scholarly journals Risk-Stratification Strategy for Sudden Cardiac Death in the Very Young Children with Asymptomatic Ventricular Preexcitation

2020 ◽  
Vol 16 (2) ◽  
pp. 83-89
Author(s):  
Moises Rodriguez-Gonzalez ◽  
Ana Castellano-Martinez ◽  
Alvaro A. Perez-Reviriego

Asymptomatic VPE refers to the presence of this abnormal ECG pattern in the absence of any symptoms. The natural history in these patients is usually benign, and most children (60%) with VPE are usually asymptomatic. However, Sudden Cardiac Death (SCD) has been reported to be the initial symptom in many patients too. The increased risk of SCD is thought to be due to the rapid conduction of atrial arrhythmias to the ventricle, via the AP, which degenerates into Ventricular Fibrillation (VF). The best method to identify high-risk patients with asymptomatic VPE for SCD is the characterization of the electrophysiological properties of the AP through an Electrophysiological Study (EPS). Also, catheter ablation of the AP with radiofrequency as definitive treatment to avoid SCD can be performed by the same procedure with high rates of success. However, the uncertainty over the absolute risk of SCD, the poor positive predictive value of an invasive EPS, and complications associated with catheter ablation have made the management of asymptomatic VPE challenging, even more in those children younger than 8-year-old, where there are no clear recommendations. This review provides an overview of the different methods to make the risk stratification for SCD in asymptomatic children with, as well as our viewpoint on the adequate approach to those young children not included in current guidelines.

Author(s):  
Elena Okisheva ◽  
Dmitry Tsaregorodtsev ◽  
Vitaly Sulimov

<p>Objectives: To evaluate predictive value of heart rate turbulence (HRT), deceleration capacity (DC) and microvolt T-wave alternans (mTWA) for risk stratification for sudden cardiac death (SCD) in patients after myocardial infarction (MI) during 60 months of follow-up.</p><p>Methods: We studied 111 patients after MI occurred &gt; 60 days (27 [9; 84] months) before enrollment (84 men; mean age 64.1±10.5 years). All subjects had 24-hour ambulatory ECG monitoring with HRT, DC and mTWA evaluation. Follow-up period was 60 months; primary endpoint was SCD, secondary endpoint included all non-sudden cardiovascular deaths.</p><p>Results: During follow-up, we registered 19 cases of SCD and 11 cases of non-sudden cardiovascular deaths (including 7 fatal MI and 3 fatal strokes). DC had high negative predictive value (97.4% for all-cause mortality and 93.7% for SCD). DC values below 4.15 for all-cause mortality and 2.0 for SCD significantly increased risk of all-cause mortality (OR 8.5, 95% CI 2.9 to 24.6, р&lt;0.001) и SCD (OR 9.6, 95% CI 3.2 to 28.5, р&lt;0.001). Combined risk assessment at 12 months revealed that the most significant combination was HRT2 and mTWA100 &gt; 53 mcV, which increased risk both of all-cause mortality (30.7-fold) and SCD (63.3-fold); however, at 60 months this predictive value for SCD decreased (OR = 20.8 (95% CI 2.8 to 114.0), p &lt;0.001).</p>Conclusion: Evaluation of HRT, DC and mTWA during 24-hour ECG monitoring may define the high risk of cardiovascular mortality and SCD in post-MI patients especially during the first 12 months after the baseline examination.


ESC CardioMed ◽  
2018 ◽  
pp. 2305-2308
Author(s):  
Efstathios K. Iliodromitis ◽  
Dimitrios Farmakis

