Arrhythmogenic Right Ventricular Cardiomyopathy in Children: A Systematic Review

被引:5
作者
Moisa, Stefana Maria [1 ,2 ]
Spoiala, Elena Lia [1 ]
Cinteza, Eliza [3 ,4 ]
Vatasescu, Radu [5 ,6 ]
Butnariu, Lacramioara Ionela [2 ,7 ]
Brinza, Crischentian [8 ,9 ]
Burlacu, Alexandru [8 ,9 ]
机构
[1] Grigore T Popa Univ Med & Pharm, Fac Med, Pediat Dept, Iasi 700115, Romania
[2] Sfanta Maria Clin Emergency Hosp Children, Iasi 700309, Romania
[3] Carol Davila Univ Med & Pharm, Fac Med, Pediat Dept, Bucharest 700115, Romania
[4] Marie Curie Emergency Clin Hosp Children, Bucharest 41451, Romania
[5] Carol Davila Univ Med & Pharm, Cardiothorac Dept, Bucharest 020021, Romania
[6] Clin Emergency Hosp, Bucharest 050098, Romania
[7] Grigore T Popa Univ Med & Pharm, Fac Med, Genet Dept, Iasi 700115, Romania
[8] Grigore T Popa Univ Med & Pharm, Fac Med, Iasi 700115, Romania
[9] Ist Cardiovasc Dis Prof Dr George IM Georgescu, Iasi 700503, Romania
关键词
arrhythmogenic right ventricular dysplasia; cardiomyopathy; children; PALMOPLANTAR KERATODERMA; RISK STRATIFICATION; DIAGNOSIS; GENETICS; EXERCISE; CRITERIA; TITIN; ARVC;
D O I
10.3390/diagnostics14020175
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
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页数:14
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