Bidirectional Ventricular Tachycardia in Familial Hypokalasmic Periodic Paralysis

Wa, Stubbs

doi:10.1177/003591577606900319

Cited by 16 publications

(8 citation statements)

References 4 publications

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“…Although uncommon, it has fascinated clinicians since its original description in 1922 as a manifestation of digitalis toxicity 1–4. It has also been reported in the setting of hypokalemic periodic paralysis 5, Andersen-Tawil syndrome 6, and fulminant myocarditis 7. More recently, BVT has also been recognized as a hallmark of catecholaminergic polymorphic ventricular tachycardia (CPVT) syndrome, a familial disorder with a high risk of sudden cardiac death during sympathetic stimulation 8.…”

Section: Introductionmentioning

confidence: 99%

Bidirectional ventricular tachycardia: Ping pong in the His–Purkinje system

et al. 2011

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Background-Bidirectional ventricular tachycardia (BVT), characterized by an alternating beatto-beat electrocardiographic QRS axis, is a rare but intriguing arrhythmia associated with digitalis toxicity, familial catecholaminergic polymorphic ventricular tachycardia (CPVT), and several other conditions which predispose cardiac myocytes to delayed afterdepolarizations (DADs) and triggered activity. Evidence from human and animal studies attributes BVT to alternating ectopic foci originating from the distal His-Purkinje system in the left and/or right ventricles, respectively.

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Section: Introductionmentioning

confidence: 99%

Bidirectional ventricular tachycardia: Ping pong in the His–Purkinje system

et al. 2011

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“…The first search yielded 58 articles, of which 11 reported HypoPP patients with cardiac arrhythmias . The second search yielded 720 articles and provided 5 additional HypoPP family or cohort studies with brief reports on cardiac arrhythmia (Fig.…”

Section: Resultsmentioning

confidence: 99%

“…Among the patients within the group of cardiac complications that occurred between attacks of paralysis and had minor hypokalemia, 3 of 4 may have had ATS. They were genetically undetermined, had frequent syncope, and showed bidirectional ventricular tachycardia (BVT) as the most prominent hallmark …”

Section: Discussionmentioning

confidence: 99%

Cardiac arrhythmias in hypokalemic periodic paralysis: Hypokalemia as only cause?

Stunnenberg

Deinum

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et al. 2014

Muscle and Nerve

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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.

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“…All KCNJ2 affected family members showed dysmorphic features characteristic for ATS. All had short stature (female 149–161 cm, −2.5 to −1 standard deviations (SD) expressed in growth charts, male 121–171 cm, −2.5 to −1 SD). Genopositive individuals also had small mandibles, dental engagement with enamel hypoplasia, and were prone to caries.…”

Section: Resultsmentioning

confidence: 99%