Clinical outcomes and programming strategies of implantable cardioverter defibrillator (ICD) devices during childhood in hypertrophic cardiomyopathy: a UK national cohort study

Abstract Background Sudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM). ICDs have been shown to be effective at terminating malignant ventricular arrhythmias but at the expense of a high incidence of complications. The optimal device and programming strategies to reduce complications in this patient group are unknown. Purpose To describe the programming strategies and clinical outcomes of ICD implantation in childhood HCM. Methods Anonymised, non-invasive clinical data were collected from a retrospective, longitudinal multi-centre cohort of children (<16 years) with HCM (n=687) and an ICD in-situ from the United Kingdom. Results 96 patients (61 male (64%), 6 non-sarcomeric (6%)) underwent ICD implantation at a median age 14yr (IQR 11–16, range 3–16) and weight 52.3 kg (IQR 34.8–63.1). Indication for ICD was primary prevention in 72 (75%). 82 (85%) had an endovascular system, 3 (3%) epicardial and 11 (12%) subcutaneous system. 61 patients (74%) were receiving one or more cardioactive medications at implantation [B blockers n=66, 70%, disopyramide n=14, 15%, amiodarone n=7, 7%, calcium channel blocker n=7, 9%, other n=5, 6%]. Programming practices varied: all had VF therapies activated (median 220bpm, IQR 212–230); 70 (73%) had a VT zone programmed (median rate 187 bpm, SD 20.9), of which 26 (27%) had therapies activated. 50 patients (61%) had antitachycardia pacing (ATP) activated. Over a median follow up of 53.6 months (IQR 27.3,108.4), 4 patients (4.2%) died following arrhythmic events despite a functioning device. 25 patients had 53 appropriate therapies (ICD shock n=47, ATP n=8), incidence rate 5.22 (95% CI 3.5–7.8). On univariable analysis, secondary prevention indication for ICD implantation was the only predictor of therapy [16 (64%) vs 8 (11.3%), p value <0.001]. 8 (8.3%) patients had 9 inappropriate therapies (ICD shock n=4, ATP n=5), incidence rate 1.37 (95% CI 0.65–2.8), caused by T wave oversensing (n=2), lead migration (n=1), supraventricular tachycardia (n=1). Device complications were seen in 30 patients (31%), including lead complications (n=16) and infection (n=10). No clinical characteristics predicted time to inappropriate therapy or lead complication. Conclusions In a contemporary cohort of children with HCM, the incidence of inappropriate therapies is lower than previously reported, yet complication rates remain higher than reported in adult patients. No clinical, device or programming strategies were associated with inappropriate therapies or lead complications. Funding Acknowledgement Type of funding source: Other. Main funding source(s): British Heart Foundation

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Clinical outcomes and programming strategies of implantable cardioverter-defibrillator devices in paediatric hypertrophic cardiomyopathy: a UK National Cohort Study

EP Europace ◽

10.1093/europace/euaa307 ◽

2020 ◽

Author(s):

Gabrielle Norrish ◽

Henry Chubb ◽

Ella Field ◽

Karen McLeod ◽

Maria Ilina ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Implantable Cardioverter Defibrillator ◽

Incidence Rate ◽

Clinical Outcomes ◽

Cardioverter Defibrillator ◽

National Cohort ◽

Icd Shock ◽

The Uk ◽

Device Complications

Abstract Aims Sudden cardiac death (SCD) is the most common mode of death in paediatric hypertrophic cardiomyopathy (HCM). This study describes the implant and programming strategies with clinical outcomes following implantable cardioverter-defibrillator (ICD) insertion in a well-characterized national paediatric HCM cohort. Methods and results Data from 90 patients undergoing ICD insertion at a median age 13 (±3.5) for primary (n = 67, 74%) or secondary prevention (n = 23, 26%) were collected from a retrospective, longitudinal multi-centre cohort of children (<16 years) with HCM from the UK. Seventy-six (84%) had an endovascular system [14 (18%) dual coil], 3 (3%) epicardial, and 11 (12%) subcutaneous system. Defibrillation threshold (DFT) testing was performed at implant in 68 (76%). Inadequate DFT in four led to implant adjustment in three patients. Over a median follow-up of 54 months (interquartile range 28–111), 25 (28%) patients had 53 appropriate therapies [ICD shock n = 45, anti-tachycardia pacing (ATP) n = 8], incidence rate 4.7 per 100 patient years (95% CI 2.9–7.6). Eight inappropriate therapies occurred in 7 (8%) patients (ICD shock n = 4, ATP n = 4), incidence rate 1.1/100 patient years (95% CI 0.4–2.5). Three patients (3%) died following arrhythmic events, despite a functioning device. Other device complications were seen in 28 patients (31%), including lead-related complications (n = 15) and infection (n = 10). No clinical, device, or programming characteristics predicted time to inappropriate therapy or lead complication. Conclusion In a large national cohort of paediatric HCM patients with an ICD, device and programming strategies varied widely. No particular strategy was associated with inappropriate therapies, missed/delayed therapies, or lead complications.

