scholarly journals Radiofrequency ablation of ventricular tachycardia in Anderson–Fabry disease: a case series

2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Mark T Mills ◽  
Thomas A Nelson ◽  
Nicholas F Kelland ◽  
Jonathan Sahu ◽  
Justin Lee ◽  
...  

Abstract Background Cardiac involvement in Anderson–Fabry disease (AFD) can lead to arrhythmia, including ventricular tachycardia (VT). The literature on radiofrequency ablation (RFA) for the treatment of VT in AFD disease is limited. Case summary We discuss RFA of drug-refractory VT electrical storm in three males with AFD. The first patient (53 years old) had extensive involvement of the inferolateral left ventricle (LV) demonstrated with cardiac magnetic resonance imaging (CMRI), with a left ventricular ejection fraction (LVEF) of 35%. Two VT ablation procedures were performed. At the first procedure, the inferobasal endocardial LV was ablated. Furthermore, VT prompted a second ablation, where epicardial and endocardial sites were ablated. The acute arrhythmia burden was controlled but he died 4 months later despite appropriate implantable cardioverter-defibrillator therapies for VT. The second patient (67 years old) had full-thickness inferolateral involvement demonstrated with CMRI and LVEF of 45%. RFA of several endocardial left ventricular sites was performed. Over a 3-year follow-up, only brief non-sustained VT was identified, but he subsequently died of cardiac failure. Our third patient (69 years old), had an LVEF of 35%. He had RFA of endocardial left ventricular apical disease, but died 3 weeks later of cardiac failure. Discussion RFA of drug-refractory VT in AFD is feasible using standard electrophysiological mapping and ablation techniques, although the added clinical benefit is of questionable value. VT storm in the context of AFD may be a marker of end-stage disease.

Author(s):  
Daniel Matos ◽  
Pedro Adragão ◽  
Cristiano Pisani ◽  
Vinicius Hatanaka ◽  
Pedro Freitas ◽  
...  

BACKGROUND Direct comparisons of combined (C-ABL) and non-combined (NC-ABL) endo-epicardial ventricular tachycardia (VT) ablation outcomes are scarce. We aimed to investigate the long-term clinical efficacy and safety of these 2 strategies in ischemic heart disease (IHD) and nonischemic cardiomyopathy (NICM) patients. METHODS Multicentric observational registry including 316 consecutive patients who underwent catheter ablation for drug-resistant VT between January 2008 and July 2019. Primary and secondary efficacy endpoints were defined as VT-free survival and all-cause death after ablation. Safety outcomes were defined by 30-days mortality and procedure-related complications. RESULTS Most of the patients were male (85%), with IHD (67%) and mean age of 63±13 years. During a mean follow-up of 3±2 years, 117 (37%) patients had VT recurrence and 73 (23%) died. Multivariate survival analysis identified electrical storm (ES) at presentation, IHD, left ventricular ejection fraction (LVEF), New York Heart Association (NYHA) class III/IV, and C-ABL as independent predictors of VT recurrence. In 135 patients undergoing repeated procedures, only C-ABL and ES were independent predictors of relapse. The independent predictors of mortality were C-ABL, ES, LVEF, age and NYHA class III/IV. C-ABL survival benefit was only seen in patients with a previous ablation (P for interaction=0.04). Mortality at 30-days was similar between NC-ABL and C-ABL (4% vs. 2%, respectively, P=0.777), as was complication rate (10.3% vs. 15.1% respectively, P=0.336). CONCLUSION A combined endo-epicardial approach was associated with greater VT-free survival and lower all-cause death in IHD and NICM patients undergoing repeated VT catheter ablations. Both strategies seem equally safe.


EP Europace ◽  
2020 ◽  
Author(s):  
Simone Savastano ◽  
Veronica Dusi ◽  
Enrico Baldi ◽  
Roberto Rordorf ◽  
Antonio Sanzo ◽  
...  

Abstract Aims The adoption of percutaneous stellate ganglion blockade for the treatment of drug-refractory electrical storm (ES) has been increasingly reported; however, the time of onset of the anti-arrhythmic effects, the safety of a purely anatomical approach in conscious patients and the additional benefit of repeated procedures remain unclear. Methods and results This study included consecutive patients undergoing percutaneous left stellate ganglion blockade (PLSGB) in our centre for drug-refractory ES. Lidocaine, bupivacaine, or a combination of both were injected in the vicinity of the left stellate ganglion. Overall, 18 PLSGBs were performed in 11 patients (age 69 ± 13 years; 63.6% men, left ventricular ejection fraction 31.6 ± 16%). Seven patients received only one PLSGB; three underwent two procedures and one required three PLSGB and two continuous infusions to control ventricular arrhythmias (VAs). All PLSGBs were performed with an anatomical approach; lidocaine, alone, or in combination was used in 77.7% of the procedures. The median burden of VAs 1 h after each block was zero compared with five in the hour before (P < 0.001); 83% of the patients were free from VAs; the efficacy at 24 h increased with repeated blocks. The anti-arrhythmic efficacy of PLSGB was not related to anisocoria. No procedure-related complications were reported. Conclusion Anatomical-based PLSGB is a safe and rapidly effective treatment for refractory ES; repeated blocks provide additional benefits. Percutaneous left stellate ganglion blockade should be considered for stabilizing patients to allow further ES management.


