Sudden Arrhythmic Death Syndrome Research Articles

Inherited arrhythmias: considerations for nurses

This article is the third instalment of the British Journal of Cardiac Nursing’s series on cardiac genetic concepts. Inherited arrhythmias are a group of conditions that affect the cardiac electrical system, typically resulting from changes in the way sodium, potassium or calcium ions are handled in the cell. Inherited arrhythmias can cause sudden cardiac death, especially in people who are undiagnosed and untreated. Most inherited arrhythmias are inherited in an autosomal dominant pattern. This article provides an overview of the aetiology, diagnosis, risk-management and treatment of patients with inherited arrhythmias at different ages and stages of life. The focus will be on inherited arrhythmias, specifically long QT, Brugada and catecholaminergic polymorphic ventricular tachycardia. There will be a brief mention of idiopathic ventricular fibrillation and sudden arrhythmic death syndrome.

Effects of mexiletine on a race-specific mutation in Nav1.5 associated with long QT syndrome.

Mutations in cardiac Nav1.5 have been associated with LQT syndrome, Brugada syndrome, and sudden arrhythmia death syndrome. Genetic studies showed that Nav1.5 mutations vary across race-ethnic groups. The Nav1.5 mutation P1090L was linked with familial long QT syndrome (LQTS) and was identified as an ethnic-specific variant in Asians. To explore the role of the P1090L in the LQT syndrome, we characterized the effects of the mutation on the Nav1.5 channel function. We found that the P1090L mutation altered the gating process of the channel and exhibited an enhanced window current. Treatment with mexiletine reversed the depolarization shift of the steady-state inactivation produced by the mutation. Mexiletine modified the recovery and development of inactivation of the mutation and reduced the mutated channel's availability. We characterized that the P1090L is a gain-of-function, the mutation presented a larger window current and increased channel availability. Treatment with mexiletine rescued the dysfunctional inactivation of the mutation. The results might potentially enable a mechanism-based approach to the treatment of LQTS.

A Presentation of Analyses of COVID-19 Vaccine Samples, Blood Samples, Urine Samples, Foot Bath Samples, Sitz Bath Samples, and Skin-Extract Samples

Some members of Korea Veritas Doctors of COVID-19 analyzed COVID-19 vaccines samples, blood samples, urine samples, foot bath samples, sitz bath samples, and skin extract samples from COVID-19 vaccinated and unvaccinated subjects. Among the objects analyzed were self-assembling micro-sized particles, probable Graphene Oxide (GO), and strands of micro-sized materials of diverse structures (probably small GO particles). Intermittently self-vibrating objects (probable GO particles) in the blood of the injected were presented. Slowly moving synthetic parasite-like objects in the blood of the injected and another object of a water ski-like fast moving object in the foot bath water were also presented. COVID-19 vaccines were known to contain programmable mRNA, GO and synthetic parasite-like objects. The effect of these materials in the blood is a widespread damage to everything inside the body including multiple organs. Those damaging effects are linked to endotheliitis, mitochondrial damage, microthrombosis, myocarditis, Sudden Adult Death Syndrome (SADS), human DNA changes and Genetically Modified Organism (GMO), prion disease and dementia, infertility and depopulation, Vaccine Aided Immune Deficiency Syndrome (VAIDS), reprogramming of innate immunity, cancer, RNA Replicons (i.e., replicating spike proteins forever as far as the host lives), and shedding by vaccinated people. Moreover, these materials may cause Vaccine-Induced Thrombotic Thrombocytopenia (VITT), and cytokine storms which is claimed to be typical COVID-19 symptoms. It can be easily argued that there is no other reason for the presence of these main components of COVID-19 vaccines including programmable mRNA, GO, and synthetic parasite-like objects, but to produce slow and painful death in people and to depopulate the world. Considering the hazardous effects of those GOs and other synthetic materials in the human body, human beings need to be detoxified from those hazardous materials and those extracted structures came out of human body need to be carefully treated and detoxified.

Brugada syndrome in Thailand: Three decades of progress.

