Remote monitoring of cardiac risk in Andersen-Tawil syndrome

<h3>Background</h3> Andersen-Tawil Syndrome (ATS) is a rare channelopathy typically characterised by periodic paralysis, cardiac arrhythmias and dysmorphic features caused by mutations in the KCNJ2 gene. There is a significant risk of cardiac morbidity and mortality making regular cardiac monitoring a crucial aspect of clinical assessment. Conventional methods are limited by short monitoring times, discomfort or invasive procedures and their complications. New technologies may overcome these limitations. <h3>Aims</h3> To determine if remote, hand-held, monitoring devices are useful for detecting cardiac arrhythmias and ECG changes in patients with ATS. <h3>Methods</h3> Suitable patients with ATS who attend the Skeletal Muscle Channelopathy clinic in our centre were given KardiaMobile devices (KMD). During the 8-week study period, patients were asked to record an ECG at home using the KMD while asymptomatic once per week and during any cardiac symptoms. Quantitative and qualitative analysis of all ECGs were completed. <h3>Results</h3> 11 patients completed the study, 6 had a severe cardiac phenotype. Average baseline QTc was prolonged. 18% of asymptomatic ECGs recorded arrythmias. 7 symptomatic ECGs were returned with relevant abnormalities, however with some traces significantly obscured by artefact. <h3>Conclusion</h3> Remote, handheld cardiac monitoring devices may be useful in detecting prolonged QTc and asymptomatic arrythmias in patients with ATS. However, techniques/training to overcome artefactual symptomatic readings need to be considered and larger studies with direct comparison to conventional cardiac monitoring methods are needed.