There are three main groups in the general population as far as sudden cardiac death (SCD) is concerned: individuals without a known history or predisposing factors for heart disease; individuals with known risk factors for heart disease or SCD; and patients with diagnosed ischaemic, structural, or electrical cardiac conditions, acquired or genetic, that are associated with an increased risk for SCD. Although SCD literature focuses mainly on patients with known heart disease, approximately 50% of SCD cases occur in individuals belonging to the first two groups. The annual incidence of SCD in the general population ranges between 0.6 and greater than 1.4 per 1000 individuals. SCD occurs more commonly in men than in women and with an incidence that increases with age due to the increase in coronary artery disease. The commonest aetiologies for SCD in the general population are coronary artery disease and cardiomyopathy, accounting for 80% and 10–15% of cases, respectively. A number of factors have been related to an increased risk for SCD in the general population including genetic predisposition, risk factors for atherosclerosis, strenuous physical activity and sports, electrocardiographic abnormalities, elevated levels of biomarkers, and abnormalities in imaging and other diagnostic techniques. However, large-scale prospective studies that confirm the feasibility, clinical efficacy, and cost-effectiveness of using these factors for broad mass screening for SCD are generally lacking and therefore risk stratification for SCD in the general population remains challenging.


2019 ◽  
Vol 21 (12) ◽  
Author(s):  
M. Akhtar ◽  
P. M. Elliott

Abstract Purpose of Review Non-ischaemic dilated cardiomyopathy (DCM) occurs in 1 in 2500 individuals in the general population and is associated with considerable morbidity and mortality. Studies involving large numbers of unselected DCM patients have led to consensus guidelines recommending implantable cardioverter-defibrillator (ICD) implantation for protection against sudden cardiac death (SCD) in those with LVEF ≤35%. The purpose of this article is to review the literature for other potential markers including serological, electrocardiographic, echocardiographic, cardiac magnetic resonance, ambulatory ECG and genetic data, to highlight other potential markers that may optimise risk stratification for SCD in this cohort and thereby allow a more personalized approach to ICD-implantation. Recent Findings Recent studies including the Danish study to assess the efficacy of ICDs in patients with non-ischemic systolic heart failure on mortality (DANISH) trial have questioned the benefits of ICD implantation in this group of patients with no changes in all-cause mortality. Recent pooled cohorts of patients with genetic DCM and in particular in those with Lamin A/C (LMNA) mutations have identified patients at increased risk of SCD and allowed the creation of algorithms to prognosticate SCD risk in mutation carriers. Furthermore, genetic testing has identified other DCM-causing genes including filamin C (FLNC) and RBM20 which may be associated with higher rates of ventricular arrhythmia. Summary To date, risk-stratification for SCD has been hampered by the utilisation of heterogenous subsets of idiopathic DCM patients and by use of static risk models where predictions are based on a single time point with a lack of consideration of disease progression. The current focus of personalised risk-stratification for SCD is shifting towards better characterisation of underlying DCM aetiology and the development of multi-parametric risk-stratification models that incorporate time-dependent disease characteristics and novel biomarkers.


2021 ◽  
Vol 22 (11) ◽  
pp. 5688
Author(s):  
Larisa Anghel ◽  
Radu Sascău ◽  
Ioana Mădălina Zota ◽  
Cristian Stătescu

Non-ischemic dilated cardiomyopathy encompasses a wide spectrum of myocardial disorders, characterized by left ventricular dilatation with systolic impairment and increased risk of sudden cardiac death. In spite of all the therapeutic progress that has been made in recent years, dilated cardiomyopathy continues to be an important cause of cardiac transplant, being associated with an enormous cost burden for health care systems worldwide. Predicting the prognosis of patients with dilated cardiomyopathy is essential to individualize treatment. Late gadolinium enhancement-cardiac magnetic resonance imaging, microvolt T-wave alternans, and genetic testing have emerged as powerful tools in predicting sudden cardiac death occurrence and maximizing patient’s selection. Despite all these new diagnostic modalities, additional tests to complement or replace current tools are required for better risk stratification. Therefore, biomarkers are an easy and important tool that can help to detect patients at risk of adverse cardiovascular events. Additionally, identifying potential biomarkers involved in dilated cardiomyopathy can provide us important information regarding the diagnostic, prognostic, risk stratification, and response to treatment for these patients. Many potential biomarkers have been studied in patients with dilated cardiomyopathy, but only a few have been adopted in current practice. Therefore, the aim of our review is to provide the clinicians with an update on the well-known and novel biomarkers that can be useful for risk stratification of patients with non-ischemic dilated cardiomyopathy.


