Ferrer ◽  
L Alvarez ◽  
...  

Abstract Introduction Adaptive cardiac resynchronization therapy (aCRT) algorithm provides ambulatory CRT optimization and synchronized left-ventricular pacing instead of conventional biventricular pacing. Purpose To analyze the impact of aCRT on the risk of life-threatening ventricular arrythmia (VA) in patients with concomitant defibrillator therapy. Methods Symptomatic HF patients, in sinus rhythm, with LVEF ≤35% and QRS complex ≥130 ms undergoing first CRT-defibrillator (CRT-D) implant were collected from the multicentre, prospective and nationwide UMBRELLA study (2012–2017). All device information was automatically stored and collected through the remote monitoring system. An experts committee analysed in a blinded manner all electrograms. The endpoint was first appropriate therapy (AT) delivered within ventricular fibrillation zone at 12-months follow-up. Results 206 patients were collected (66.1±8.7 years; 73.3% male). Fifty nine patients composed the aCRT group and 147 composed the non-aCRT group. At implant, LBBB was present in 93% of patients, functional class III or IV in 69.9%, non-ischemic HF in 63.1% of patients and mean LVEF was 26.5±5.6%. Optimal medical treatment was achieved in a majority (B-blockers in 92%; RAASi in 89% and MRA in 72.2%). The percentage of ventricular pacing through 12 months was 96.1±9.4% in non-aCRT patients and 97.5±2.7% in aCRT patients (p=0.261). At 1-year of follow-up, 16 patients were delivered an AT (event rate: 7.8%). Most of these episodes (n=14) were due to sustained monomorphic ventricular tachycardia while the rest were caused by sustained polymorphic ventricular tachycardia/VF. A lower incidence of AT was observed in aCRT patients (3.4%) compared to non-aCRT patients (9.5%) but with no statistical differences (OR=0.33, CI 0.07–1.51, p=0.155). Conclusions In patients undergoing CRT provided by aCRT algorithm the risk of malign VA after 1-year of follow-up was low. Randomized studies are needed to clarify the impact of this dynamic algorithm on the arrhythmic risk of HF patients. Funding Acknowledgement Type of funding source: None


2020 ◽  
Vol 4 (5) ◽  
pp. 1-4
Author(s):  
Rui Files Flores ◽  
Fernando Mané ◽  
Nuno Antunes ◽  
Vítor Hugo Pereira

Abstract Background Hypertrophic cardiomyopathy (HCM) is a genetically determined myocardial disease that constitutes the main cause of sudden cardiac death (SCD) in young athletes. Apical HCM (ApHCM) represents a complex subset of patients, whose risk of SCD seems not negligible. Most applied scores likely underestimate the risk of heart events in this subset of patients. Case summary We report the case of a 55-year-old man who was admitted in the emergency department after an episode of aborted sudden death due to ventricular fibrillation. The electrocardiogram made at admission was noted for atrial fibrillation and a new-onset left bundle branch block. Emergency coronary angiography was normal. The electrocardiogram was repeated and showed symmetrical and profound inversion of T waves in the lateral leads. Transthoracic echocardiogram and cardiac magnetic resonance revealed left ventricular apical hypertrophy suggestive of apical variant of HCM. A cardiac defibrillator was implanted for secondary prevention of SCD. After 6 months of follow-up no further rhythmic events were noted. Discussion Although low, the risk of SCD of ApHCM patients is not negligible. This case illustrates the need for searching of new predictors of rhythmic risk in patients with ApHCM.


2019 ◽  
Vol 3 (2) ◽  
Author(s):  
Junji Yamaguchi ◽  
Yasutoshi Nagata ◽  
Yasuteru Yamauchi ◽  
Kenzo Hirao

Abstract Background Verapamil-sensitive fascicular ventricular tachycardia (VT) is the most common type of idiopathic left ventricular tachycardia, and it is divided into three types. Upper septal ventricular tachycardia (US-VT) is likely in patients with prior episodes of left posterior fascicular (LPF)-VT ablation, however, little is known about the recurrence mechanism of US-VT. Case summary A 53-year-old man had an US-VT after two catheter ablation sessions for a common idiopathic LPF-VT. The US-VT was successfully treated by ablating the proximal site of the LPF without making any further branch or fascicular block. This successful ablation point corresponded completely with the earliest pre-systolic potential (P2) site of the LPF-VT during the 1st session of catheter ablation. Discussion An US-VT recurrence could occur if a critical slow conduction is not affected by the catheter ablation. This recurrence might be the result of changing the re-entrant circuit after damage to the LPF. In order to eliminate the LPF-VT and prevent an US-VT recurrence, the earliest P2 site should be investigated carefully and ablated sufficiently.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Faisal F Syed ◽  
Peter Noseworthy ◽  
Christopher McLeod ◽  
Suraj Kapa ◽  
Siva Mulpuru ◽  
...  