Our group began investigating the cause of sudden unexplained death syndrome in Thailand in 1994 and found that among sudden unexplained death syndrome patients, the Brugada phenotype was ubiquitous. Following this important observation, Brugada syndrome (BrS) became our main research focus and has galvanized our collaboration with several global prominent scientists over the past 30 years. Through this collaborative research, we made major progress toward better understanding of the syndrome and gained knowledge in genetic background, pathophysiology, and new management. Two consensus reports were published to help define diagnostic criteria, risk stratification, and management of BrS patients. In this review, we share our experiences and progress of our research and development of our program that was designed to identify the cause of sudden death, understand pathophysiology of the syndrome, and develop effective and safe management and therapy of BrS patients. Although our work in Thailand was challenging at the beginning, it later blossomed into a multicollaborative research enterprise that has produced several important findings that have shed light on the pathophysiology of BrS and development of a new effective treatment modality, catheter ablation.

Causes of sudden cardiac death according to age and sex in persons aged 1–49 years

Knowledge of causes of sudden cardiac death (SCD) according to age is important in clinical decision making and to lower the risk of SCD in family members of the deceased. The purpose of this study was to report overall and sex-stratified causes of SCD according to age in persons aged 1-49 years. The study population consisted of all persons in Denmark aged 1-35 years in 2000-2009 and 36-49 years in 2007-2009, which equals 27.1 million person-years. Danish death certificates, discharge summaries, autopsy reports, and data from nationwide registries were used to identify all SCD cases. The SCD cases were divided into 5-year age groups. In the 10-year study period, there were 14,294 deaths, of which 1362 (10%) were classified as SCD. Potentially inherited cardiac disease accounted for a high proportion (43%-78%) of autopsied SCD in all age groups. A significant proportion (19%-54%) of SCD was caused by sudden arrhythmic death syndrome in all age groups. Autopsy rates in both sudden unexpected death cases and SCD cases declined significantly with increasing age (74% in the youngest age group vs 35% in the oldest). The proportion of SCD cases that were identified with a potentially inherited cardiac disease postmortem was high in all studied age groups, while autopsy rates in sudden and unexpected death cases declined markedly with increasing age. Our findings indicate that diagnoses of inherited heart disease are likely missed in some SCD cases, along with the opportunity for treatment and prevention in surviving relatives.

Sudden cardiac death related to physical exercise in the young: a nationwide cohort study of Australia.

Sudden cardiac death (SCD) during physical exercise is devastating. To evaluate causes and circumstances of exercise-related SCD in the young in Australia. We reviewed the National Coronial Information System database for deaths in Australia relating to cardiovascular disease in cases aged 10-35 years between 2000 and 2016. Cases who had undertaken physical exercise at the time of the event were included. We collected demographics, circumstances of death, type of physical exercise, bystander cardiopulmonary resuscitation (CPR) and automated external defibrillator (AED) use prior to ambulance arrival. Over a 17-year period, 1925 SCD cases were identified, of which 110 (6%) cases (median age 27 years (interquartile range 21-32 years); 92% male) were related to sports/physical exercise. Thirteen (12%) cases occurred in active athletes. Most common causes were coronary artery disease (CAD; 37%) and sudden arrhythmic death syndrome (SADS; 20%). Among Aboriginal and Torres Strait Islanders (n = 10), all deaths were related to CAD. Australian Rules Football (24%), running/jogging (14%) and soccer (14%) were the most frequent physical exercise activities. Prior symptoms were present in 39% (chest pain 37%, pre-syncope/syncope 26%). Most (87%) were witnessed, with bystander CPR in 70%. AED use prior to ambulance arrival was 8%. The present study demonstrates the high occurrence of CAD and SADS in SCD in the young related to physical exercise. Aboriginal and Torres Strait Islanders were disproportionately affected by CAD. Although events were commonly witnessed, AED was seldom used prior to ambulance arrival and highlights an important opportunity to improve outcomes in the post-arrest chain of survival.

Abstract P1086: Lrrc10 Is A Multifaceted Modulator Of Cardiac Calcium And Sodium Channels

