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Archivos de Pediatría del Uruguay
versão impressa ISSN 0004-0584versão On-line ISSN 1688-1249
Resumo
PEREZ, Joaquín et al. Arrhythmogenic right ventricular cardiomyopathy in pediatrics. Arch. Pediatr. Urug. [online]. 2020, vol.91, n.4, pp.237-242. Epub 01-Ago-2020. ISSN 0004-0584. https://doi.org/10.31134/ap.91.4.6.
Introduction:
the classical cardiomyopathy phenotypic classification includes hypertrophic, dilated, restrictive and non-compacted types. Arrythmogenic Right Ventricle Cardiomyopathy (ARVC) is a hereditary, infiltrative variety that causes the progressive replacement of ventricular myocardium with fibroadipose tissue that mainly affects the right ventricle.1-2-3 Although the onset of symptoms is uncommon in children, they seem to show more sudden death episodes than adults.
Clinical case:
12 year-old male, with arrhythmogenic right ventricle cardiomyopathy (ARVC), showing ventricular arrhythmia at 7 years of age, (extrasystoles and unsustained ventricular tachycardia). Further studies confirmed electrical abnormality, so we performed an electrophysiological study with ablation. The procedure resulted in a slight temporary improvement, however, but the arrhythmia was not completely eliminated. This maintained the risk of sudden death, so we implanted a portable cardiodesfibrilador, which did not record discharges since then.
Discussion
. for this patient’s diagnosis we used the criteria set by Task Force 2010.4 The imaging diagnosis was based on the demonstration of morphological (aneurysms) or functional alterations; akinesia, dyskinesia or asynchrony of the wall, which show myocardium fibrofatty substitution. 3 Recurring right ventricle arrhythmias, despite intense pharmacological treatment, led to a radiofrequency myocardium ablation and later to the placement of an implantable cardiodesfibrillator.
Conclusions:
it is a rare pathology with extremely poor prognosis due to the severe and uncontrollable arrhythmias triggered by the altered myocardium tissue. The most effective treatment is the placement of a cardiodesfibrillator.
Palavras-chave : Arrhythmogenic right ventricular dysplasia; Pediatrics.