Introduction: Although the vast majority of mitral valve prolapse (MVP) is benign, women with bileaflet MVP (biMVP), complex ventricular ectopy (VE), and abnormal T waves may comprise the recently described malignant biMVP syndrome. The mechanism of ventricular arrhythmia is unknown. To further characterize the arrhythmic substrate, we reviewed our center’s ablation experience in 6 biMVP patients with prior cardiac arrest and recurrent ICD shocks for drug refractory ventricular fibrillation (VF). Methods and Results: Six women with biMVP (median age 31.5 [range 24.2 - 58.7] years, EF 65 [45 - 67]%, all ≤moderate mitral regurgitation) experienced 6 (3 - 25) appropriate ICD shocks over 4.8 (2.8 - 10.7) years and underwent index ablation between 2/2007 - 10/2013. All had multiple VE morphologies (median 7 [3 - 24]) with variable coupling intervals but with a predominant VE trigger for the VF. A median 2 (1 - 4) VE foci were ablated. Sites of successful ablation of VF-triggering and other dominant VE were left ventricular papillary muscles [PM] (1 anterior, 1 posterior, 1 both), fascicles (1 anterior, 1 posterior), or both (1 both PM and posterior fascicle). Outflow tract VE was also present and targeted (1 left, 1 right)i. Two underwent repeat ablation (288 and 312 days) for recurrent complex VE without shocks, with different foci to the index ablation (1 posterior fascicle, 1 both fascicles). The VF-triggering VE in all patients was confirmed as originating from within the left fascicular system, which in 3/6 was at a papillary muscle. Acute procedural success was seen in all with no complications to date. A VF storm occurred within 24 hours of ablation in a single patient. At follow-up of a mean 662 (47 - 2099) days, 1 patient received a single shock (p=0.03 vs. preablation). Symptomatic VE was reduced in all; while 3/6 continue Class 1c antiarrhythmics and 5/6 have beta blockade. Conclusion: Malignant biMVP syndrome is characterized by fascicular and papillary muscle PVCs that trigger ventricular fibrillation, yet in all patients, the VE is multifocal. Ablation of at least one focus appears to improve symptoms and reduce shocks.


Open Heart ◽  
2019 ◽  
Vol 6 (1) ◽  
pp. e000990 ◽  
Author(s):  
Jan Berg ◽  
Marina Lovrinovic ◽  
Nora Baltensperger ◽  
Christine K Kissel ◽  
Jan Kottwitz ◽  
...  

ObjectiveClinical data on the effect of non-steroidal anti-inflammatory drugs (NSAIDs) in myopericarditis are limited. Since NSAIDs are standard therapy in pericarditis, we retrospectively investigated their safety in myopericarditis.MethodsIn a retrospective case-control study, we identified 60 patients with myopericarditis from September 2010 to August 2017. Diagnosis was based on clinical criteria, elevated high-sensitivity troponin T and cardiac magnetic resonance imaging (CMR). All patients received standard heart failure therapy if indicated. Twenty-nine patients (62%) received NSAIDs (acetylsalicylic acid: n=7, average daily dose =1300 mg or ibuprofen: n=22, average daily dose =1500 mg) for an average duration of 4 weeks. To create two cohorts with similar baseline conditions, 15 patients were excluded. Three months after diagnosis, 29 patients were re-evaluated by CMR to measure late gadolinium enhancement (LGE).ResultsBaseline characteristics of those treated with or without NSAIDs were similar. Mean age was 34 (±13) years, 6 (13%) were women. Mean left ventricular ejection fraction (LVEF) was 56% (±5). 82 % of the patients (14 of 17) treated with NSAIDs experienced a decrease in LGE at 3 months, while it was only 58 % (7 of 12) of those without NSAIDs (p=0.15). At 12-month follow-up, one of the patients treated without NSAIDs experienced polymorphic ventricular tachycardia (VT) with cardiac arrest, while one of the patients with NSAIDs experienced non-sustained VT.ConclusionsThis is the first case-control study demonstrating that NSAIDs are safe in patients with myopericarditis and preserved LVEF. Our data suggest that this drug class should be tested prospectively in a large randomised clinical trial.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Riccardo Morgagni ◽  
Giovanni Battista Forleo ◽  
Francesco Romeo