L-type calcium channels (LTCC) are vital for heart physiology as they allow entry of calcium to the cardiomyocyte, ultimately leading to contraction. These channels are exquisitely modulated by auxiliary subunits and a vast array of regulatory proteins. Heart-specific leucine-rich repeat-containing protein 10 (Lrrc10) plays a key role as a regulator of LTCC. Previous work has shown that Lrrc10 knockdown in zebrafish disrupts development of the cardiac tube, while ablation of Lrrc10 in mice results in heart dysfunction and maladaptation to increased cardiac afterload. Moreover, human mutations in Lrrc10 gene have been linked to dilated cardiomyopathy, and sudden unexplained nocturnal death syndrome (SUNDS). Still, the molecular mechanisms underlying Lrrc10 regulation of LTCC is not fully established. Beyond this, it is unknown whether Lrrc10 modulates other cardiac ion channels. Here, we hypothesized that Lrrc10 serves as a versatile regulator of four-domain voltage-gated ion channels, while mutations in Lrrc10 differentially disrupt channel regulation. Using whole-cell and single-channel electrophysiology, we found that Lrrc10: 1) upregulates LTCC currents by increasing peak open probability; 2) accelerates calcium-dependent inactivation; and 3) evokes a hyperpolarizing-shift in voltage-dependence of activation. Flow cytometric FRET 2-hybrid assay showed that Lrrc10 interacts with LTCC cytoplasmic subunits, specifically the N- and C-termini. We further used FRET 2-hybrid analysis to evaluate the interaction of Lrrc10 with related cardiac ion channels. We observed robust Lrrc10 interaction with Na V 1.5 channels. Multi-channel electrophysiology recordings revealed increased late sodium currents, suggesting that Lrrc10 also modulates Na V 1.5 activity. Additionally, electrophysiological studies showed that Lrrc10 variants linked to dilated cardiomyopathy and SUNDS disparately alter modulation of calcium and sodium channels. In conclusion, Lrrc10 is a multifaceted regulator of four-domain, voltage-gated cardiac ion channels, a finding that will lend critical insights into heart function and disease.

Effects of Mexiletine on a Race-specific Mutation in Nav1.5 Associated With Long QT Syndrome.

The voltage-gated sodium channel Nav1.5 plays an essential role in the generation and propagation of action potential in cardiomyocytes. Mutations in Nav1.5 have been associated with LQT syndrome, Brugada syndrome, and sudden arrhythmia death syndrome. Genetic studies showed that Nav1.5 mutations vary across race-ethnic groups. Here we investigated an Asian-specific mutation Nav1.5-P1090L associated with LQT syndrome. We found that Nav1.5-P1090L mutation perturbed the sodium channel function. It altered the gating process of the channel and exhibited an enhanced window current. Treatment with mexiletine reversed the depolarization shift of the steady-state inactivation produced by P1090L. Mexiletine also modified the recovery from steady-state inactivation and the development of inactivation of P1090L. It rescued the dysfunctional inactivation of P1090L and reduced the P1090L channel’s availability.

Cardiac arrest with successful cardiopulmonary resuscitation and survival induce histologic changes that correlate with survival time and lead to misdiagnosis in sudden arrhythmic death syndrome

BackgroundSudden arrhythmic death syndrome (SADS), defined as sudden cardiac death (SCD) with a morphologically normal heart, is an important cause of sudden death. Hypoperfusion due to cardiac arrest followed by successful cardiopulmonary resuscitation (CPR) may induce histologic changes that mimic pathologic conditions. Detailed characterisation of such features and whether they could confound SADS diagnosis are not described. MethodsRetrospective observational study analysing all consecutive cases of sudden death prospectively referred to a UK national cardiac pathology centre between 2017 and 2021. Cases showing hypoperfusion features were identified after review of clinical information and examination by expert cardiac pathologists. ResultsOut of 2,568 SCD cases, 126 (4.9%) were identified with hypoperfusion changes. Macroscopically, the commonest finding was left ventricular focal or diffuse subendocardial haemorrhage (13.5%). Microscopically, haemorrhage and contraction band necrosis (n = 50, 37.7%), subendocardial acute infarction (n = 44, 34.1%), interstitial mixed inflammatory cell infiltrates (n = 31, 24.9%), healing granulation tissue (n = 9, 7.1%) and subendocardial fibrosis (n = 1, 0.7%) were observed. These changes correlated to duration of survival following resuscitation. In a subcohort of 41 cases, autopsy pathologists misinterpreted such changes as ischaemic myocardial infarction (n = 7; 17%), myocarditis (n = 5; 12.1%), or other pathologies (n = 2; 4.8%) in 14 SADS cases. ConclusionWe provide a comprehensive characterisation of hypoperfusion-related changes in the heart following successful CPR with survival, which are time related. These features can lead to diagnostic confusion among pathologists but knowledge of history of resuscitation with survival should help with general and expert pathology assessment and improve SADS diagnostic yield, prompting genetic screening of decedents’ relatives.

Burden of sudden cardiac death in persons aged 1-40 years in the Czech Republic.

