ArticlePediatric cardiology2016
Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D): Review of 16 Pediatric Cases and a Proposal of Modified Pediatric Criteria.
Article in Pediatric cardiology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 1 of them a synthesis that pooled it.
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Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 38 citations in OpenAlex.
- Arrhythmogenic Right Ventricular Cardiomyopathy in Pediatric Patients: An Important but Underrecognized Clinical Entity.Frontiers in pediatrics · 2021Pooled it
- Outcomes of Ventricular Tachycardia Catheter Ablation in Pediatric Arrhythmogenic Right Ventricular Cardiomyopathy.Circulation. Arrhythmia and electrophysiology · 2025Article
- Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review.Diagnostics (Basel, Switzerland) · 2024Review
- Inherited Arrhythmias in the Pediatric Population: An Updated Overview.Medicina (Kaunas, Lithuania) · 2024Review
- The safety of sports in children with inherited arrhythmia substrates.Frontiers in pediatrics · 2023Review
- The Many Faces of Arrhythmogenic Cardiomyopathy: An Overview.The application of clinical genetics · 2023Review
- The phenotypic and genetic features of arrhythmogenic cardiomyopathy in the pediatric population.Frontiers in cardiovascular medicine · 2023Article
- Arrhythmogenic Cardiomyopathy: Diagnosis, Evolution, Risk Stratification and Pediatric Population-Where Are We?Journal of cardiovascular development and disease · 2022Review
- Anatomical-MRI Correlations in Adults and Children with Arrhythmogenic Right Ventricular Cardiomyopathy.Diagnostics (Basel, Switzerland) · 2021Review
- Electrocardiographic features of arrhythmogenic right ventricular cardiomyopathy in school-aged children.Heart and vessels · 2021Article
- Right Heart Morphology and Its Association With Excessive and Deficient Cardiac Visceral Adipose Tissue.Clinical Medicine Insights. Cardiology · 2021Review
- Propafenone-Induced QRS Widening in a Child With Arrhythmogenic Right Ventricular Cardiomyopathy: A Case Report and Literatures Review.Frontiers in pediatrics · 2020Article
- Sudden Cardiac Death in Children Affected by Cardiomyopathies: An Update on Risk Factors and Indications at Transvenous or Subcutaneous Implantable Defibrillators.Frontiers in pediatrics · 2020Review
- Clinical features and arrhythmic complications of patients with pediatric-onset arrhythmogenic right ventricular dysplasia.Anatolian journal of cardiology · 2019Article
- An Update on Pediatric Cardiomyopathy.Current treatment options in cardiovascular medicine · 2019Review
- Fatty Infiltration of the Myocardium and Arrhythmogenesis:Frontiers in physiology · 2018Review
- High proportion of genetic cases in patients with advanced cardiomyopathy including a novel homozygous Plakophilin 2-gene mutation.PloS one · 2017Article
- Arrhythmogenic Right Ventricular Dysplasia in Neuromuscular Disorders.Clinical Medicine Insights. Cardiology · 2016Review
- Cardiac arrest secondary to arrhythmogenic right ventricular cardiomyopathy in an adolescent male.Indian pacing and electrophysiology journalArticle
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Authors and funding
7 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a heritable cardiomyopathy characterized by fibro-fatty replacement of right ventricular myocardium. Diagnostic criteria, established in 1994 and modified in 2010, are based on predominately adult manifestations of ARVC/D. The goal of this paper is to review a single-center experience with pediatric ARVC/D and propose modifications of current diagnostic criteria to appropriately include pediatric ARVC/D. We identified 16 pediatric cases of ARVC/D from our tertiary care center. Patient demographics, presentation, course, genetic testing, and family history were reviewed. Sixteen patients were diagnosed with ARVC/D through the modified diagnostic criteria, genetic testing, and pathology. Five patients had positive family histories. Five patients presented with cardiac arrest, and six were found to have ventricular tachycardia. Two patients presented with heart failure. Six autopsies, six explanted hearts, and three biopsies found massive fibro-fatty infiltration of the right ventricular wall. Six patients underwent heart transplantation, and two have received automatic implantable cardioverter defibrillator. Two patients had identifiable genetic mutations previously noted in the literature. One patient had a novel mutation of a known ARVC/D gene. Many pediatric patients do not meet the current ARVC/D diagnostic criteria, resulting in delays in diagnosis and treatment. The current criteria need further revision to encompass pediatric manifestations of ARVC/D. In our opinion, pathological and clinical findings alone are sufficient for accurate diagnosis of pediatric ARVC/D. Creating modified pediatric criteria would facilitate prompt diagnosis and management of ARVC/D and facilitate structured research with the goal of improving outcomes.
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