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Risk of death without appropriate defibrillator shock in patients with advanced renal dysfunction

EP Europace ◽

10.1093/europace/euy305 ◽

2019 ◽

Vol 21 (3) ◽

pp. 459-464 ◽

Cited By ~ 3

Author(s):

Ido Goldenberg ◽

Tal Mor ◽

Eyal Nof ◽

Arwa Younis ◽

Anat Berkovitch ◽

...

Keyword(s):

High Risk ◽

Kidney Disease ◽

Shock Therapy ◽

P Value ◽

High Risk Population ◽

Icd Implantation ◽

Risk Population ◽

Risk Of Death ◽

Kaplan Meier ◽

Icd Shock

Abstract Aims Heart failure patients with advanced chronic kidney disease (CKD) may experience an increased rate of non-arrhythmic mortality due to associated comorbidities. We aimed to evaluate the risk of mortality without appropriate implantable cardioverter-defibrillator (ICD) shocks in this high-risk population. Methods and results The study population comprised 3542 patients who received an ICD, were enrolled, and prospectively followed-up in the Israeli ICD registry. Study patients were categorized into two groups: those with advanced CKD [defined by a glomerular filtration rate of <30 mL/min/1.73 m2 or being on dialysis at time of implantation (n = 197)], and those without advanced CKD (n = 3344). The primary endpoint was the risk of death without receiving appropriate ICD shock. Kaplan–Meier survival analysis showed that at 5 years of follow-up the rates of death without prior ICD shock were significantly higher in the advanced kidney disease group (46%) compared with the non-advanced CKD group (19%; log-rank P-value <0.001). Consistently, multivariate analysis showed that the risk of death without receiving appropriate ICD shock therapy at 5 years was 2.5-fold (P < 0.001) higher among advanced CKD patients. In contrast, the rate of appropriate ICD shock therapy at 5 years among advanced CKD patients was only 9%, with a very high mortality rate (63%) within 3.5 years subsequent to shock therapy. Conclusion Nearly one-half of ICD with advanced CKD die within 5 years without receiving an appropriate ICD shock. These findings stress the importance of appropriate patient selection for primary ICD implantation in this high-risk population.

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Association between self-care and prognosis in 1123 patients with chronic heart failure

European Heart Journal ◽

10.1093/ehjci/ehaa946.1041 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

E Calero ◽

E Hidalgo ◽

R Marin ◽

L Rosenfeld ◽

I Fernandez ◽

...

Keyword(s):

Heart Failure ◽

Chronic Heart Failure ◽

Acute Heart Failure ◽

Nyha Class ◽

Self Care ◽

P Value ◽

Funding Source ◽

Class Iii ◽

Cox Models ◽

Lower Tertile

Abstract Background Self-care is a crucial factor in the education of patients with heart failure (HF) and directly impacts in the progression of the disease. However, little is published about its major clinical implications as admission or mortality in patients with HF. Aims and methods The aim of the study was to analyze time to admission due to acute heart failure and mortality associated with poor self-care in patients with chronic HF. We prospectively recruited consecutive patients with stable chronic HF referred to a nurse-led HF programme. Selfcare was evaluated at baseline with the 9 item European Heart Failure Self-Care Behavior Scale. Scores were standardized and reversed from 0 (worst selfcare) to 100 (better self care). For the purpose of this study we analyzed the associations of worse self-care (defined as scores below the lower tertile of the scale) with demographic, disease-related (clinical) and psychosocial factors in all patients at baseline. Results We included 1123 patients, mean age 72±11, 639 (60%) were male, mean LVEF 45±17 and 454 (40,4%) were in NYHA class III or IV. Mean score of the 9-item ESCBE was 69±28. Score below 55 (lower tertile) defined impaired selfcare behaviour. Those patients with worse self-care had more ischaemic heart disease, more COPD, and they achieved less distance in the 6 minute walking test. Regarding psychosocial items patients in lower tertile of self-care needed a caregiver more frequently, they present more cognitive impairment, depressive symptoms and worse score in terms of health self-perception. Multivariate Cox Models showed that a score below 55 points in 9-item ESCBE was independently associated with higher readmission due to acute heart failure [HR 1.26 (1.02–1.57), p value=0.034] and with mortality [HR 1.24 CI95% (1.02–1.50), p value=0.028] Conclusion Poor self-care measured with the modified 9-item ESCBE was associated with higher risk of admission due to acute decompensation and higher risk of mortality in patients with chronic heart failure. These results highlight the importance of assessing self-care and provide measures to improve them. Funding Acknowledgement Type of funding source: Public hospital(s). Main funding source(s): Hospital Univesitario de Bellvitge

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Lumbar Disc Herniation and Preoperative Modic Changes: A Prospective Analysis of the Clinical Outcomes After Microdiscectomy

Global Spine Journal ◽

10.1177/2192568220976089 ◽

2021 ◽

pp. 219256822097608

Author(s):

Dinesh Kumarasamy ◽

Shanmuganathan Rajasekaran ◽

Sri Vijay Anand K. S ◽

Dilip Chand Raja Soundararajan ◽

Ajoy Prasad Shetty T ◽

...

Keyword(s):

Patient Satisfaction ◽

Back Pain ◽

Clinical Outcomes ◽

Lumbar Disc Herniation ◽

Disc Herniation ◽

Rating Scale ◽

Lumbar Disc ◽

Modic Changes ◽

P Value ◽

Functional Scores

Study design: Prospective comparative cohort study. Objectives: The study aims to elucidate the relationship between Modic endplate changes and clinical outcomes after a lumbar microdiscectomy. Methods: Consecutive patients undergoing microdiscectomy for lumbar disc herniation (LDH) were prospectively studied. Pre-operative clinical and radiological parameters were recorded. The pain was assessed by Numeric pain rating scale (NPRS), and functional assessment by Oswestry Disability Index (ODI). Minimal clinically important difference (MCID) in outcome was calculated for both the groups. Complications related to surgery were studied. Follow-up was done at 6 weeks, 3 months, 6 months and 1 year. Mac Nab criteria were used to assess patient satisfaction at 1 year. Results: Out of 309 patients, 86 had Modic changes, and 223 had no Modic changes. Both groups had similar back pain (p-value: 0.07) and functional scores (p-value: 0.85) pre-operatively. Postoperatively patients with Modic changes had poorer back pain and ODI scores in the third month, sixth month and 1 year (p-value: 0.001). However, MCID between the groups were not significant (p-value: 0.18 for back pain and 0.58 for ODI scores). Mac Nab criteria at 1 year were worse in Modic patients (p-value: 0.001). No difference was noted among Modic types in the pre-operative and postoperative pain and functional outcomes. Four patients in Modic group (4.7%) and one patient in the non-Modic group (0.5%) developed postoperative discitis (p-value: 0.009). Conclusions: Preoperative Modic changes in lumbar disc herniation is associated with less favorable back pain, functional scores and patient satisfaction in patients undergoing microdiscectomy.

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Projecting the long-term benefits of single pill combination therapy for patients with hypertension in five countries

European Heart Journal ◽

10.1093/ehjci/ehaa946.2781 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

C Borghi ◽

J.G Wang ◽

A.V Rodionov ◽

M Rosas ◽

I.S Sohn ◽

...

Keyword(s):

