From the Guidelines
The management of Arrhythmogenic Right Ventricular (ARV) dysplasia should focus on preventing life-threatening arrhythmias, controlling symptoms, and slowing disease progression, with implantable cardioverter-defibrillator (ICD) placement recommended for high-risk patients with sustained ventricular tachycardia, syncope, or family history of sudden cardiac death, as stated in the 2017 AHA/ACC/HRS guideline 1.
Key Recommendations
- ICD placement is recommended for patients with ARV dysplasia and an additional marker of increased risk of sudden cardiac death (SCD), such as resuscitated SCA, sustained VT, or significant ventricular dysfunction with RVEF or LVEF ≤35% 1.
- Beta-blockers are recommended for patients with ARV dysplasia and ventricular arrhythmias (VA) 1.
- Avoiding intensive exercise is recommended for patients with a clinical diagnosis of ARV dysplasia 1.
- Genetic counseling and genetic testing can be useful for diagnosis and for gene-specific targeted family screening 1.
Treatment Approach
The treatment approach for ARV dysplasia typically includes:
- Antiarrhythmic medications, such as beta-blockers, to control symptoms and reduce ICD shocks.
- Catheter ablation for recurrent ventricular tachycardia despite medical therapy.
- Exercise restriction, with complete avoidance of competitive and endurance sports.
- Heart failure symptoms are managed with standard therapies, including ACE inhibitors, beta-blockers, and diuretics as needed.
Family Screening
Family screening is crucial since ARVD is genetic (autosomal dominant), with first-degree relatives requiring clinical evaluation, ECG, echocardiography, and possibly cardiac MRI 1.
Additional Considerations
Heart transplantation may be considered in end-stage disease with refractory heart failure or uncontrollable arrhythmias. The management approach is based on the progressive nature of ARVD, where fibro-fatty replacement of right ventricular myocardium creates arrhythmogenic substrates and eventually leads to ventricular dysfunction. It is worth noting that the 2015 ESC guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death also provide recommendations for the treatment of ARVC, including the use of beta-blockers and catheter ablation 1. However, the 2017 AHA/ACC/HRS guideline 1 is more recent and provides more comprehensive recommendations for the management of ARV dysplasia.
From the Research
Definition of Arrhythmogenic Right Ventricular Dysplasia
Arrhythmogenic Right Ventricular (ARV) dysplasia, also known as Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC), is a heart muscle disorder characterized by the replacement of the right ventricular myocardium with fibrofatty tissue, leading to electrical instability and life-threatening ventricular arrhythmias 2, 3, 4, 5, 6.
Clinical Manifestations
The clinical manifestations of ARV dysplasia include:
- Structural and functional malformations of the right ventricle, such as fatty infiltration, dilatation, and aneurysms
- ECG abnormalities, such as T-wave inversion and QRS dispersion
- Ventricular tachycardias with a left bundle branch block pattern
- Sudden cardiac death, often in young, apparently healthy individuals and athletes 2, 3, 4, 5
Diagnosis
The diagnosis of ARV dysplasia is based on a combination of clinical, electrocardiographic, imaging, and histopathological criteria, including:
- Electrocardiographic abnormalities, such as T-wave inversion and QRS dispersion
- Imaging studies, such as echocardiography, angiography, and cardiac magnetic resonance imaging, to evaluate right ventricular structure and function
- Histopathological examination of endomyocardial biopsy specimens to detect fibrofatty replacement of the myocardium 3, 4, 5
Management
The management of ARV dysplasia aims to prevent life-threatening ventricular arrhythmias and sudden cardiac death, and includes:
- Antiarrhythmic drug therapy, such as beta-blockers, sotalol, and amiodarone, to suppress ventricular tachycardia recurrences
- Catheter ablation to eliminate arrhythmia foci
- Implantable cardioverter-defibrillator (ICD) therapy to prevent sudden cardiac death
- Restriction from competitive sports and vigorous training to reduce the risk of sudden cardiac death 2, 3, 4, 5, 6
- Treatment with beta-blockers and angiotensin-converting enzyme inhibitors to reduce the risk of ventricular arrhythmias and sudden cardiac death 6
Risk Stratification
Risk stratification is crucial to identify patients at high risk of sudden cardiac death and to guide treatment decisions, including: