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February 5, 2026Pacing and Clinical Electrophysiology0 citations

When Substrate Meets Device: ICD Implantation and Therapy across the Spectrum of Cardiomyopathies

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NSNicolò SistiState Administration of Foreign Experts AffairsACAndrea CardonaAdvanced Cell Diagnostics (United States)LLLudovico LazzariSanta Maria Nuova Hospital

Key Result

ICD therapy in cardiomyopathies requires individualized programming and system selection to balance arrhythmic protection against disease-specific risks and device-related complications.

Key Points

  • This review aims to evaluate the impact of structural and electrophysiological variations in cardiomyopathies on ICD performance.
  • Structured narrative synthesis of current evidence and experiences
  • Assessment of ICD implantation, programming, and management across HCM, ARVC, and CA
  • Identification of specific technical challenges and complication trends for each cardiomyopathy phenotype
  • Emphasized lead placement and programming strategies in hypertrophic cardiomyopathy to reduce inappropriate therapies
  • Explored lead stability and sensing challenges in arrhythmogenic right ventricular cardiomyopathy
  • Discussed individualized defibrillation thresholds and indications for cardiac amyloidosis
  • Highlighted the importance of tailored programming and remote monitoring for improved outcomes

Structured PICO

P
Population
Patients with cardiomyopathies, specifically hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and cardiac amyloidosis (CA)
I
Intervention
Implantable cardioverter-defibrillators (ICDs) implantation, programming, and management
O
Outcome
Phenotype-specific technical challenges, programming strategies, and complication trends

ICD therapy in cardiomyopathies requires individualized approaches to implantation and programming based on the specific disease phenotype (HCM, ARVC, CA) to optimize safety and efficacy.

Abstract

ABSTRACT Introduction Implantable cardioverter‐defibrillators (ICDs) are the cornerstone of sudden cardiac death prevention in cardiomyopathies, but disease‐specific structural and electrophysiological substrates profoundly affect device performance and outcomes. Understanding these distinctions is critical for tailoring therapy and improving patient safety. Areas covered This review examines current evidence and practical experience regarding ICD implantation, programming, and management in hypertrophic cardiomyopathy (HCM), arrhythmogenic right ventricular cardiomyopathy (ARVC), and cardiac amyloidosis (CA). We performed a structured narrative synthesis to identify phenotype‐specific technical challenges, programming strategies, and complication trends. For HCM, lead placement and programming to minimize inappropriate therapies are emphasized; for ARVC, issues of lead instability, oversensing, and ATP efficacy are explored; and for CA, defibrillation thresholds, sensing difficulties, and individualized indications are discussed. Expert opinion ICD therapy in cardiomyopathies must be individualized, balancing arrhythmic protection against disease‐specific risks and device‐related complications. Tailored programming, careful system selection, and remote monitoring are key to improving outcomes. Emerging technologies such as modular and extravascular ICD systems promise safer and more phenotype‐driven care.

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Cite This Study

Sisti et al. (2026) conducted a review in Cardiomyopathies (HCM, ARVC, CA). Implantable cardioverter-defibrillators (ICDs) was evaluated. ICD therapy in cardiomyopathies requires individualized programming and system selection to balance arrhythmic protection against disease-specific risks and device-related complications.

synapsesocial.com/papers/698435aaf1d9ada3c1fb4b2dhttps://doi.org/10.1111/pace.70149
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