title: Cardiac arrhythmias in hypokalemic periodic paralysis: Hypokalemia as only cause?
abstract: It is unknown how often cardiac arrhythmias occur in hypokalemic periodic paralysis (HypoPP) and if they are caused by hypokalemia alone or other factors. This systematic review shows that cardiac arrhythmias were reported in 27 HypoPP patients. Cases were confirmed genetically (13 with an R528H mutation in CACNA1S, 1 an R669H mutation in SCN4A) or had a convincing clinical diagnosis of HypoPP (13 genetically undetermined) if reported prior to the availability of genetic testing. Arrhythmias occurred during severe hypokalemia (11 patients), between attacks at normokalemia (4 patients), were treatment-dependent (2 patients), or unspecified (10 patients). Nine patients died from arrhythmia. Convincing evidence for a pro-arrhythmogenic factor other than hypokalemia is still lacking. The role of cardiac expression of defective skeletal muscle channels in the heart of HypoPP patients remains unclear. Clinicians should be aware of and prevent treatment-induced cardiac arrhythmia in HypoPP.
authors.affiliation: Donders Institute for Brain, Cognition and Behaviour, Department of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Bas C
authors.initials: BC
authors.lastname: Stunnenberg
authors.forename: Jaap
authors.initials: J
authors.lastname: Deinum
authors.forename: Thera P
authors.initials: TP
authors.lastname: Links
authors.forename: Arthur A
authors.initials: AA
authors.lastname: Wilde
authors.forename: Hessel
authors.initials: H
authors.lastname: Franssen
authors.forename: Gea
authors.initials: G
authors.lastname: Drost
mesh_terms: D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology* / Q000235:genetics; D015220:Calcium Channels / Q000235:genetics; D020746:Calcium Channels, L-Type; D002423:Cause of Death; D002648:Child; D003422:Critical Care; D004562:Electrocardiography; D005260:Female; D006801:Humans; D007008:Hypokalemia / Q000150:complications* / Q000235:genetics; D020514:Hypokalemic Periodic Paralysis / Q000150:complications* / Q000235:genetics; D008297:Male; D018908:Muscle Weakness / Q000209:etiology; D009154:Mutation; D062553:NAV1.4 Voltage-Gated Sodium Channel / Q000235:genetics; D010375:Pedigree; D055815:Young Adult
chemical_list: C412223:CACNA1S protein, human; D015220:Calcium Channels; D020746:Calcium Channels, L-Type; D062553:NAV1.4 Voltage-Gated Sodium Channel; C106251:SCN4A protein, human
keywords: CACNA1S; HypoPP; SCN4A; cardiac arrhythmias; hypokalemia; hypokalemic periodic paralysis