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The purpose of this series is to highlight a range of rare health conditions. Rare health conditions are those that affect no more and usually fewer than 1 person in every 2000. Many healthcare assistants and nurses will encounter some of these conditions, given the high number of them. This 49th article will explore three of these conditions: sudden arrhythmic death syndrome (SADS); sudden infant death syndrome (SIDS); and Hansen's disease.

Patients with tetralogy of Fallot are at risk for ventricular arrhythmias and sudden cardiac death. These abnormalities are associated with pulmonary regurgitation, right ventricular enlargement, and a substrate of discrete, slowly-conducting isthmuses. Although these arrhythmic events are rare, their prediction is challenging. This review will address contemporary risk assessment and prevention strategies. Numerous variables have been proposed to predict who would benefit from an implantable cardioverter-defibrillator. Current risk stratification models combine independently associated factors into risk scores. Cardiac magnetic resonance imaging, QRS fragmentation assessment, and electrophysiology testing in selected patients may refine some of these models. Interaction between right and left ventricular function is emerging as a critical factor in our understanding of disease progression and risk assessment. Multicenter studies evaluating risk factors and risk mitigating strategies such as pulmonary valve replacement, ablative strategies, and use of implantable cardiac-defibrillators are needed moving forward.

Recent publications have raised serious concerns regarding the safety of digoxin for atrial fibrillation (AF). However, the subgroup of patients with reduced ejection fraction and AF have been speculated to derive clinical benefit from digoxin. We aimed to assess the impact of digoxin on mortality and cardiovascular hospitalizations in the Atrial Fibrillation and Congestive Heart Failure (AF-CHF) trial since all AF-CHF patients had an ejection fraction ≤35% and AF.Using marginal structural modeling, a contemporary statistical method that overcomes limitations of traditional modeling techniques and reduces bias, we assessed the impact of digoxin on the pre-specified primary and secondary outcomes of the AF-CHF trial, i.e., all-cause, cardiac and arrhythmic death as well as cardiovascular hospitalization. Among 1376 patients, 869 (65%) were on digoxin at one-year follow-up. Over a mean (SD) follow-up of 37 (19) months (maximum 74 months), 445 (32%) patients died, 357 (26%) from cardiovascular causes and 159 (12%) from arrhythmic death. Digoxin was significantly associated with all-cause, cardiac, and arrhythmic death, with estimated hazard ratios (HR) of 1.39 (95% confidence interval [CI] 1.11-1.73, P = 0.004), 1.44 (95% CI 1.13-1.82, P = 0.003), and 2.03 (95% CI 1.63-2.54, P 0.0001), respectively. Digoxin was not associated with cardiovascular hospitalizations [HR 1.12 (95% CI 0.91-1.37), P = 0.29].Digoxin is associated with increased all-cause mortality among patients with combined heart failure with reduced ejection fraction and AF, which is predominantly driven by arrhythmic deaths. In contrast, cardiovascular hospitalizations were not impacted by digoxin.

Background: Guidelines have previously suggested that atrial fibrillation (AF) is associated with an increased risk of arrhythmic death in Brugada syndrome (BrS) patients. However, only two articles consisting of 17 AF patients with BrS supported these views. The risk stratification of BrS patients with AF remains controversial. Thus, a meta-analysis is used to estimate the risk stratification of BrS patients with AF. Methods: We searched for relevant studies published from 2000 to December 30, 2018. A total of 1712 patients with BrS from five studies were included: 200 patients (12%) were reported with AF, among whom 37 patients (19%) had arrhythmic events. Results: BrS patients with AF in all studies (OR 1.92, 95% CI:0.91to 4.04, P =0.09; Heterogeneity: P = 0.03, I2=61%) and some European studies (OR 1.12, 95% CI: 0.18 to 6.94, P=0.91; Heterogeneity: P = 0.006, I2=80%) did not display a higher risk of arrhythmic events than those without AF, but BrS patients with AF in Japanese studies (OR 2.32, 95% CI: 1.37 to 3.93, P=0.002; Heterogeneity: P = 0.40, I2=0%) had a higher risk of arrhythmic events than those without AF. The proportion of BrS patients with AF was greater in Japanese studies than in some European studies (16% vs. 9%, P Conclusions: On the whole, BrS patients with AF showed no higher risk of arrhythmic events than those without AF, but BrS patients with AF in Japan had a higher risk of arrhythmic events than those without AF.

