Living after sudden death: A case report of Naxos disease

Noain, J.A. García; Golet, Amparo Cantin; Calzada, Jorge Navarro; Mellado, Ascension Muñoz; Duarte, J. Mozota

doi:10.4103/0972-5229.106503

Cited by 5 publications

(1 citation statement)

References 10 publications

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“…Naxos-specific guidelines are rare. Among those, an ICD is indicated for patients who are symptomatic or present structural progression especially before the age of 35 [8,44]. Naxos patients, like ARVC patients, should abstain from competitive sports as myocardial stress can exacerbate the dysplasia [40].…”

Section: Arrhythmias and Sudden Cardiac Deathmentioning

confidence: 99%

Naxos Disease: Current Knowledge and Future Advances

Leopoulou¹,

Mattsson²,

Kåks³

et al. 2021

Cardiomyopathy - Disease of the Heart Muscle

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Naxos disease is a genetic cardiocutaneous syndrome manifesting with a cardiomyopathy that belongs in the arrhythmogenic right ventricular cardiomyopathy (ARVC) spectrum and follows an autosomal recessive pattern. It manifests with wooly hair, keratosis of the extremities and right ventricular dysfunction. It is accompanied by risk of arrhythmias as well as sudden cardiac death (SCD), even at a young age. Furthermore, the disease often progresses to right ventricular heart failure, but can also affect the left ventricle. Patient management follows current guidelines on ARVC and principles for heart failure management. Bioengineering and research about pluripotent stem cells seem to have potential to improve future management of the disease. This chapter covers current knowledge on Naxos disease regarding clinical features, epidemiology, pathogenesis, guidelines on patient management and provides insights in research frontlines.

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Section: Arrhythmias and Sudden Cardiac Deathmentioning

confidence: 99%