The aim of the study was to ascertain the incidence, circumstances and causes of sudden cardiac death in persons aged 1-40 years in the Czech Republic. De-identified autopsy reports of all individuals who died suddenly between the ages of 1-40 years during the period 2014-2019 inclusive in a selected area of the Czech Republic were analysed retrospectively. Persons with substantial cardiovascular pathology defined by histopathological criteria and those with a negative autopsy were included in the study. The latter were designated as sudden arrhythmic death syndrome. In total, 245 sudden cardiac death cases were identified resulting in an incidence rate of 2.4/100,000 person-years. Among the deceased, we found an enormous gender gap with men representing 81% of cases. More than 80% of deaths occurred during everyday activities or sleep, whereas only 7% were sports-related. The most common cause of death was coronary artery disease detected in 38%, which was followed by cardiomyopathies in 15%, sudden arrhythmic death syndrome in 12%, left ventricular hypertrophy in 10%, and congenital heart defects in 7%. Coronary artery disease is the predominant cause of sudden cardiac death in the young population of the Czech Republic. Hence, effective preventive measures targeted at the reduction of risk factors associated with early coronary artery disease should be reinforced. The second most prevalent cause in our population are potentially heritable heart conditions such as cardiomyopathies and sudden arrhythmic death syndrome. This fact has already prompted the introduction of molecular autopsy and cardiogenetic care for relatives in the Czech Republic.

Gua Sha, An Asian Traditional Therapy That Could Mimic Physical Injury

Gua Sha is a traditional Asian medicine with different names in many Asian countries. It is a treatment to scrape the petechiae of the skin with ointment. It has generally been administered to patients with upper respiratory infections, heat syndrome, and pain. Herein, we report the case of a 31-year-old Cambodian man who was found dead at a farm accommodation. During the autopsy, multiple linear intradermal hemorrhages without subcutaneous damage were observed in the upper chest and both shoulders. The cause of death was sudden unexpected nocturnal death syndrome because he was a young Asian man who died while sleeping without specific findings. Since it is an unfamiliar traditional medicine in Korea compared to other Asian countries, forensic officers without background knowledge about Gua Sha initially suspected that he was severely assaulted or pressed against a particular object enough to cause crush asphyxia. Because of the difficulty of going to the hospital during the coronavirus disease 2019 pandemic, Gua Sha was used to treat headaches and mild fever. Therefore, this case shows that understanding the culture of other countries could be helpful for investigations especially with the increase of multicultural families in Korea.

Novel chemical-physical autopsy investigation in sudden infant death and sudden intrauterine unexplained death syndromes.

Aim: Verify the presence of inorganic nanoparticle entities in brain tissue samples from sudden infant death syndrome (SIDS)/sudden intrauterine unexplained death syndrome (SIUDS) cases. The presence of inorganic debris could be a cofactor that compromises proper brain tissue functionality. Materials & methods: A novel autopsy approach that consists of neuropathological analysis procedures combined with energy dispersive spectroscopy/field emission gun environmental scanning electron microscopy investigations was implemented on 10 SIDS/SIUDS cases, whereas control samples were obtained from 10 cases of fetal/infant deathfrom known cause. Results: Developmental abnormalities of the brain were associated with the presence of foreign bodies. Although nanoparticles were present as well in control samples, they were not associated with histological brain anomalies, as was the case in SIDS/SIUDS. Conclusion: Inorganic particles present in brain tissues demonstrate their ability to cross the hemato-encephalic barrier and to interact with tissues and cells in an unknown yet pathological fashion. This gives a rationale to consider them as cofactors of lethality.

Sport Paradox: Sudden cardiac death

One of the advantages of exercise/physical exercise is improvement of cardiovascular health, however if exercise is done incorrectly, it can lead to negative side-effects that are detrimental to the health of cardiovascular system. One of these side-effects is Sudden Cardiac Death (SDC) or Sprots-related Sudden Cardiac Death (SrSCD). The purpose of this literature review is to find out the cause of SrSCD dan how to prevent it. This Study finds that SrSCD occurs mostly in athletes aged >35 and the majority cause is due to coronary arterial disease (CAD), while in athletes aged <35 the majority cause is due to sudden arrhythmic death syndrome (SADS). SCD can be prevented by doing preparticipation screening, using the “start low, go slow” approach in exercise, following an exercise program that fits to the individual health condition, and management of risk factors.