Background:People living with human immunodeficiency virus (HIV+) have greater risk for sudden arrhythmic death than HIV-uninfected (HIV–) individuals. HIV-associated abnormal cardiac repolarization may contribute to this risk. We investigated whether HIV serostatus is associated with ventricular repolarization lability by using the QT variability index (QTVI), defined as a log measure of QT-interval variance indexed to heart rate variance.Methods:We studied 1123 men (589 HIV+ and 534 HIV–) from MACS (Multicenter AIDS Cohort Study), using the ZioXT ambulatory electrocardiography patch. Beat-to-beat analysis of up to 4 full days of electrocardiographic data per participant was performed using an automated algorithm (median analyzed duration [quartile 1–quartile 3]: 78.3 [66.3–83.0] hours/person). QTVI was modeled using linear mixed-effects models adjusted for demographics, cardiac risk factors, and HIV-related and inflammatory biomarkers.Results:Mean (SD) age was 60.1 (11.9) years among HIV– and 54.2 (11.2) years among HIV+ participants (PConclusions:HIV+ men have greater beat-to-beat variability in QT interval (QTVI) than HIV– men, especially in the setting of HIV viremia and heightened inflammation. Among HIV+ men, higher QTVI suggests ventricular repolarization lability, which can increase susceptibility to arrhythmias, whereas lower heart rate variability signals a component of autonomic dysfunction.

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.

Cardiac arrhythmias present a number of challenges for the clinician. In all cases, any identified predisposing conditions should be managed, as possible. Signalment can be particularly important because some breeds have an increased prevalence of diseases associated with sudden arrhythmic death, including Boxers and Doberman Pinschers. The abolition of arrhythmias during exercise often is a favorable sign; whereas, their provocation with exertion is concerning, and in a horse, potentially dangerous to people. Cardiac arrhythmias can vary tremendously in frequency and severity over time. Ectopic complexes are identified and classified, as possible, by origin, site, and timing. Standards for choosing and continuing antiarrhythmic therapy are not clearly defined for many situations. Antiarrhythmic drugs might suppress potentially life-saving ventricular escape rhythms. Treatment goals for the individual patient also should be defined. Potential adverse effects of an antiarrhythmic drug must be considered against the desired benefits.

Funding Acknowledgements Type of funding sources: None. Background Although I-123 meta-iodobenzylguanidine (mIBG) has been applied to patients with chronic heart failure (CHF), a diagnostic tool for differential prediction of fatal arrhythmic events (ArE) and heart-failure death (HFD) has been pursued. Purpose The aim of this study was to create a calculator of mortality risk for differentiating mode of cardiac death using a machine learning (ML) method, and to test the accuracy in a new cohort of patients with CHF. Methods A total of 529 patients with CHF was used as the training database for ML. The ArE group consisted of patients with arrhythmic death, sudden cardiac death and appropriate therapy by implantable cardioverter defibrillator. A heart-to-mediastinum ratio (H/M) standardized to the medium-energy collimator condition was calculated with a planar anterior mIBG scintigram. The best classifier models for predicting HFD and ArE were determined by four-fold cross validation. Input variables included age, sex, New York Heart Association (NYHA) functional class, left ventricular ejection fraction, ischemic etiology, mIBG H/M and washout rate, and b-type natriuretic peptide (BNP) or NT Pro BNP, estimated glomerular filtration rate, hemoglobin, and complications such as diabetes and hypertension. After creating the ML-based model, the constructed classifier functions for ArE, HFD, and survival were exported for subsequent use. A new cohort of patients (n = 312, age 67 ± 13 years, 2015 or later) was used to test the ML-based model. Results The training database included 141 events (27%) with ArE (7%) and HFD (20%). Receiver-operating characteristic analysis by four-fold validation showed area under the curve value of 0.90 for HFD and 0.73 for ArE. Among various ML methods, the logistic regression method demonstrated the most stable calculation of the probability of ArE followed by random forest and gradient boosted tree methods. Therefore, the logistic-regression method was used for calculating both HFD and ArE probabilities. In the test cohort, patients with a high HFD probability >8% resulted in 6.3-fold higher HFD than those with low probability (≤ 8%). Patients with high ArE probability >8% showed 2.5-fold higher ArE than those with low probability (≤ 8%). Conclusion The ML-based mortality risk calculator could be used for stratifying patients at high and low risks, which might be useful for estimating appropriate treatment strategy.