2018 ◽  
Vol 7 (2) ◽  
pp. 79 ◽  
Author(s):  
Shohreh Honarbakhsh ◽  
Rui Providencia ◽  
Pier D Lambiase ◽  
◽  
◽  
...  

Brugada syndrome (BrS) is one of the most common inherited channelopathies associated with an increased risk of sudden cardiac death. Appropriate use of an ICD in high-risk patients is life-saving. However, there remains a lack of consensus on risk stratification, and even on the diagnosis of BrS itself. Some argue that people with a type 1 Brugada ECG pattern but no symptoms should not be diagnosed with BrS, and guidelines recommend observation without therapy in these patients. Others argue that the presence of a spontaneous (rather than drug-induced) type 1 ECG pattern alone is enough to label them as high-risk for arrhythmic events, particularly if syncope is also present. Syncope and a spontaneous type 1 ECG pattern are the only factors that have consistently been shown to predict ventricular arrhythmic events and sudden cardiac death. Other markers have yielded conflicting data. However, in combination they may have roles in risk scoring models. Epicardial catheter ablation in the right ventricular outflow tract has shown promise in studies as an alternative management option to an ICD, but longer follow-up is required to ensure that the ablation effect is permanent.


2021 ◽  
Vol 6 (1) ◽  
Author(s):  
Rameen Shakur ◽  
Juan Pablo Ochoa ◽  
Alan J. Robinson ◽  
Abhishek Niroula ◽  
Aneesh Chandran ◽  
...  

AbstractThe cardiac troponin T variations have often been used as an example of the application of clinical genotyping for prognostication and risk stratification measures for the management of patients with a family history of sudden cardiac death or familial cardiomyopathy. Given the disparity in patient outcomes and therapy options, we investigated the impact of variations on the intermolecular interactions across the thin filament complex as an example of an unbiased systems biology method to better define clinical prognosis to aid future management options. We present a novel unbiased dynamic model to define and analyse the functional, structural and physico-chemical consequences of genetic variations among the troponins. This was subsequently integrated with clinical data from accessible global multi-centre systematic reviews of familial cardiomyopathy cases from 106 articles of the literature: 136 disease-causing variations pertaining to 981 global clinical cases. Troponin T variations showed distinct pathogenic hotspots for dilated and hypertrophic cardiomyopathies; considering the causes of cardiovascular death separately, there was a worse survival in terms of sudden cardiac death for patients with a variation at regions 90–129 and 130–179 when compared to amino acids 1–89 and 200–288. Our data support variations among 90–130 as being a hotspot for sudden cardiac death and the region 131–179 for heart failure death/transplantation outcomes wherein the most common phenotype was dilated cardiomyopathy. Survival analysis into regions of high risk (regions 90–129 and 130–180) and low risk (regions 1–89 and 200–288) was significant for sudden cardiac death (p = 0.011) and for heart failure death/transplant (p = 0.028). Our integrative genomic, structural, model from genotype to clinical data integration has implications for enhancing clinical genomics methodologies to improve risk stratification.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
N Soroush ◽  
A Aarnoudse ◽  
F Shokri ◽  
M Van Den Berg ◽  
F Ahmadizar ◽  
...  