2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
R Spacek ◽  
O Jiravsky ◽  
M Hudec ◽  
J Fismol ◽  
L Sknouril

Abstract Introduction Electrical storm (ES) is an emergent condition which requires a sofisticated approach. Massive sympathetic surge almost always connected with ES precipitates recurrent ventricular arrhythmias. Performing stellate ganglion block (SGB) to alleviate the sympathetic activity on myocardium is becoming a standard of care in many centers. However, there is no clear data to predict in which patients the SGB will be ineffective. Purpose To identify predictors of SGB failure in patients with ES. Methods We analyzed our case series of SGB – the procedure was performed in 31 patients with ES in our center from March 2017 to December 2018. Results Mean left ventricular ejection fraction was 27% (±9%), 74% of patients had ischaemic cardiomyopathy. The most frequent type of arrhythmia was monomorphic ventricular tachycardia (VT), occurring in 71% of patients, followed by polymorphic VT in 13% of cases. After SGB, the burden of ventricular arrhythmias failed to decrease by at least 50% in 10% of cases - these patients were marked as non-responders. Slow monomorphic VT (under 160/min) was observed in all of these patients. On the other hand, fast monomorphic VT or polymorphic VT seemed to respond very well to SGB. We also observed, that patients with ES after acute coronary syndromes were good responders as well. The effect of SGB was not related to age, gender, EF LK or the etiology of cardiomyopathy. Conclusions According to our experience, the failure of SGB in the treatment of ES is not frequent. It typically occurs in patients with slow monomorphic VT. It is probable that such arrhythmias are sustained primarily due to the extensive myocardial substrate, and not because of the sympathetic surge. The situation is quite the opposite in patients with fast VT and acute ischemia.


2021 ◽  
Author(s):  
Julian Müller ◽  
Michael Behnes ◽  
Tobias Schupp ◽  
Dominik Ellguth ◽  
Gabriel Taton ◽  
...  

AbstractBoth acute myocardial infarction complicated by ventricular tachyarrhythmias (AMI–VTA) and electrical storm (ES) represent life-threatening clinical conditions. However, a direct comparison of both sub-groups regarding prognostic endpoints has never been investigated. All consecutive implantable cardioverter-defibrillator (ICD) recipients were included retrospectively from 2002 to 2016. Patients with ES apart from AMI (ES) were compared to patients with AMI accompanied by ventricular tachyarrhythmias (AMI–VTA). The primary endpoint was all-cause mortality at 3 years, secondary endpoints were in-hospital mortality, rehospitalization rates and major adverse cardiac event (MACE) at 3 years. A total of 198 consecutive ICD recipients were included (AMI–VTA: 56%; ST-segment elevation myocardial infarction (STEMI): 22%; non-ST-segment myocardial infarction (NSTEMI) 78%; ES: 44%). ES patients were older and had higher rates of severely reduced left ventricular ejection fraction (LVEF) < 35%. ES was associated with increased all-cause mortality at 3 years (37% vs. 19%; p = 0.001; hazard ratio [HR] = 2.242; 95% CI 2.291–3.894; p = 0.004) and with increased risk of first cardiac rehospitalization (44% vs. 12%; p = 0.001; HR = 4.694; 95% CI 2.498–8.823; p = 0.001). This worse prognosis of ES compared to AMI–VTA was still evident after multivariable adjustment (long-term all-cause mortality: HR = 2.504; 95% CI 1.093–5.739; p = 0.030; first cardiac rehospitalization: HR = 2.887; 95% CI 1.240–6.720; p = 0.014). In contrast, the rates of MACE (40% vs. 32%; p = 0.326) were comparable in both groups. At long-term follow-up of 3 years, ES was associated with higher rates of all-cause mortality and rehospitalization compared to patients with AMI–VTA.


EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
D Nascimento Matos ◽  
D Cavaco ◽  
P Carmo ◽  
MS Carvalho ◽  
G Rodrigues ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. INTRODUCTION Catheter ablation outcomes for drug-resistant ventricular tachycardia (VT) in nonischemic cardiomyopathy (NICM) are suboptimal when compared to ischemic cardiomyopathy. We aimed to analyse the long-term efficacy and safety of percutaneous catheter ablation in this subset of patients. METHODS Single-center observational retrospective registry including consecutive NICM patients who underwent catheter ablation for drug-resistant VT during a 10-year period. The efficacy endpoint was defined as VT-free survival after catheter ablation, while safety outcomes were defined by 30-days mortality and procedure-related complications. Independent predictors of VT recurrence were assessed by Cox regression. RESULTS In a population of 68 patients, most were male (85%), mean left ventricular ejection fraction (LVEF) was 34 ± 12%, and mean age was 58 ± 15 years. All patients had an implantable cardioverter-defibrillator. Twenty-six (38%) patients underwent epicardial ablation (table 1). Over a median follow-up of 3 years (IQR 1-8), 41% (n = 31) patients had VT recurrence and 28% died (n = 19). Multivariate survival analysis identified LVEF (HR= 0.98; 95%CI 0.92-0.99, p = 0.046) and VT storm at presentation (HR = 2.38; 95%CI 1.04-5.46, p = 0.041) as independent predictors of VT recurrence. The yearly rates of VT recurrence and overall mortality were 21%/year and 10%/year, respectively. No patients died at 30-days post-procedure, and mean hospital length of stay was 5 ± 6 days. The complication rate was 7% (n = 5, table 1), mostly in patients undergoing epicardial ablation (4 vs 1 in endocardial ablation, P = 0.046). CONCLUSION LVEF and VT storm at presentation were independent predictors of VT recurrence in NICM patients after catheter ablation. While clinical outcomes can be improved with further technical and scientific development, a tailored endocardial/epicardial approach was safe, with low overall number of complications and no 30-days mortality. Abstract Figure.


2017 ◽  
Vol 4 (3) ◽  
pp. 34
Author(s):  
William Wung ◽  
Alison G Chang ◽  
Thomas WR Smith

A 65-year-old male with a history of coronary artery disease and ankylosing spondylitis presented with focal ECG changes and elevated cardiac biomarkers suggestive of an acute lateral ST-elevation myocardial infarction. Emergent coronary angiography surprisingly showed non-obstructive coronary artery disease. Further workup including a cardiac MRI, viral serologies, and an endomyocardial biopsy was consistent with focal Coxsackie viral myocarditis. The patient subsequently developed recurrent, pulseless ventricular tachycardia requiring multiple rounds of ACLS, and his left ventricular ejection fraction acutely dropped from 55% to 20%. An emergent intra-aortic balloon pump was placed, and an intravenous lidocaine infusion and high-dose corticosteroids were started for the patient’s electrical storm and myocarditis, respectively. The patient was eventually discharged in stable condition with an implantable cardiac defibrillator. No further episodes of ventricular tachycardia were noted at six-month follow-up. In patients with acute ECG changes, elevated cardiac biomarkers, and no evidence of obstructive coronary artery disease, myocarditis should be considered as a leading diagnosis given the potentially life-threatening sequelae as seen in our patient.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Yoshikazu Yazaki ◽  
Mitsuaki Horigome ◽  
Kazunori Aizawa ◽  
Takeshi Tomita ◽  
Hiroki Kasai ◽  
...  

Background : We previously described severity of heart failure and ventricular tachycardia (VT) as independent predictors of mortality in patients with cardiac sarcoidosis (CS). Medical treatment for chronic heart failure has been established over the last few decades. Prophylactic use of implantable cardioverter defibrillator (ICD) and cardiac resynchronization therapy (CRT or CRT-D) have been introduced in patients with severe heart failure. We therefore hypothesized that the prognosis of CS improves due to such advances in the management of heart failure and VT. Methods : To confirm our hypothesis, we analyzed 43 CS patients diagnosed between 1988 and 2006 and treated with corticosteroids. We classified two sequential referral patients diagnosed between 1988 and 1997 (n=19) and between 1998 and 2006 (n=24), and compared treatment and prognosis between the two cohorts. Results : Left ventricular ejection fraction (LVEF) and dimensions were similar between the two cohorts. Although age in the 1988–1997 referral cohort was significantly younger than that in the 1998–2006 referral cohort (54±14years versus 62±10years, p<0.05), survival in the earlier cohort was significantly worse (log-rank=4.41, p<0.05). The 1- and 5-year mortality rates were 88% and 71% in the 1988–1997 referral cohort, and 96% and 92% in the 1998–2006 referral cohort, respectively. The 1998–2006 referral cohort showed significantly higher incidence of ICD or CRT-D implantation (29% versus 6%, p<0.05), β-blocker use (46% versus 6%, p<0.01) and addition of methotrexate (21% versus 0%, p<0.05), and increased maintenance dose (7.0±1.9mg/day versus 5.0±0.9mg/day, p<0.01) compared to the 1988–1997 referral cohort. Multivariate analysis including age, LVEF, and sustained ventricular tachycardia (sVT) identified diagnosis between 1988 and 1997 (hazard ratio [HR]: 19.8, p<0.01) and LVEF (HR: 0.83/1% increase, p<0.01) as independent predictors of mortality. Conclusions : Survival in the recent CS patients is significantly better than previously described. Recent advances in the device therapies and medical treatments including modified immunosuppression alter the clinical outcome in patients with CS.


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