Between 12–20 % of all Sudden Cardiac Death (SDC) occurs in individuals without previous symptoms of cardiac disease, which is known as Sudden Arrhythmic Death Syndrome (SADS). Recent studies identified the NOS1AP gene as new genetic marker to increase the risk of SDC. Here, we analyzed five SNPs of the NOS1AP gene by PCR-HRM in order to evaluate its ability as diagnostic and predictive biomarkers of the SADS.

Objective: To compare the effects of high-intensity interval training versus moderate-intensity continuous training on risk markers of arrhythmic death in patients who recently suffered from an acute coronary syndrome. Design: Double-blind (patient and evaluator) randomized controlled trial. Setting: Cardiovascular Prevention and Rehabilitation Centre (EPIC Centre) of the Montreal Heart Institute, Montreal, Canada. Subjects: A total of 43 patients were randomized following an acute coronary syndrome. Interventions: Patients were assigned to either high-intensity interval training (n = 18) or isocaloric moderate-intensity continuous training (n = 19), three times a week for a total of 36 sessions. Main measures: Heart rate recovery for 5 minutes, heart rate variability for 24 hours, occurrence of ventricular arrhythmias, and QT dispersion were measured before and after the 36 sessions of training. Results: Among the 43 patients randomized, 6 participants in the high-intensity interval training group stopped training for reasons unrelated to exercise training and were excluded from the analyses. Heart rate recovery improved solely in the high-intensity interval training group, particularly at the end of recovery period ( p < 0.05). There were no differences in heart rate variability, occurrence of ventricular arrhythmias, or QT dispersion parameters between the groups at study end. Conclusion: Despite the lack of power to detect any large difference between the two interventions with respect to risk markers of arrhythmic death, high-intensity interval training appears safe and may be more effective at improving heart rate recovery relative to moderate-intensity continuous training in our patients following acute coronary syndrome.

Objectives. Patients with heart failure and reduced left ventricular ejection fraction (HFrEF) are prone to ventricular tachyarrhythmias. We tested whether biomarkers C-terminal Endothelin 1 (CT-ET...

