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Pediatric-Onset Arrhythmogenic Right Ventricular Cardiomyopathy
Quick Takes
- Pediatric ARVC presentation is predominated by arrhythmias and ECG abnormalities.
- Progression of the phenotype occurs in 50% of patients not meeting diagnostic criteria.
Study Questions:
What are the clinical findings and outcomes of arrhythmogenic right ventricular cardiomyopathy (ARVC) in the pediatric age group?
Methods:
This was a retrospective cohort study of patients <18 years old with ARVC from the Netherlands Arrhythmogenic Cardiomyopathy registry. Pediatric probands and pediatric relatives of probands were included. ARVC diagnosis was based on 2010 Task Force Criteria. The primary outcome was occurrence of potentially life-threatening arrhythmia.
Results:
There were 12 probands and 68 pediatric relatives identified in the 1,109-patient registry. Proband presentations were all arrhythmia related—sustained ventricular tachycardia, recurrent syncope, or sudden cardiac death. Most had an abnormal electrocardiogram (ECG) and nearly half had low ejection fraction. A small percentage of the relatives manifested phenotypic disease at initial evaluation including two presenting with sudden cardiac death. Initially, 60 of 68 did not meet diagnostic criteria; but over a median 8.5 years of follow-up, 50% of relatives developed new phenotypic findings and 30% met diagnostic criteria. Arrhythmia developed before imaging abnormalities. Cardiac magnetic resonance imaging (CMR) abnormalities developed before echocardiography abnormalities.
Conclusions:
Pediatric ARVC proband presentation is characterized by ventricular arrhythmia. ARVC phenotype progresses in half of patients initially not meeting diagnostic criteria. ECG, Holter, and CMR are most revealing of phenotypic findings during ongoing surveillance.
Perspective:
The 6% representation in this 1,100-patient registry reflects the relative rarity of pediatric ARVC phenotypic disease. The more common arrhythmia presentation should raise suspicions in selected patients and guide surveillance for cascade surveillance. ECG, Holter, and CMR appear to be best suited for surveillance in adolescence, though the optimal frequency of surveillance is unclear.
Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Implantable Devices, Genetic Arrhythmic Conditions, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure, Echocardiography/Ultrasound, Magnetic Resonance Imaging
Keywords: Arrhythmias, Cardiac, Arrhythmogenic Right Ventricular Dysplasia, Death, Sudden, Diagnostic Imaging, Echocardiography, Electrocardiography, Heart Failure, Magnetic Resonance Imaging, Pediatrics, Phenotype, Stroke Volume, Syncope, Tachycardia, Ventricular
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