Combination Therapy ◽

South Korea ◽

Health Outcomes ◽

Clinical Outcomes ◽

Funding Source ◽

Combination Therapies ◽

Private Company ◽

Treatment Pathways ◽

Single Pill ◽

The Impact

Abstract Background It is well established that single pill combination (SPC) therapies have the potential to improve patient adherence versus multi-pill regimens, thereby improving blood pressure control and clinical outcomes in populations with hypertension. Purpose To develop a microsimulation model, capturing different treatment pathways, to project the impact on clinical outcomes of using single pill combination therapies for the management of hypertension in five countries (Italy, Russia, China, South Korea and Mexico). Methods The model was designed to project health outcomes between 2020 and 2030 for populations with hypertension managed according to four different treatment pathways: current treatment practices [CTP], single drug with dosage titration first then sequential addition of other agents [start low and go slow, SLGS], free choice combination with multiple pills [FCC] and combination therapy in the form of a single pill [SPC]. Model inputs were derived from Global Burden of Disease 2017 dataset, including demographics, health status/risk factors, transition probabilities and treatment attributes/healthcare utilization, and the model incorporated real-world challenges to healthcare delivery such as access to care, SBP measurement error, adherence and therapeutic inertia. Simulated outcomes of mortality, incidence of chronic kidney disease (CKD), stroke and ischemic heart disease (IHD), and disability-adjusted life years (DALYs) due to these conditions were estimated for population of 1,000,000 simulated patients for each treatment pathway and country. Results SPC therapy was projected to improve health outcomes over SLGS, FCC and CTP over 10 years in all five countries. SPC was forecast to reduce mortality by 5.4% (Italy), 4.9% (Russia), 4.5% (China), 2.3% (South Korea) and 3.6% (Mexico) versus CTP and showed greater projected reductions in mortality than SLGS and FCC. DALYs were projected to be reduced with SPC therapy by between 5.7% (Italy) and 2.2% (South Korea) compared with CTP and reductions in the incidence of clinical events were also projected with SPC therapy, with decreases in the range of 11.5% (Italy) to 4.9% (South Korea) versus CTP. Conclusions Ten-year projections of clinical outcomes associated with different anti-hypertensive treatment pathways in five countries indicated that both combination therapies (FCC and SPC) are likely to reduce the disease burden of hypertension compared with conventional management approaches, with SPC showing the greatest overall benefits due to improved adherence. Funding Acknowledgement Type of funding source: Private company. Main funding source(s): Sanofi, Gentilly, France

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Percutaneous coronary intervention in spontaneous coronary artery dissection: a case-control study

European Heart Journal ◽

10.1093/ehjci/ehaa946.1523 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

D Kotecha ◽

A.D.P.E Premawardhana ◽

M Garcia-Guimaraes ◽

D Pellegrini ◽

A.D Wood ◽

...

Keyword(s):

Coronary Artery ◽

Case Control Study ◽

Case Control ◽

Artery Dissection ◽

Funding Source ◽

Complication Rates ◽

Spontaneous Coronary Artery Dissection ◽

Coronary Artery Dissection ◽

Control Study ◽

Reduced Flow

Abstract Background Spontaneous Coronary Artery Dissection (SCAD) is an important cause of acute coronary syndrome particularly in young-middle aged women. Revascularisation is challenging due to an underlying disrupted and friable coronary vessel wall leading to widely reported worse outcomes than for atherosclerotic coronary disease. Therefore, a conservative approach where possible is favoured however in some cases haemodynamic instability, ongoing ischaemia and reduced distal flow mandates consideration of revascularisation. Purpose To compare SCAD survivors managed with PCI or conservatively in terms of presentation characteristics, complications and long-term outcomes. Methodology and results 226 angiographically confirmed SCAD survivors (95% female,47±9.7yrs) who underwent PCI were compared in a case control study with two hundred and twenty-five angiographically confirmed SCAD survivors (92% female, 49±9.9yrs) who were conservatively managed. Patients were recruited from UK, Spanish and Dutch SCAD registries and both groups were well matched in terms of baseline demographics. Those treated with PCI were more likely to present with proximal SCAD (30.8% vs 7.6% P<0.01) and ST elevation myocardial infarction (STEMI) or cardiac arrest with reduced flow (32.3% vs 6.3% P<0.01). Intervention was performed with stents in 72.4%, plain old balloon angioplasty in 21.1% and wiring in 6.4% of cases and more often for multi-segment disease (40.8% vs 26.3% P<0.01). In cases with initial reduced flow undergoing PCI an improvement in flow was seen in 83%. Analysis of UK PCI cases (n=144) reveal complications in 56 (38.8%). However, when assessed for significance defined by a reduction in flow in a proximal/mid vessel, stent extension into left main stem, iatrogenic dissection requiring PCI and CABG as a consequence of PCI only 13 cases (9%) met criteria. Iatrogenic dissection accounts for the majority (76.9%). SCAD lesion length was associated with presence of complications (P=0.025). There was a non-significant trend towards major adverse cardiovascular events (MACE) occurring more frequently in those undergoing PCI (18% vs 11% P=0.067) driven by revascularisation (5% vs 1% P=0.036). Median follow up was 2.7 years. Conclusions PCI in SCAD is often performed in higher risk patients; in those presenting with reduced flow, the majority demonstrate improvement. Importantly whilst overall complication rates were similar to those widely reported, clinically significant complications were low. Multivariate modelling will reveal factors associated with complications to aid future decision making in this challenging patient group. Funding Acknowledgement Type of funding source: Public grant(s) – National budget only. Main funding source(s): British Heart Foundation

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Uncemented versus cemented arthroplasty after metal-on-metal total hip replacement in patients with femoral neck fractures: a retrospective study

Journal of International Medical Research ◽

10.1177/03000605211012210 ◽

2021 ◽

Vol 49 (5) ◽

pp. 030006052110122

Author(s):

Wenlu Liu ◽

Huanyi Lin ◽

Xianshang Zeng ◽

Meiji Chen ◽

Weiwei Tang ◽

...

Keyword(s):

Femoral Neck ◽

Clinical Outcomes ◽

Total Hip Replacement ◽

Hip Replacement ◽

Femoral Neck Fractures ◽

Harris Hip Score ◽

Complication Rates ◽

Total Hip ◽

Metal On Metal

Objective To compare the clinical outcomes of primary metal-on-metal total hip replacement (MoM-TR) converted to uncemented total hip replacement (UTR) or cemented total hip replacement (CTR) in patients with femoral neck fractures (AO/OTA: 31B/C). Methods Patient data of 234 UTR or CTR revisions after primary MoM-TR failure from March 2007 to January 2018 were retrospectively identified. Clinical outcomes, including the Harris hip score (HHS) and key orthopaedic complications, were collected at 3, 6, and 12 months following conversion and every 12 months thereafter. Results The mean follow-up was 84.12 (67–100) months for UTR and 84.23 (66–101) months for CTR. At the last follow-up, the HHS was better in the CTR- than UTR-treated patients. Noteworthy dissimilarities were correspondingly detected in the key orthopaedic complication rates (16.1% for CTR vs. 47.4% for UTR). Statistically significant differences in specific orthopaedic complications were also detected in the re-revision rate (10.3% for UTR vs. 2.5% for CTR), prosthesis loosening rate (16.3% for UTR vs. 5.9% for CTR), and periprosthetic fracture rate (12.0% for UTR vs. 4.2% for CTR). Conclusion In the setting of revision of failed primary MoM-TR, CTR may demonstrate advantages over UTR in improving functional outcomes and reducing key orthopaedic complications.

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The association of artificial intelligence-enabled electrocardiogram-derived age (physiologic age) with atherosclerotic cardiovascular events in the community

European Heart Journal ◽

10.1093/ehjci/ehaa946.2905 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

J Medina-Inojosa ◽

A Ladejobi ◽

Z Attia ◽

M Shelly-Cohen ◽

B Gersh ◽

...

Keyword(s):