Abstract Background Digoxin is one of the oldest cardiovascular medications still used to treat heart failure and atrial fibrillation. Due to its narrow therapeutic window, it is associated with life threatening intoxication and arrhythmias, and with QTc-shortening. Common genetic variation in the nitric oxide synthase-1 adaptor protein (NOS1AP) has been associated with QTc interval prolongation. Purpose We investigated whether the rs10494366 variant of the NOS1AP gene modified the risk of SCD in patients using digoxin. Methods In a prospective population-based cohort study, we included data of the three cohorts, started as of January 1st, 1991 until January 1st 2014. Digoxin current use on the date of cardiac death in cases and the same day of follow-up in the remainder of the cohort was a time-dependent exposure. The main outcome was SCD defined as sudden and unexpected death as a result of cardiac causes, according to international criteria. Identification and adjudication of SCD was performed independently, before the start of this study. We used Cox proportional hazard regression analysis to investigate the associations between NOS1AP rs10494366 variant and incident SCD among digoxin users compared to non-users. Associations were adjusted for age, sex (model 1) in addition to BMI, prevalent diabetes, myocardial infarction, baseline hypertension and smoking status (past, current, never) (model 2). Results We included 14,594 individuals, with a mean age of 65.3 (SD 10.3) years. Almost 59% were female. The cumulative incidence of SCD was 9.5% (609 cases) by the end of follow up. Among them, 98 (16%) individuals were exposed to digoxin at the time of death. In model 1, NOS1AP rs10494366 variant was not associated with SCD in the total study population. However, an interaction term of the gene with the daily dose of digoxin was significantly associated with increased risk of SCD (p-value 0.0001). In model 2, the risk of SCD in current users of digoxin was 4.2 [95% CI 1.3–13.8] for the GG genotype; 2.1 [95% CI 1.1–4.2] for the GT genotype, and 1.5 [95% CI 0.7–3.2] for the TT genotype. Conclusion NOS1AP rs10494366 variant modified the risk of sudden cardiac death in users of digoxin. Our study suggests that individuals with the homozygous minor GG allele have a fourfold increased risk of sudden cardiac death. Funding Acknowledgement Type of funding source: None


Heart ◽  
2020 ◽  
pp. heartjnl-2020-317701
Author(s):  
Guixin Wu ◽  
Jie Liu ◽  
Shuiyun Wang ◽  
Shiqin Yu ◽  
Ce Zhang ◽  
...  

ObjectiveElevated levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) are associated with heart failure-related death in hypertrophic cardiomyopathy (HCM), but the relationship between NT-proBNP level and sudden cardiac death (SCD) in HCM remains undefined.MethodsThe study prospectively enrolled 977 unrelated patients with HCM with available NT-proBNP results who were prospectively enrolled and followed for 3.0±2.1 years. The Harrell’s C-statistic under the receiver operating characteristic curve was calculated to evaluate discrimination performance. A combination model was constructed by adding NT-proBNP tertiles to the HCM Risk-SCD model. The correlation between log NT-proBNP level and cardiac fibrosis as measured by late gadolinium enhancement (LGE) or Masson’s staining was analysed.ResultsDuring follow-up, 29 patients had SCD. Increased log NT-proBNP levels were associated with an increased risk of SCD events (adjusted HR 22.27, 95% CI 10.93 to 65.63, p<0.001). The C-statistic of NT-proBNP in predicting SCD events was 0.80 (p<0.001). The combined model significantly improved the predictive efficiency of the HCM Risk-SCD model from 0.72 to 0.81 (p<0.05), with a relative integrated discrimination improvement of 0.002 (p<0.001) and net reclassification improvement of 0.67 (p<0.001). Furthermore, log NT-proBNP levels were significantly correlated with cardiac fibrosis as detected either by LGE (r=0.257, p<0.001) or by Masson’s trichrome staining in the myocardium (r=0.198, p<0.05).ConclusionNT-proBNP is an independent predictor of SCD in patients with HCM and may help with risk stratification of this disease.


Author(s):  
Marianna Leopoulou ◽  
Jo Ann LeQuang ◽  
Joseph V. Pergolizzi ◽  
Peter Magnusson

Dilated cardiomyopathy (DCM) is characterized by the phenotype of a dilated left ventricle with systolic dysfunction. It is classified as hereditary when it is deemed of genetic origin; more than 50 genes are reported to be related to the condition. Symptoms include, among others, dyspnea, fatigue, arrhythmias, and syncope. Unfortunately, sudden cardiac death may be the first manifestation of the disease. Risk stratification regarding sudden death in hereditary DCM as well as preventive management poses a challenge due to the heterogeneity of the disease. The purpose of this chapter is to present the epidemiology, risk stratification, and preventive strategies of sudden cardiac death in hereditary DCM.


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