Table 4.

Heart channelopathies.

Disease	Gene (protein)	Pharmacotherapy	Pharmacological perspectives
Long QT syndrome (LQTS)	KCNQ1 (Kv7.1) KCNH2 (HERG) KCNE1 (Mink) KCNE2 (MiRP1) KCNJ2 (Kir2.1) KCNJ5 (Kir3.4) SCN5A (Nav1.5) SCN4B (Nav2.4) CACNA1C (Cav1.2) Non-channel: AKAP9 (yatio) CALM1 (calmodulin) CALM2 (calmodulin) CAV3 (caveolin) SNTA1 (syntrophin a1) ANKB (ankyrin B)	Symptomatic therapy aims at restoring normal heart rhythm: – Implantable cardioverter defibrillator (ICD) – β-adrenoceptor antagonists, such as nadolol and propranolol, plus flecainide. – Mexiletine or ranolazine can be used as add-on therapy in LQT3 – Left cardiac sympathetic denervation (LCSD) when β-blockers fail	Gene- and mutation- specific drugs
Short QT syndrome (SQTS)	KCNH2 (HERG) KCNQ1 (Kv7.1) KCNJ2 (Kir2.1)	– Implantable cardioverter defibrillator (ICD) – Quinidine, a class I antiarrhythmic, in SQT1 owing to HERG channels block
Brugada syndrome (BrS)	SCN5A (Nav1.5) SCN1B (Nav2.1) SCN3B (Nav2.3) KCNE3 (MiRP2) CACNA1C (Cav1.2) CACNB2 (Cavβ2) HCN4 (Hcn4)	Symptomatic therapy aims at restoring normal heart rhythm: – Implantable cardioverter defibrillator (ICD) is the only available option – Quinidine (K channel blocker) or isoproterenol (L-type calcium current activator) are used for acute arrhythmias. Their use as an alternative to ICD remains to be verified	Gene- and mutation-specific therapy
Catecholaminergic polymorphic ventricular tachycardia (CPVT)	RYR2 (RyR2) Non-channel: CASQ2 (calsequestrin-2) CALM1 (calmodulin-1) TRDN (triadin)	Symptomatic therapy aims at restoring normal heart rhythm: – high doses of β-blockers, such as nadolol, plus flecainide – Left cardiac sympathetic denervation (LCSD) where β-blockers fail	– Gene- and mutation- specific drugs – Gene therapy in mouse models