Artificial Intelligence ◽

Bypass Graft ◽

P Value ◽

Funding Source ◽

Atherosclerotic Cardiovascular Disease ◽

Age Gap ◽

Predicted Risk ◽

Age And Sex

Abstract Background We have demonstrated that artificial intelligence interpretation of ECGs (AI-ECG) can estimate an individual's physiologic age and that the gap between AI-ECG and chronologic age (Age-Gap) is associated with increased mortality. We hypothesized that Age-Gap would predict long-term atherosclerotic cardiovascular disease (ASCVD) and that Age-Gap would refine the ACC/AHA Pooled Cohort Equations' (PCE) predictive abilities. Methods Using the Rochester Epidemiology Project (REP) we evaluated a community-based cohort of consecutive patients seeking primary care between 1998–2000 and followed through March 2016. Inclusion criteria were age 40–79 and complete data to calculate PCE. We excluded those with known ASCVD, AF, HF or an event within 30 days of baseline.A neural network, trained, validated, and tested in an independent cohort of ∼ 500,000 independent patients, using 10-second digital samples of raw, 12 lead ECGs. PCE was categorized as low<5%, intermediate 5–9.9%, high 10–19.9%, and very high≥20%. The primary endpoint was ASCVD and included fatal and non-fatal myocardial infarction and ischemic stroke; the secondary endpoint also included coronary revascularization [Percutaneous Coronary Intervention (PCI) or Coronary Artery Bypass Graft (CABG)], TIA and Cardiovascular mortality. Events were validated in duplicate. Follow-up was truncated at 10 years for PCE analysis. The association between Age-Gap with ASCVD and expanded ASCVD was assessed with cox proportional hazard models that adjusted for chronological age, sex and risk factors. Models were stratified by PCE risk categories to evaluate the effect of PCE predicted risk. Results We included 24,793 patients (54% women, 95% Caucasian) with mean follow up of 12.6±5.1 years. 2,366 (9.5%) developed ASCVD events and 3,401 (13.7%) the expanded ASCVD. Mean chronologic age was 53.6±11.6 years and the AI-ECG age was 54.5±10.9 years, R2=0.7865, p<0.0001. The mean Age-Gap was 0.87±7.38 years. After adjusting for age and sex, those considered older by ECG, compared to their chronologic age had a higher risk for ASCVD when compared to those with <−2 SD age gap (considered younger by ECG). (Figure 1A), with similar results when using the expanded definition of ASCVD (data not shown). Furthermore, Age-Gap enhanced predicted capabilities of the PCE among those with low 10-year predicted risk (<5%): Age and sex adjusted HR 4.73, 95% CI 1.42–15.74, p-value=0.01 and among those with high predicted risk (>20%) age and sex adjusted HR 6.90, 95% CI 1.98–24.08, p-value=0.0006, when comparing those older to younger by ECG respectively (Figure 1B). Conclusion The difference between physiologic AI-ECG age and chronologic age is associated with long-term ASCVD, and enhances current risk calculators (PCE) ability to identify high and low risk individuals. This may help identify individuals who should or should not be treated with newer, expensive risk-reducing therapies. Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): Mayo Clinic

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Sustained ventricular tachycardia of left, right or both bundle branch block morphology in patients with Arrhythmogenic Cardiomyopathy

European Heart Journal ◽

10.1093/ehjci/ehaa946.0452 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Milman ◽

M Laredo ◽

R Roudijk ◽

G Peretto ◽

A Andorin ◽

...

Keyword(s):

Ventricular Tachycardia ◽

Right Bundle Branch Block ◽

Left Bundle Branch Block ◽

Clinical Course ◽

Left Ventricular ◽

Funding Source ◽

Arrhythmogenic Cardiomyopathy ◽

Icd Implantation ◽

Bundle Branch Block ◽

Group 3

Abstract Aims In arrhythmogenic cardiomyopathy (ACM) sustained monomorphic ventricular tachycardia (VT) typically displays left bundle branch block (LBBB) morphology. Sustained VT with right bundle branch block (RBBB) morphology is very rare despite the frequent left ventricular involvement. The present study sought to assess the prevalence of spontaneous sustained LBBB-VT, RBBB-VT or both as well as clinical and genetic differences associated with these VT types. Methods and results Twenty-six centers from 11 European countries provided information on 952 patients with ACM and >1 episode of sustained VT observed during the patients' clinical course. VT was classified as: LBBB-VT; RBBB-VT or LBBB+RBBB-VT. Among 952 patients, 881 (92.5%) had LBBB-VT alone, 71 (7.5%) had RBBB-VT [alone in 42 (4.4%) patients or with LBBB-VT in 29 (3.0%) patients]. Male prevalence was 90.5%, 79.2% and 55.9% in the RBBB-VT, LBBB-VT and LBBB+RBBB-VT groups, respectively (P=0.001). Patients' age at first VT did not differ amongst the 3 VT groups. ICD implantation was more frequent for the RBBB-VT and the LBBB+RBBB groups (≈90% each) vs. 67.9% for the LBBB-VT group (P=0.001). Death incidence (9.5%–17.2%) was not significantly different between the 3 groups (P=0.425). Plakophylin-2 mutations predominated in the LBBB-VT and LBBB-VT+RBBB-VT groups (47.2% and 27.3%, respectively) and Desmoplakin mutations in the RBBB-VT group (36.7%). Conclusion This large European survey demonstrates: 1) Sustained RBBB-VT is documented in 7.5% patients with ACM; 2) Males markedly predominate in the RBBB-VT and LBBB-VT groups but not in the LBBB+RBBB VT group; 3) Distribution of desmosomal mutations appears to be different in the 3 VT groups. Funding Acknowledgement Type of funding source: None

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