The goal of this study was to investigate the characteristics of mitral valve prolapse (MVP) in a post-mortem study of consecutive sudden cardiac deaths (SCDs) in subjects up to 90 years of age.Up to 2.3% of subjects with MVPs experience SCD, but by convention SCD is rarely confirmed by autopsy. In a post-mortem study of persons 40 years of age, 7% of SCDs were caused by MVP; bileaflet involvement, mitral annular disjunction (MAD), and replacement fibrosis were common.In the San Francisco POST SCD (Postmortem Systematic Investigation of Sudden Cardiac Death) study, autopsies have been performed on1,000 consecutive World Health Organization-defined (presumed) cases of SCD in subjects aged 18 to 90 years since 2011; a total of 603 were adjudicated. Autopsy-defined sudden arrhythmic death (SAD) required absence of nonarrhythmic cause; MVP diagnosis required leaflet billowing. One hundred antemortem echocardiograms were revised to identify additional MVPs missed on autopsy.Among the 603 presumed SCDs, 339 (56%) were autopsy-defined SADs, with MVP identified in 7 (1%). Six additional MVPs were identified by review of echocardiograms, for a prevalence of at least 2% among 603 presumed SCDs and 4% among 339 SADs (vs. 264 non-SADs; p = 0.02). All 6 additional MVPs had monoleaflet rather than bileaflet involvement and mild mitral regurgitation, ruling out hemodynamic cause. Less than one-half had MAD with replacement fibrosis, but all had multisite interstitial fibrosis.In a countywide post-mortem study of all adult cases of SCD, MVP prevalence was at least 4% of SADs, but one-half were missed on autopsy. Monoleaflet MVP was often underdiagnosed post-mortem. Compared with young cases of SCD, lethal MVP in older cases of SCD did not consistently have bileaflet anatomy, replacement fibrosis, or MAD.

Nevoid basal cell carcinoma syndrome (NBCCS), also referred to as Gorlin's syndrome, is an autosomal dominant inherited condition that predisposes affected individuals to various tumors such as cardiac fibromas. Though technically benign, cardiac fibromas may result in malignant arrhythmias and sudden death. The pertinent literature pertaining to pediatric cases of cardiac fibromas and their clinical features were reviewed. We present the case of an asymptomatic teenage with de novo NBCCS who was diagnosed with both NBCCS and cardiac fibroma later in life. The patient was noted to have clinically significant ventricular arrhythmias that were eliminated with tumor resection. There are no established best practice guidelines for the management of cardiac fibromas in patients with NBCCS. Given the risk of sudden arrhythmic death, the presence of ventricular arrhythmias should prompt strong consideration of tumor resection.

BACKGROUND & AIMSThe use of hydroxycholoroquin for COVID 19 treatment and prophylaxis raised issues concerning its cardiac safety owing to possibility of QT prolongation and arrhythmias.1 There was no study on long term electrocardiographic telemetry monitoring of patients taking hydroxychloroquin and we planned a continuous electrocardiographic holter telemetry of these patients for a period of seven days.MethodsHealthcare workers taking hydroxycholoroquin as pre exposure prophylaxis, patients taking hydroxychloroquin were monitored by holter electrocardiographic telemetry with continuous beat to beat analysis for seven days with capacity to report any arrhythmic event or significant QT prolongation instantly to medical faculty.Results25 participants with mean age 42.4 ± 14.1 years, 40% females. 20% patients needed to stop HCQ. Four patients developed QT prolongation > 500 ms and needed to stop HCQ, one patient had accelerated idioventricular rhythm and stopped treatment. one had short episodes of atrial fibrillation. No malignant arrhythmia or ventricular arrhythmia or torsades were noted. No episode of significant conduction disturbance and arrhythmic death noted. Baseline mean QTc was 423.96 ± 32.18 ms, mean QTc corrected at 24 hours 438.93 ± 37.95, mean QTc 451.879 ± 37.99 at 48 hours, change in baseline mean QTc to max QTc was 30.74 ± 21.75 ms at 48 hours. All those develop QTc prolongation > 500 ms were greater than 50 years of age.ConclusionAmbulatory telemetry ECG monitoring seems to detect early QT prolongation and stopping drug timely prevented malignant arrhythmias. HCQ seems to have less risk of QT prolongation in young healthy individuals.

