Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by the progressive replacement of the normal myocardium by fibroadipocytic tissue. The importance of an early diagnosis is supported by a higher risk of sudden cardiac death in the pediatric population. We r...
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
MDPI AG
2024-01-01
|
| Series: | Diagnostics |
| Subjects: | |
| Online Access: | https://www.mdpi.com/2075-4418/14/2/175 |
| _version_ | 1797344264532787200 |
|---|---|
| author | Stefana Maria Moisa Elena Lia Spoiala Eliza Cinteza Radu Vatasescu Lacramioara Ionela Butnariu Crischentian Brinza Alexandru Burlacu |
| author_facet | Stefana Maria Moisa Elena Lia Spoiala Eliza Cinteza Radu Vatasescu Lacramioara Ionela Butnariu Crischentian Brinza Alexandru Burlacu |
| author_sort | Stefana Maria Moisa |
| collection | DOAJ |
| description | Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by the progressive replacement of the normal myocardium by fibroadipocytic tissue. The importance of an early diagnosis is supported by a higher risk of sudden cardiac death in the pediatric population. We reviewed the literature on diagnosis, risk stratification, and prognosis in the pediatric population with ARVC. In case reports which analyzed children with ARVC, the most common sign was ventricular tachycardia, frequently presenting as dizziness, syncope, or even cardiac arrest. Currently, there is no gold standard for diagnosing ARVC in children. Nevertheless, genetic analysis may provide a proper diagnosis tool for asymptomatic cases. Although risk stratification is recommended in patients with ARVC, a validated prediction model for risk stratification in children is still lacking; thus, it is a matter of further research. In consequence, even though ARVC is a relatively rare condition in children, it negatively impacts the survival and clinical outcomes of the patients. Therefore, appropriate and validated diagnostic and risk stratification tools are crucial for the early detection of children with ARVC, ensuring a prompt therapeutic intervention. |
| first_indexed | 2024-03-08T10:59:47Z |
| format | Article |
| id | doaj.art-09af98d247694943a26c6b8afa1545eb |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-08T10:59:47Z |
| publishDate | 2024-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-09af98d247694943a26c6b8afa1545eb2024-01-26T16:01:36ZengMDPI AGDiagnostics2075-44182024-01-0114217510.3390/diagnostics14020175Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic ReviewStefana Maria Moisa0Elena Lia Spoiala1Eliza Cinteza2Radu Vatasescu3Lacramioara Ionela Butnariu4Crischentian Brinza5Alexandru Burlacu6Pediatrics Department, Faculty of Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iasi, RomaniaPediatrics Department, Faculty of Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iasi, RomaniaPediatrics Department, Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, 700115 Bucharest, RomaniaCardio-Thoracic Department, “Carol Davila” University of Medicine and Pharmacy, 020021 Bucharest, Romania“Sfanta Maria” Clinical Emergency Hospital for Children, 700309 Iasi, RomaniaFaculty of Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iasi, RomaniaFaculty of Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iasi, RomaniaArrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease characterized by the progressive replacement of the normal myocardium by fibroadipocytic tissue. The importance of an early diagnosis is supported by a higher risk of sudden cardiac death in the pediatric population. We reviewed the literature on diagnosis, risk stratification, and prognosis in the pediatric population with ARVC. In case reports which analyzed children with ARVC, the most common sign was ventricular tachycardia, frequently presenting as dizziness, syncope, or even cardiac arrest. Currently, there is no gold standard for diagnosing ARVC in children. Nevertheless, genetic analysis may provide a proper diagnosis tool for asymptomatic cases. Although risk stratification is recommended in patients with ARVC, a validated prediction model for risk stratification in children is still lacking; thus, it is a matter of further research. In consequence, even though ARVC is a relatively rare condition in children, it negatively impacts the survival and clinical outcomes of the patients. Therefore, appropriate and validated diagnostic and risk stratification tools are crucial for the early detection of children with ARVC, ensuring a prompt therapeutic intervention.https://www.mdpi.com/2075-4418/14/2/175arrhythmogenic right ventricular dysplasiacardiomyopathychildren |
| spellingShingle | Stefana Maria Moisa Elena Lia Spoiala Eliza Cinteza Radu Vatasescu Lacramioara Ionela Butnariu Crischentian Brinza Alexandru Burlacu Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review Diagnostics arrhythmogenic right ventricular dysplasia cardiomyopathy children |
| title | Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review |
| title_full | Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review |
| title_fullStr | Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review |
| title_full_unstemmed | Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review |
| title_short | Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review |
| title_sort | arrhythmogenic right ventricular cardiomyopathy in children a systematic review |
| topic | arrhythmogenic right ventricular dysplasia cardiomyopathy children |
| url | https://www.mdpi.com/2075-4418/14/2/175 |
| work_keys_str_mv | AT stefanamariamoisa arrhythmogenicrightventricularcardiomyopathyinchildrenasystematicreview AT elenaliaspoiala arrhythmogenicrightventricularcardiomyopathyinchildrenasystematicreview AT elizacinteza arrhythmogenicrightventricularcardiomyopathyinchildrenasystematicreview AT raduvatasescu arrhythmogenicrightventricularcardiomyopathyinchildrenasystematicreview AT lacramioaraionelabutnariu arrhythmogenicrightventricularcardiomyopathyinchildrenasystematicreview AT crischentianbrinza arrhythmogenicrightventricularcardiomyopathyinchildrenasystematicreview AT alexandruburlacu arrhythmogenicrightventricularcardiomyopathyinchildrenasystematicreview |