Strength of clinical indication and therapeutic impact of the implantable cardioverter defibrillator in patients with hypertrophic cardiomyopathy

2022 ◽  
Author(s):  
Carlo Fumagalli ◽  
Valentina De Filippo ◽  
Chiara Zocchi ◽  
Luigi Tassetti ◽  
Martina Perazzolo Marra ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Implantable Cardioverter Defibrillator ◽  
Clinical Indication ◽  
Therapeutic Impact ◽  
Cardioverter Defibrillator

Worldwide differences in primary prevention implantable cardioverter defibrillator utilization and outcomes in hypertrophic cardiomyopathy

2021 ◽  
Author(s):  
Victor Nauffal ◽  
Peter Marstrand ◽  
Larry Han ◽  
Victoria N Parikh ◽  
Adam S Helms ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Primary Prevention ◽  
Implantable Cardioverter Defibrillator ◽  
Cox Proportional Hazards ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Cardioverter Defibrillator ◽  
Significant Difference ◽  
Implantation Rates ◽  
The Impact

Abstract Aims  Risk stratification algorithms for sudden cardiac death (SCD) in hypertrophic cardiomyopathy (HCM) and regional differences in clinical practice have evolved over time. We sought to compare primary prevention implantable cardioverter defibrillator (ICD) implantation rates and associated clinical outcomes in US vs. non-US tertiary HCM centres within the international Sarcomeric Human Cardiomyopathy Registry. Methods and results We included patients with HCM enrolled from eight US sites (n = 2650) and five non-US (n = 2660) sites and used multivariable Cox-proportional hazards models to compare outcomes between sites. Primary prevention ICD implantation rates in US sites were two-fold higher than non-US sites (hazard ratio (HR) 2.27 [1.89–2.74]), including in individuals deemed at high 5-year SCD risk (≥6%) based on the HCM risk-SCD score (HR 3.27 [1.76–6.05]). US ICD recipients also had fewer traditional SCD risk factors. Among ICD recipients, rates of appropriate ICD therapy were significantly lower in US vs. non-US sites (HR 0.52 [0.28–0.97]). No significant difference was identified in the incidence of SCD/resuscitated cardiac arrest among non-recipients of ICDs in US vs. non-US sites (HR 1.21 [0.74–1.97]). Conclusion  Primary prevention ICDs are implanted more frequently in patients with HCM in US vs. non-US sites across the spectrum of SCD risk. There was a lower rate of appropriate ICD therapy in US sites, consistent with a lower-risk population, and no significant difference in SCD in US vs. non-US patients who did not receive an ICD. Further studies are needed to understand what drives malignant arrhythmias, optimize ICD allocation, and examine the impact of different ICD utilization strategies on long-term outcomes in HCM.

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

EP Europace ◽  
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.

Effectiveness of subcutaneous implantable cardioverter-defibrillator testing in patients with hypertrophic cardiomyopathy

2017 ◽  
Vol 231 ◽  
pp. 115-119 ◽  
Author(s):  
Niccoló Maurizi ◽  
Ilaria Tanini ◽  
Iacopo Olivotto ◽  
Ernesto Amendola ◽  
Giuseppe Limongelli ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Implantable Cardioverter Defibrillator ◽  
Cardioverter Defibrillator
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