Sudden unexpected death in epilepsy (SUDEP) has been identified as one of the most prevalent causes of mortality in epilepsy, and SUDEP has consequently become an important topic of research. The causes appear multifactorial, including epilepsy-induced cardiac arrest. Current understanding of autopsy negative sudden unexplained death (SUD) in general population and its relation to sudden arrhythmic death syndrome (SADS) could shed some light in SUDEP. Mutual attention to the findings of sudden death in cardiology and epilepsy are discussed here. We performed a narrative review on SUDEP, epilepsy and molecular/genetic autopsy in this population. A proposal of an extended terminology for SUDEP classification is discussed in light of recent issues related to molecular autopsy and genetics. The extended classification might be a step forward in research protocols and a tool for better understanding SUDEP.

INTRODUCTION. Elevated plasma levels of asymmetric dimethylarginine (ADMA), an inhibitor of NO synthase, are associated with adverse outcome. There is no data available, whether ADMA levels are associated with arrhythmic death (AD) in patients with ischemic cardiomyopathy (ICM) or non-ischemic, dilated cardiomyopathy (DCM). METHODS AND RESULTS. A total of 110 ICM, 52 DCM and 30 control patients were included. Primary outcome parameter of this prospective study was arrhythmic death (AD) or resuscitated cardiac arrest (RCA). Plasma levels of ADMA were significantly higher in ICM (p0.715 μmol/l) or the two lower tertiles (≤0.715 μmol/l) did not show a higher risk for AD or RCA (p=0.221) or overall mortality (p=0.548). In patients with left ventricular ejection fraction ≤35%, ADMA was not associated with AD or RCA (HR=1.35, p=0.084) or with overall mortality (HR=1.24, p=0.162). CONCLUSIONS. Plasma levels of ADMA were elevated in patients with ICM or DCM as compared to controls, but were not significantly predictive for overall mortality or the risk for arrhythmic death.

Background Sudden cardiac death (SCD) in the young (age 1-35) is commonly attributed to structural and arrhythmogenic syndromes, for which there is often an underlying genetic basis. Expert recommendation emphasises the importance of genetic testing in such cases, however to date this remains the first and only national programme in Europe to facilitate this. Aim To review detection rates of genetic variants in samples tested via the SADS BioBank and possibly demonstrate the merits of this novel resource for primary prevention for family members. Methods Family screening and consent for genetic testing was carried out in the Family Heart Screening Clinic. Result analysis of samples sent for molecular autopsy via the BioBank from its induction in January 2015 was performed. Genetic analysis was conducted via the same internationally accredited next generation sequencing lab. Results From January 2015 to July 2019, 161 samples had been stored in the SADS BioBank following confirmed SADS death on autopsy; 33% female and 67% male. Of these, 24 (14.9%) samples were sent for genetic testing: 21 for a 380 gene molecular autopsy and 3 for a targeted hypertrophic cardiomyopathy panel (173 genes). Of 24 samples tested, 10 (42%) yielded positive genetic variants: 4 American College of Medical Genetics (ACMG) Class IV or V mutations considered pathogenic, and 6 ACMG class III variants of uncertain significance (VUS). Familial cascade screening following confirmed pathogenic mutations resulted in detection of 3 (33.3%) positive genotypes in 9 first-degree relatives. Screening of relatives of Class III positive probands resulted in diagnosis of an Inherited Cardiac Condition (ICC) in 25% of first-degree relatives. 8.2% of first-degree relatives of probands with negative gene testing were given an ICC diagnosis following clinical screening. Conclusions This short study demonstrates the unique potential the SADS BioBank has to offer in terms of identifying those most at risk and optimising prevention strategies for relatives, thus highlighting the role for such a resource in terms of preventative screening in the future. Pathogenic Variant (ACMG Class IV & V) Variant of Uncertain Significance(ACMG Class III) No Gene Variant Identified Number Detected (n = 24) 4 6 14 1st Degree Relatives Screened (n = 86) 17 20 49 2nd Degree Relatives Screened (n = 46) 4 23 19 Genotype Detected (n = 4) 3 1 0 Phenotype Detected (n = 10) 1 5 4 Breakdown of clinical and genetic results of family screening by ACMG class. Abstract Figure.

