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. 2021 May 24;29(12):1825–1832. doi: 10.1038/s41431-021-00904-y
No.
Yes.
Therapy (please describe) Pharmaceutical treatment (usually beta-blockers) as primary and secondary prevention. In rare individual cases additional pacemaker and/or an implantable cardioverter defibrillator (ICD), and/or left cardiac sympathetic denervation (LCSD) is used. The implantation of an ICD with or without the performance of LCSD is mostly reserved for patients in which optimal non-invasive therapy and lifestyle modifications fail to protect against ventricular arrhythmias. Only in exceptional individual cases ICD implantation might be indicated for primary prevention. For more specific therapeutic recommendations please see [22].
Pharmacotherapy might differ between genotypes. Also, life style advices are different for the different genotypes [23, 24].
Prognosis (please describe) In general, even in asymptomatic subjects carrying the disease associated variant, regular preventive medical check-ups to recognise disease progression early, and appropriate treatment improve prognosis. On appropriate treatment and with appropriate life style adjustments prognosis is good in the vast majority of patients and may not differ from normal [25, 26].
Management (please describe) Regular preventive medical consultation of carriers of a variant affecting function, adjustment or intensification of therapy if appropriate (including defibrillator implantation and/or LCSD), if necessary.
The molecular-genetic information is very important for counselling and clinical management related to the different subtypes of the disease.