Table. Management of the most common hereditary arrhythmia syndromes^*1.

Syndrome and lifestyle changes	Pharmacotherapy	Implantable cardioverter defibrillator (ICD) for high-risk patients
LQTS (class I, evidence level B)		(With beta-blockers)
Avoid certain drugs No competitive sports Bring down fever early Avoid electrolyte disturbances (hypokalemia, hypomagnesemia) Carry an emergency card Consider changes to worklife if appropriate	Beta-blockers In patients with prolonged QTc (class I, evidence level B) In patients with normal QTc (class IIa, evidence level B)	Patients who have survived sudden cardiac death (class I, evidence level A) Symptoms persisting despite therapy (class IIa, evidence level B)
	In some individual cases:
	Mexiletine plus beta-blockers for LQTS 3 (In some individual cases ranolazine or flecainide plus beta-blockers)
Catecholaminergic polymorphous ventricular tachycardia		(With beta-blockers)
See LQTS	Beta-blockers (class I, evidence level C), in some individual cases flecainide (plus beta-blockers)	Patients who have survived sudden cardiac death (class I, evidence level C)
Brugada syndrome (consensus report)
Avoid certain drugs No competitive sports Bring down fever early Avoid electrolyte disturbances (hypokalemia, hypomagnesiemia) Carry an emergency card Consider changes to worklife if appropriate	Quinidine in patients with electrical storm (class IIb, evidence level C) Quinidine may also be given prophylactically in the context of clinical studies	Patients who have survived sudden cardiac death (class I, evidence level C) In patients with type 1 ECG and unexplained syncope (class IIa, evidence level C) In patients with type 1 ECG and ventricular tachycardia (class IIa, evidence level C)
Arrhythmogenic right ventricular cardiomyopathy
No competitive sports	Sotalol (class IIa, evidence level C), or amiodarone or beta-blockers	As primary prophylaxis after persistent ventricular tachycardia/ventricular fibrillation despite optimal pharmacotherapy (class I, evidence level B) As primary prophylaxis in patients with extensive structural changes, one or more affected family members, or unexplained syncope despite pharmacotherapy (class IIa, evidence level C)
Dilated cardiomyopathy		(Extract from the indication list)
No competitive sports	ACE inhibitors and beta-blockers (diuretics, aldosterone antagonists, AT1-blockers): treatment for cardiac insufficiency Amiodarone may be given for persistent ventricular tachycardia (class IIb, evidence level C)	Significant LV dysfunction and persistent intolerable VT/VF despite optimal pharmacotherapy (class I, evidence level A) LVEF<35% despite optimal pharmacotherapy in patients with nyha ii–iii (class i, evidence level b) and patients with nyha i (class iib, evidence level b)
Hypertrophic cardiomyopathy
No competitive sports No isometric exercise	Beta-blockers (if contraindicated, in some individual cases calcium antagonists)	In patients with persistent VT/VF despite optimal pharmacotherapy (class I, evidence level B) 1 or more „major risk factors“ despite optimal therapy (class IIa, evidence level C)

^*1Adapted from (10 and 21); LQTS, long QT syndrome; VT, ventricular tachycardia; VF, ventricular fibrillation; LVEF, left-ventricular ejection fraction; LV, left-ventricular