ObjectiveUnlike for patients with other forms of cardiomyopathies, those with severe ventricular dysfunction due to Duchenne muscular dystrophy (DMD) are not offered implantable cardioverter-defibrillator (ICD) therapy routinely. This prospective study aimed to determine the views of DMD-patients and their carers about discussing sudden death risk and their acceptance of ICDs.Design and settingAdults with DMD (n=9) and parents/carers (n=9) participated in audio-recorded, 60–90 min focus group sessions (patients 2; parents/carers 2) conducted through either a face-to-face session at a neutral venue or a videoconference. Sessions were facilitated by a clinical psychologist, experienced in conducting focus group research. All participants understood the rationale for the study and the nature of ICD therapy. The same predefined themes were explored with each group. Recordings were transcribed, analysed thematically by two researchers, working independently and then agreed. Differences in responses between patient and carer groups were also studied and compared. Participants all provided informed written consent and the study had ethical approval.ResultsThree main themes emerged: (1) access to/quality of information provided by professionals and patient engagement with them; (2) decision-making about ICDs; (3) individuals’ own ‘lived experience’ of DMD.ConclusionsThe main findings were: (1) patients with DMD want to have their risk of sudden arrhythmic death discussed, when relevant and (2) if ICD therapy were established as beneficial, they would welcome an individualised discussion about its appropriateness for them.

IMPORTANCE: QT-prolonging medications (QTPMs) are a reported risk factor for sudden cardiac death (SCD) when defined by consensus criteria that presume an arrhythmic cause. The effect of QTPM on autopsy-defined sudden arrhythmic death (SAD) is unknown. OBJECTIVE: To evaluate the association between QTPM and autopsy-defined SAD vs nonarrhythmic cause of sudden death. DESIGN, SETTING, AND PARTICIPANTS: This prospective countywide case-control study included World Health Organization–defined (presumed) SCD cases who underwent autopsy as part of the San Francisco Postmortem Systematic Investigation of Sudden Cardiac Death Study (POST SCD) to determine arrhythmic or nonarrhythmic cause, and control deaths due to trauma (hereinafter referred to as trauma controls) in San Francisco County, California, from February 1, 2011, to March 1, 2014. Multivariate regression was used to evaluate the association of QTPM with the risk of presumed SCD, autopsy-defined SAD, and non-SAD compared with trauma controls. Medication exposure, determined by prescription lists and postmortem toxicologic findings, was used to calculate a summative QTPM exposure score (range, 0-20). Data were analyzed from September 1, 2018, to June 15, 2019. EXPOSURE: QT-prolonging medication exposure, as measured by QTPM score (1 indicated low; 2-4, moderate; and >4, high). MAIN OUTCOMES AND MEASURES: Death due to trauma, presumed SCD, and autopsy-defined non-SAD and SAD with no postmortem findings of extracardiac cause. RESULTS: A total of 629 patients (mean [SD] age, 61.4 [15.7] years; 439 men [69.8%]) were included, 525 with presumed SCDs and 104 traumatic death controls. Individuals with presumed SCDs had higher exposure and were more likely to be taking any QTPM (291 [55.4%] vs 28 [26.9%]; P

The risk of sudden cardiac death (SCD) is high in heart failure (HF) patients. Sudden arrhythmic death (SAD) is a frequent cause of exit in HF patients at the lower end of the HF spectrum, and implantable cardioverter–defibrillators have been recommended to prevent these life-threatening rhythm disturbances in select patients. However, less is known regarding the cause of SCD in patients at the upper end of the HF spectrum, despite the fact that the majority of out-of-hospital SCD victims have unknown or near-normal/normal left ventricular ejection fraction (LVEF). In this review, we report the epidemiology, summarize the mechanisms, discuss the diagnostic challenges, and propose a stepwise approach for the prevention of SAD in HF with near-normal/normal LVEF.