Clinical Genetics of Inherited Arrhythmogenic Disease in the Pediatric Population.

Sudden death is a rare event in the pediatric population but with a social shock due to its presentation as the first symptom in previously healthy children. Comprehensive autopsy in pediatric cases identify an inconclusive cause in 40–50% of cases. In such cases, a diagnosis of sudden arrhythmic death syndrome is suggested as the main potential cause of death. Molecular autopsy identifies nearly 30% of cases under 16 years of age carrying a pathogenic/potentially pathogenic alteration in genes associated with any inherited arrhythmogenic disease. In the last few years, despite the increasing rate of post-mortem genetic diagnosis, many families still remain without a conclusive genetic cause of the unexpected death. Current challenges in genetic diagnosis are the establishment of a correct genotype–phenotype association between genes and inherited arrhythmogenic disease, as well as the classification of variants of uncertain significance. In this review, we provide an update on the state of the art in the genetic diagnosis of inherited arrhythmogenic disease in the pediatric population. We focus on emerging publications on gene curation for genotype–phenotype associations, cases of genetic overlap and advances in the classification of variants of uncertain significance. Our goal is to facilitate the translation of genetic diagnosis to the clinical area, helping risk stratification, treatment and the genetic counselling of families.

Risk of sudden cardiac death and preventive measures in athletes

Arrhythmias, which are fatal in some patients, can be triggered by sports in vulnerable people. It is estimated that 1:40,000–1:250,000 athletes will suffer a sudden cardiac death (SCD). However, female athletes appear to have some level of cardiac protection, since suffering from SCD considerably less than male athletes during sports. Athletes with underlying coronary, valvular or myocardial disease, as well as channelopathies, may be particularly prone to SCD from exercise- and sports-related physical activity. There are three main causes of SCD in young athletes: Sudden Arrhythmic Death Syndrome (56%), congenital anomalous coronary arteries (7%–14%), and hypertrophic cardiomyopathy (36%–48%). In the context of exercise, acute ischemia, myocardial infarction, and stroke risk are increased by catecholamine surge and exercise-induced stress. In middle-aged athletes, excessive cardiovascular activity is associated with a higher risk of mortality related to cardiovascular disease. It is possible to detect at-risk athletes by conducting cardiac screening, which involves a family history, physical examination, and a resting electrocardiogram. Consequently, efforts have been made to better understand the causes of SCD in athletes and to develop appropriate prevention methods.

The evidence for the implantable loop recorder in patients with inherited arrhythmia syndromes: a review of the literature.

Risk stratification of patients with inherited arrhythmia syndromes (IASs) can be challenging. Recent guidelines acknowledge a place for considering the implantable loop recorder (ILR) to outrule malignant arrhythmia as a cause of syncope in certain inherited arrhythmia patients who are at low risk of sudden cardiac death. In this comprehensive literature review, we evaluate the available evidence for the use of the ILR in the IASs and in relatives of victims of sudden arrhythmic death syndrome.

Elicitation of children's understanding of information in pediatric genetic counseling encounters: A discourse-oriented perspective.

Affirmation of children's understanding of information provided in genetic counseling encounters is crucial to obtaining children's informed consent/assent in pediatric genetic counseling encounters. It is also important for the proper management of a genetic condition. Currently, there is a relative scarcity of research on how understanding of complex genetic information by children is elicited in the process of pediatric genetic counseling. In this study, we apply theme-oriented discourse analysis to examine 23 video/audio-recorded genetic counseling encounters in Hong Kong. The encounters involve children aged between 3 and 17years old who are suspected to have or diagnosed with Sudden Arrhythmic Death Syndrome (SADS). Specifically, we examine a range of communicative strategies that genetic professionals employ to elicit children's understanding of information in this genetic counseling setting. We also examine how children's epistemic status is negotiated between genetic professionals, parents, and children. The study reveals that genetic professionals typically use direct questioning (e.g., "do you understand?" or "do you have any questions?"). Less typical are examples where genetic professionals explore children's epistemic access and invite children to recall information after they deliver it. The study reveals two discourse strategies that genetic professionals and parents employ to justify a child's low epistemic status: (1) construction of "current ignorance" and "future competence" in children and (2) association with a child's character. In the examined counseling encounters, genetic professionals and parents tend to construct a low epistemic status in younger children and allocate the responsibility for understanding relevant information to the parents and the "future" competent children. The study highlights the impact of genetic professionals' and parents' assumptions on children's knowledge and comprehensibility at different ages, and the role that children themselves play in conforming or contesting these assumptions.