Introduction: Left Ventricular Non Compaction (LVNC) is an unclassified cardiomyopathy characterized by left ventricular hypertrabeculation and deep intertrabecular recesses in communication with the ventricular cavity. LVNC is often associated with other cardiomyopathies or with genetical disorders, and is familial at least in 25% of cases. The diagnosis is based on echocardiographic criteria (Chin et al. 1990; Jenni et al.2001; Stollberger et al. 1987-2002). Hypothesis: The aim of this study was to assess the value of echocardiographic parameters in predicting the occurrence of arrhythmic events in patients with familial LVNC. Methods: we studied 26 subjects, aged 39.9 ± 16.2 years, 11 males and 15 females, at a single institution, representing 7 families with LVNC. None had concomitant hypertension, diabetes mellitus or other significant cardiovascular disorder. Patients were evaluated at 3-month interval either clinically or with 12-Lead ECG, echocardiography and 24-hour Holter monitoring. The average duration of follow up was 36 ± 9 months. Echocardiographic parameters, ejection fraction (EF) and end-diastolic diameter (EDD), were matched with ventricular arrhythmias (VA) to assess their predictive value. The Kaplan-Meier method was used to calculate the probability of ventricular events. Results: Left Ventricular (LV) systolic function was depressed in 10 of 26 patients (38.5%), with a mean EF of 44 ± 3.2% at the first visit. Nine of 26 patients (34.6%) had LV dilatation (EDD ≥ 60 mm): among them only 5 patients (55.5%) had EF < 45%. Six of 26 patients (23.1%) underwent episodes of ventricular tachycardia (VT) during follow up. All 6 patients (100%) had LV dilatation, among them only 3 patients (50%) had depressed EF. By Chi square test and Kaplan Meier analysis, the only echocardiographic predictor of VA was LV dilatation (p < 0.001). Conclusions: In families with isolated LVNC, LV dilatation was the only echocardiographic predictor associated with subsequent development of ventricular arrhythmias. This finding might be helpful to optimally target preventive therapies in patients with familial LVNC.


EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
L Vitez ◽  
B Krajacic ◽  
V Starc ◽  
M Bunc

Abstract Introduction Transcatheter aortic valve replacement (TAVI) is a novel treatment method for sever aortic stenosis that has a positive impact on reverse left ventricular (LV) remodeling. The morphology and function of the LV can be assessed by transthoracic echocardiography (TTE) and high resolution electrocardiography (HR-ECG).  Purpose   The purpose of our study was to investigate whether morphological and functional myocardial changes after TAVI seen on TTE can be tracked by advanced ECG parameters using HR-ECG. Methods Consecutive TAVI patients with transfemoral approach were included in this pilot prospective study. A 5 minute HR-ECG and TTE were performed before and one year after TAVI. Spatial QRS vector amplitude (3D_QRSm), total 12-lead QRS amplitude (12leadV), spatial QRS-T angle (QRST_aM) and spatial ventricular gradient (SVG) were measured using HR-ECG and interventricular septum diastolic diameter (IVSd), left posterior wall diameter (LVPWd), left ventricular ejection fraction (LVEF) and tissue Doppler peak septal systolic velocity (Sm) were obtained by TTE. Student"s test was used for pairs to analyze the differences in sets of variables before and after TAVI and Pearson correlation coefficient to analyze the relationship between TTE and HR-ECG parameters.  Results 15 patients (5 male; 33.3%) were included in the study. At one year follow-up we found statistically significant reduction in LV wall thickness and increase in LVEF (61.6 ± 6.50 % vs. 66.9 ± 7.84 %, p = 0.043). In the subgroup with narrow QRS (8 patients), we found a statistically significant correlation between change of left posterior wall thickness (deltaLVPW) and spatial QRS vector amplitude (delta3D_QRSm) (r = 0.795; p = 0.018), and between change of left ventricular ejection fraction (deltaLVEF) and spatial QRS-T angle (deltaQRST_aM) (r = 0.604; p = 0.038). No significant correlations were found in the subgroup of patients with wide QRS complex.  Conclusions Our pilot study showed that morphological and functional myocardial changes after TAVI in patients with narrow QRS complex can be followed with advanced ECG parameters using HR-ECG. HR-ECG could prove to be a simple and inexpensive method of follow-up after TAVI in patients with narrow QRS. To ascertain the usefulness and reliability of HR-ECG, a study on a larger number of patients would be required.


2018 ◽  
Vol 1 (46) ◽  
pp. 28-30
Author(s):  
Agnieszka Wojdyła-Hordyńska ◽  
Grzegorz Hordyński ◽  
Patrycja Pruszkowska-Skrzep ◽  
Oskar Kowalski

Implantable cardioverter-defibrillator (ICD) is an indicated therapy for Long QT (LQT) patients after syncope or cardiac arrest survival. Premature ventricular contractions (PVCs) triggers ablation is also a known, yet, still rare, method aiming at ventricular tachycardia or torsade de pointes treatment in LQT syndrome. We present a 21-year-old female with no previous medical history admitted after syncope during cycling. Family history revealed sudden cardiac death in a 36-year-old father of the patient. Genetic test presented a puntiform mutation of KCNH2 gene. Beta-blocker and life vest therapy were introduced. Three months later the patient was admitted due to presyncopy and documented polymorphic ventricular tachycardia initiated by premature ventricular contractions. The clinical PVC triggers ablation was performed and cardioverter-defibrillator implanted. The PVC in the left aortic cusp was successfully ablated, and an ICD was implanted to utilise an atrial pacing. During the next 12-months follow up no VTs were recorded.


2008 ◽  
Vol 7 ◽  
pp. 35-35
Author(s):  
I TATJERHERNANZ ◽  
J GONZALEZCOSTELLO ◽  
A TORRENSOSES ◽  
J MARISTANYDAUNERT ◽  
A ARIZASOLE ◽  
...  

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