Resuscitated sudden cardiac death in Andersen-Tawil syndrome

Recurrent syncope in the Andersen Tawil syndrome – Cardiac or neurological?

“…The majority of these occurred either at the onset of or during sleep, consistent with a hypothesis generated in a previous case report [2] , that autonomic instability might precipitate haemodynamically compromising ventricular arrhythmias in patients with Andersen-Tawil syndrome. The device was reprogrammed to allow a longer delay before shock delivery resulting in long self terminating runs of polymorphic ventricular tachycardia associated with pre-syncope.…”

“…The inheritance of ATS is autosomal dominant, though there is incomplete penetrance [2] . Approximately 20% of those with a KCNJ2 mutation are asymptomatic.…”

Andersen–Tawil syndrome: Report of 3 novel mutations and high risk of symptomatic cardiac involvement

“…The clinical spectrum included clinically and electrocardiographically asymptomatic subjects (25% of observed cases) and also 2 SCD survivors. Although only a few cases of sudden cardiac death in ATS have been reported previously, a 45‐year‐old uncle of our proband had fatal SCD. ICDs were implanted in 2 SCD survivors and in another 4 of our patients with recurrent syncope and complex ventricular arrhythmia for primary SCD prevention.…”

Kir 2.1 channelopathies: the Andersen–Tawil syndrome

“…Twentyfour-hour ambulatory ECG monitoring enables a determination of the tachycardia burden and provides a baseline comparison for the assessment of the efficacy of antiarrhythmic therapy. Our recent recognition that vasovagal syncope may be a harbinger of a more worrisome clinical ATS phenotype [2] and the previous documentation of torsades de pointes [33] and cardiac arrest in this population [2,3,6] enforces the need for a comprehensive cardiac evaluation and ongoing cardiac monitoring in ATS patients.…”