BACKGROUND : Loss-of-function mutations in SCN5A are present in ~20％ of Brugada syndrome（BrS）patients. Copy number variations（CNVs）have been shown to be associated with several inherited arrhythmia syndromes. OBJECTIVE : The purpose of this study was to investigate SCN5A CNVs among BrS probands.METHODS : The study cohort consisted of 151 BrS probands who were symptomatic or had a family history of BrS, sudden death, syncope, or arrhythmic diseases. We performed sequence analysis of SCN5A by the Sanger method. To detect CNVs in SCN5A, we performed multiplex ligation-dependent probe amplification analysis of the 151 BrS probands. RESULTS : We identified pathogenic SCN5A mutations in 20 probands by the Sanger method. In 140 probands in whom multiplex ligation-dependent probe amplification was successfully performed, 4 probands were found to present different CNVs（deletion in 3 and duplication in 1）. Three of them had fatal arrhythmia events ; the remaining 1 was asymptomatic but had a family history. Mean age at diagnosis was 23 ± 14 years. All of the baseline 12-lead electrocardiograms showed PQ-interval prolongation. The characteristics of these 4 probands with CNVs were similar to those of the probands with mutations leading to premature truncation of the protein or missense mutations causing peak I_Na reduction＞90％. CONCLUSION : We identified SCN5A CNVs in 2.9％ of BrS probands who were symptomatic or had a family history.

Cardiac Resynchronization Therapy（CRT）is the therapeutic option for patients with advanced heart failure. We can detect characteristic left ventricular abnormal movements called “septal flash” and “apical shuffle motion” as a visual feature of dyssynchrony by echocardiography, and CRT can be effective for patients with such findings suggesting dyssynchrony. Such findings detected by echocardiography can give us information on potential candidates for CRT. We can also evaluate the myocardium itself in the posterior and lateral walls where the left ventricular lead is usually fixed, and it can be inferred that myocardial scar in lead-positioned site has poor effect for CRT. Therefore, echocardiography can help us decide lead position. It can also be used to evaluate the disappearance of dyssynchronization by evaluating left ventricular size / volume and, contractility ability after CRT. Assessment of right-side cardiac function is also important, because deterioration of tricuspid annular plane systolic excursion（TAPSE）and the presence of pulmonary hypertension predict poor prognosis after CRT. In this article, we explain the importance of and some points of evaluation in echocardiography before and after CRT in the clinical setting.

The remote monitoring（RM）-related workload of clinical engineers is increasing with the rise in RM management of patients with cardiac implantable electrophysiological devices. As a result the workload related to RM needs to be reduced safely and efficiently. In addition, because several clinical engineers are involved in RM-related work, a standard operating procedure（SOP）is required to perform identical work. We started RM management using an SOP in 2016. After that, we investigated workload changes between 1 year before（August 2015 to July 2016）and 1 year after（August 2016 to July 2017）SOP introduction. Along with an increase in patients with RM management（109–136 ; increase rate, 1.25 times）, the total number of alerts also increased（2,002–2,367 per year ; 1.18 times）. However, the number of alerts decreased relatively compared the increase in the number of patients. Alerts related to atrial fibrillation decreased from 244 to 60 per year. The RM-related workload of clinical engineers can be safely and efficiently reduced using the SOP approach.