The role of ILR monitoring in patients with a family history of SADS, a view from real clinical practice

Abstract Background/Introduction Implantable Loop Recorder (ILR) device monitoring is an established method for long term heart rhythm monitoring in patients with inherited cardiac conditions. Many present with a family history of Sudden Arrhythmic Death Syndrome (SADS). The value of ILR findings in the investigation of SADS relatives has not been well documented. Purpose We aimed to evaluate the impact of ILR monitoring on the management plans of patients with a family history of SADS. Methods We performed a retrospective analysis of the ILR reports and electronic patient records of all patients at the inherited cardiac disease clinic with a family history of SADS and an ILR implanted. Patient demographics, ILR implant indication and specific changes to management plans were recorded and analysed using descriptive statistics. Results All 135 patients with ILR monitoring at the inherited cardiac disease clinic were screened and 87 patients (57.6% female, 41.7±14.0 years) with SADS relatives were included in the study. The mean follow up period was 657.9±392.3 days from ILR implant. Indications for ILR implantation included syncope (n=31, 15.7%), presyncope (45, 22.7%), palpitations (44, 22.2%), chest pain (9, 4.5%), short term heart rhythm monitor findings (6, 3.0%), ECG findings (6, 3.0%), asymptomatic indications (10, (5.1%) including patients with more than one relative with SADS, a family history of conduction disease or family history of long QT syndrome), and atypical symptoms (2 (1%) including seizures and sleep paralysis). Some patients had more than one indication for ILR at the time of implant. As a direct result of ILR monitoring, 43 (49.4%) patients had a change to their management plan. 6 specific definitions for management changes were used: Permanent pacemaker implantation (2, 2.3%), subsequent electrophysiology study (3, 3.5%), medication change (7, 8.1%), arrhythmia excluded as a cause for patient symptoms (26, 29.9%), prompted ILR implant in first degree relative (11, 12.6%) and ILR re-implant for further monitoring for premature conduction disease (1, 1.2%). Patients whose indication for ILR implant was palpitations had the highest likelihood for change of management with 27 changes associated with this indication, of which exclusion of arrhythmia as a cause for symptoms (15) was the most frequent outcome. The indications, syncope and presyncope both yield 21 management changes each. Conclusion The use of ILR devices in family relatives of patients with SADS provides information that may directly impact on patient management, with syncope providing the highest yield and reassurance the most common outcome in our cohort. ILR monitoring helped guide a wide range of other management strategies which included changes to medications and the need for further cardiac procedures. This data represented clinical practice in a niche patient cohort who are at risk for inherited cardiac conditions and associated arrhythmias. Funding Acknowledgement Type of funding sources: None. Indication for ILR vs management changeIndications for ILR implant

Radiofrequency ablation in Brugada syndrome.

Radiofrequency ablation in Brugada syndrome.

Unique clinical features and long term follow up of survivors of sudden cardiac death in an Asian multicenter study

There has been no long-term clinical follow-up data of survivors or victims of sudden cardiac death (SCD). The Taiwan multi-center sudden arrhythmia death syndrome follow-up and clinical study (TFS-SADS) is a collaborative multi-center study with median follow-up time 43 months. In this cohort, the clinical characteristics of these SADS patients were compared with those with ischemic heart disease (IHD). In this SCD cohort, around half (42%) were patients with IHD, which was different from Caucasian SCD cohorts. Among those with normal heart, most had Brugada syndrome (BrS). Compared to those with SADS, patients with IHD were older, more males and more comorbidities, more arrhythmic death, and lower left ventricular ejection fraction. In the long-term follow-up, patients with SADS had a better survival than those with IHD (p < 0.001). In the Cox regression analysis to identify the independent predictors of mortality, older age, lower LVEF, prior myocardial infarction and history of out-of-hospital cardiac arrest were associated with higher mortality and beta blocker use and idiopathic ventricular fibrillation or tachycardia (IVF/IVT) with a better survival during follow-up. History of prior MI was associated with more arrhythmic death. Several distinct features of SCD were found in the Asia–Pacific region, such as higher proportion of SADS, poorer prognosis of LQTS and better prognosis of IVF/IVT. Patients with SADS had a better survival than those with IHD. For those with SADS, patients with channelopathy had a better survival than those with cardiomyopathy.