Arrhythmogenic right ventricular dysplasia/cardiomyopathy

Orgeron, Gabriela M.; Crosson, Jane E.

doi:10.1017/s1047951116002249

Cited by 7 publications

(6 citation statements)

References 34 publications

(53 reference statements)

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“…Although ARVD has been reported as a major cause of sudden cardiac death in the young patients (1), the prevalence of ARVD in the pediatric population might be higher than reported (17). In previous studies, affected patients usually present during the second to fifth decades of life (3, 18). In our study, the mean age of the patients was 12 years, and the youngest patient was an infant boy aged 1.5 years.…”

Section: Discussionmentioning

confidence: 94%

“…This early phase of the disease is called as the “concealed phase,” and the ventricular morphology with functions is preserved. However, the risk of sudden cardiac death due to ventricular arrhythmias exists in this stage, and it might be the first and only manifestation of the disorder (2, 4, 6, 18). We examined previous echocardiography and cardiac MRI findings of 2 patients who presented with cardiac arrest and the other with chest pain at the time of admission.…”

Section: Discussionmentioning

confidence: 99%

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Clinical features and arrhythmic complications of pediatric-onset arrhythmogenic right ventricular dysplasia

et al. 2019

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Objective:Arrhythmogenic right ventricular dysplasia (ARVD) is a myocardial genetic disease that occurs primarily in the right ventricle. Patients with ARVD may present with severe ventricular arrhythmias, syncope, and cardiac arrest. The purpose of this study is to evaluate the clinical features and arrhythmic complications of patients with pediatric-onset ARVD.Methods:Patients diagnosed with ARVD between January 2010 and January 2019 were included in this study.Results:A total of 19 patients with ARVD were evaluated. Of them, 15 patients were male, and their mean age was 12±4 years. The most common symptoms were palpitations (n=6), syncope (n=4), and heart failure symptoms (n=2). Five patients were asymptomatic. Thirteen patients had an epsilon wave; all patients ≥14 years had a T wave inversion in V1–3. Premature ventricular contractions (PVCs) were observed in 15 patients, and ventricular tachycardia (VT) was observed in 9 patients. All patients underwent cardiac magnetic resonance imaging (MRI). Echocardiography and cardiac MRI of two patients were normal at the time of admission; patients were in the concealed phase, and the diagnosis was made by ECG, Holter monitoring, and genetic findings. We administered a beta-blocker in all patients. Two patients underwent an electrophysiological study and ablation because of PVC/VT. An implantable cardiac defibrillator was implanted in 8 patients. The mean follow-up period was 21.5±11 months. Two patients were deceased with incessant VT and heart failure, and one patient was deceased with multiorgan dysfunction after biventricular assist device implantation (n=3).Conclusion:Diagnosis of pediatric-onset ARVD might be much more difficult in children. Sudden cardiac death might be prevented in the early period by raising the awareness of physicians about the disorder. Prevention of sudden death with implantable cardiac defibrillators is crucial in the management of these patients. It should be kept in mind that children with structurally normal hearts may present with an earlier concealed phase and can be diagnosed with ARVD.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Clinical features and arrhythmic complications of pediatric-onset arrhythmogenic right ventricular dysplasia

et al. 2019

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“…Fat replacement of up to 50% of the free wall was also found in some areas of the right ventricle, but this was very localized. There is a possibility that physical activity may have triggered the hot phase of the disease, and mechanical stress may have caused the decoupling of more myocytes, with the consequent secondary injury with predisposition to ventricular arrhythmias and sudden death [17].…”

Section: Description Of the Canine Casementioning

confidence: 99%

Left Ventricle Arrhythmogenic Cardiomyopathy in Canines and Felines

Belerenian,

Daniel Rodríguez,

Castillo

et al. 2024

Exploring the Causes, Prevention and Management of Cardiomyopathy [Working Title]

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This chapter describes pathological, electrocardiographic, echocardiographic, and clinical findings of two cases, one in a canine and the other in a feline, which suggest the presence of Left Ventricle Arrhythmogenic Cardiomyopathy. It is considered to be of interest for comparative medicine. To the authors’ knowledge, it’s the first ever description of the pathology carried out in companion animals. The canine patient arrived at the clinic with arrhythmias. After 48 hours, he had a sudden death and the heart was studied, finding a partial replacement of the free wall and septum of the left ventricle with fatty tissue. The feline patient also had a sudden death, and the heart was studied, where transmural replacement of the myocardium from the free wall of the left ventricle was found, which was replaced by adipose tissue.

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“…Konservatyvusis gydymo būdas apima gyvensenos pakeitimą: pacientams rekomenduojama vengti intensyvaus, įtempto, varžybinio sporto ir svorių kilnojimo, apsiribojant mažo intensyvumo fizine veikla [8].…”

Section: Tyrimo Rezultataiunclassified

Aritmogeninė Dešiniojo Skilvelio Kardiomiopatija: Patofiziologija, Klinika, Diagnostika Ir Gydymas

Jasaitė

2024

Health Sciences

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Aritmogeninė dešiniojo skilvelio kardiomiopatija (ADSK), anksčiau vadinta aritmogenine dešiniojo skilvelio displazija (ADSD), yra paveldima kardiomiopatija, kuriai būdingos skilvelinės aritmijos ir padidėjusi staigios širdinės mirties rizika. Dėl nespecifinio ligos pobūdžio ir plataus klinikinio pasireiškimo spektro diagnostika dažnai būna sudėtinga. Pagrindinis ADSK gydymo tikslas – užkirsti kelią ligos progresavimui, kontroliuoti simptomus bei sumažinti staigios mirties riziką.

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Arrhythmogenic right ventricular dysplasia/cardiomyopathy

Cited by 7 publications

References 34 publications

Clinical features and arrhythmic complications of pediatric-onset arrhythmogenic right ventricular dysplasia

Clinical features and arrhythmic complications of pediatric-onset arrhythmogenic right ventricular dysplasia

Left Ventricle Arrhythmogenic Cardiomyopathy in Canines and Felines

Aritmogeninė Dešiniojo Skilvelio Kardiomiopatija: Patofiziologija, Klinika, Diagnostika Ir Gydymas

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