BACKGROUND: Although highly effective in preventing arrhythmic death, there is a high prevalence of anxiety, depression and reduced quality of life among patients who have received an implantable cardioverter defibrillator (ICD). Whether mortality, ICD shock and readmission are predicted by patient-reported outcomes is unknown.AIM: The aim of this study was to describe patient-reported outcomes among patients with ICDs compared by: ICD indication and generator type (ICD or cardiac resynchronisation therapy ICD), and to determine whether patient-reported outcomes at discharge predict mortality, ICD therapy and readmission.METHODS: A national cross-sectional survey at hospital discharge ( n=998) with register follow-up. Patient-reported outcomes included the Hospital Anxiety and Depression Scale, Short Form-12, HeartQoL, EQ-5D and Edmonton Symptom Assessment Scale. Register data: ICD therapy, readmissions and mortality within one year following discharge.RESULTS: Patients with primary prevention ICDs had significantly worse patient-reported outcomes at discharge than patients with secondary prevention ICDs. Likewise, patients with cardiac resynchronisation therapy ICDs had significantly worse patient-reported outcomes at discharge than patients without cardiac resynchronisation therapy. One-year mortality was predicted by patient-reported outcomes, with the highest hazard ratio (HR) being anxiety (HR 2.02; 1.06-3.86), but was not predicted by indication or cardiac resynchronisation therapy. ICD therapy and ventricular tachycardia/ventricular fibrillation were not predicted by patient-reported outcomes, indication or cardiac resynchronisation therapy. Overall, patient-reported outcomes predicted readmissions, e.g. symptoms of anxiety and depression predicted all readmissions within 3 months (HR 1.50; 1.13-1.98) and 1.47 (1.07-2.03), respectively).CONCLUSION: Patients with primary indication ICDs and cardiac resynchronisation therapy ICDs report worse patient-reported outcomes than patients with secondary indication and no cardiac resynchronisation therapy. Patient-reported outcomes such as mental health, quality of life and symptom burden predict one-year mortality and acute and planned hospital readmissions.

Growth differentiation factor (GDF)‐15 and soluble ST2 (sST2) are established prognostic markers in acute and chronic heart failure. Assessment of these biomarkers might improve arrhythmic risk stratification of patients with non‐ischaemic, dilated cardiomyopathy (DCM) based on left ventricular ejection fraction (LVEF). We studied the prognostic value of GDF‐15 and sST2 for prediction of arrhythmic death (AD) and all‐cause mortality in patients with DCM. We prospectively enrolled 52 patients with DCM and LVEF ≤ 50%. Primary end‐points were time to AD or resuscitated cardiac arrest (RCA), and secondary end‐point was all‐cause mortality. The median follow‐up time was 7 years. A cardiac death was observed in 20 patients, where 10 patients had an AD and 2 patients had a RCA. One patient died a non‐cardiac death. GDF‐15, but not sST2, was associated with increased risk of the AD/RCA with a hazard ratio (HR) of 2.1 (95% CI = 1.1‐4.3; P = .031). GDF‐15 remained an independent predictor of AD/RCA after adjustment for LVEF with adjusted HR of 2.2 (95% CI = 1.1‐4.5; P = .028). Both GDF‐15 and sST2 were independent predictors of all‐cause mortality (adjusted HR = 2.4; 95% CI = 1.4‐4.2; P = .003 vs HR = 1.6; 95% CI = 1.05‐2.7; P = .030). In a model including GDF‐15, sST2, LVEF and NYHA functional class, only GDF‐15 was significantly associated with the secondary end‐point (adjusted HR = 2.2; 95% CI = 1.05‐5.2; P = .038). GDF‐15 is superior to sST2 in prediction of fatal arrhythmic events and all‐cause mortality in DCM. Assessment of GDF‐15 could provide additional information on top of LVEF and help identifying patients at risk of arrhythmic death.