3.3.1 Long QT Syndrome

COR	Recommendations Long QT Syndrome	LOE	References
I	ICD implantation along with the use of beta-blockade is indicated for patients with a diagnosis of LQTS who are survivors of SCA. In select LQTS patients, medical therapy and/or cardiac sympathetic denervation may be considered as an alternative.	B-NR	56 57
I	ICD implantation is indicated in LQTS patients with symptoms (arrhythmic syncope or VT) in whom beta-blockade is either ineffective or not tolerated and cardiac sympathetic denervation or other medications are not considered effective alternatives.	B-NR	58 59
IIb	ICD therapy may be considered for primary prevention in LQTS patients with established clinical risk factors and/or pathogenic mutations (see text).	C-LD	60
III Harm	ICD implantation is not indicated in asymptomatic LQTS patients who are deemed to be at low risk of SCA and have not been tried on beta-blocker therapy.	C-LD	61