Implantable cardiac defibrillator events in patients with arrhythmogenic right ventricular cardiomyopathy

Olgierd Woźniak1, Karolina Borowiec2, Marek Konka2

  • 1Department of Congenital Heart Diseases, National Institute of Cardiology, Warsaw, Poland owozniak@wp.pl.

Insights

Arrhythmogenic right ventricular cardiomyopathy (ARVC) patients with right ventricle dysfunction, younger age, or a history of ventricular tachycardia face higher risks of appropriate ICD interventions. These findings aid in refining risk stratification for ARVC patients needing defibrillators.

Area of Science:

  • Cardiology
  • Electrophysiology
  • Genetics

Background:

  • Arrhythmogenic right ventricular cardiomyopathy (ARVC) poses a significant risk of sudden cardiac death.
  • Current strategies for optimal risk stratification in ARVC patients are still under investigation.
  • Implantable cardioverter-defibrillators (ICDs) are crucial for managing life-threatening arrhythmias in ARVC.

Purpose of the Study:

  • To identify predictors of appropriate and inappropriate ICD interventions in ARVC patients.
  • To enhance risk stratification models for ARVC patients undergoing ICD implantation.
  • To analyze long-term outcomes of ICD therapy in a cohort of ARVC patients.

Main Methods:

  • A 20-year, single-centre observational study involving 65 adult ARVC patients who received ICDs.
  • Analysis of primary or secondary prevention ICD implantation for sudden cardiac death.
  • Evaluation of appropriate (shock/pacing) and inappropriate ICD interventions as study endpoints.

Main Results:

  • During a median 7.75-year follow-up, 66.2% of patients experienced appropriate ICD interventions.
  • Predictors for appropriate ICD interventions included right ventricle dysfunction (HR 2.85), age <40 years (HR 2.37), and history of sustained ventricular tachycardia (HR 2.55).
  • No significant predictors for inappropriate ICD therapy were identified; 27.7% of patients had inappropriate interventions.

Conclusions:

  • Right ventricle dysfunction, younger age at implantation (<40 years), and a history of sustained ventricular tachycardia are key predictors of appropriate ICD interventions in ARVC.
  • These identified predictors can inform improved risk stratification protocols prior to ICD implantation in ARVC patients.
  • Further research may refine patient selection for ICDs to optimize outcomes in ARVC.
Abstract

Related Concept Videos

Cardiomyopathy V: Interprofessional Care01:29

Cardiomyopathy V: Interprofessional Care

Managing cardiomyopathy involves addressing underlying or precipitating causes, treating heart failure with medications, and implementing dietary changes and a balanced exercise and rest regimen.Lifestyle ModificationsCardiomyopathy patients should adopt a low-sodium diet to reduce fluid retention and manage heart failure. A personalized exercise and rest plan helps maintain physical fitness without overstraining the heart. Avoiding alcohol and tobacco is essential to prevent further damage to...
119
Cardiomyopathy II: Dilated Cardiomyopathy01:30

Cardiomyopathy II: Dilated Cardiomyopathy

Dilated cardiomyopathy, or DCM, is a progressive myocardial disorder characterized by ventricular chamber dilation and contractile dysfunction.EtiologyVarious factors can cause DCM, including hypertension and heavy alcohol intake, which contribute to the weakening and enlargement of the heart muscle. Viral infections, such as Coxsackievirus B, adenoviruses, and influenza, can lead to DCM by causing inflammation and damage to heart tissue. Certain chemotherapeutic agents, including daunorubicin,...
146
Disturbances in Heart Rhythm01:29

Disturbances in Heart Rhythm

Arrhythmia or dysrhythmia refers to an abnormal heart rhythm caused by a defect in the heart's conduction system. It can cause the heart to beat irregularly, too quickly, or too slowly, leading to symptoms like chest pain, shortness of breath, and fainting. Factors such as stress, caffeine, alcohol, nicotine, cocaine, certain drugs, congenital defects, diseases, and electrolyte abnormalities can trigger arrhythmias.
Arrhythmias are categorized by their speed, rhythm, and origin. A slow heart...
1.9K
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
187
ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias01:25

ECG Interpretation of Arrhythmias II: Atrial, Junctional and Ventricular Arrhythmias

Arrhythmia is a condition characterized by an irregular heart rhythm, with ECG changes that differ based on its origin and nature. The types of arrhythmias discussed below include atrial, junctional, and ventricular arrhythmias.Atrial ArrhythmiasPremature Atrial Complexes (PACs): PACs are early atrial beats caused by stress, caffeine, alcohol, electrolyte imbalances, hypoxia, hyperthyroidism, or certain medications (e.g., bronchodilators and decongestants). The ECG shows early P waves with an...
253
Dysrhythmias VI: Management of Dysrhythmias01:25

Dysrhythmias VI: Management of Dysrhythmias

Dysrhythmia management involves a multifaceted approach, incorporating pharmacological treatments, medical procedures, surgical interventions, lifestyle modifications, and patient education.Pharmacological ManagementAntiarrhythmic Drugs:Class I (Sodium Channel Blockers): This class includes quinidine and procainamide, which reduce the speed of impulse conduction in the heart, stabilize the cardiac membrane, and control arrhythmias. Quinidine and procainamide are Class IA agents that prolong the...
213