Dataset Information

Electrocardiographic amplitudes: a new risk factor for sudden death in hypertrophic cardiomyopathy.

ABSTRACT:

Aims

Assessment of ECG-features as predictors of sudden death in adults with hypertrophic cardiomyopathy (HCM).

Methods and results

ECG-amplitude sums were measured in 44 normals, 34 athletes, a hospital-cohort of 87 HCM-patients, and 29 HCM-patients with sudden death or cardiac arrest (HCM-CA). HCM-patients with sudden death or cardiac arrest had substantially higher ECG-amplitudes than the HCM-cohort for limb-lead and 12-lead QRS-amplitude sums, and amplitude-duration products (P = 0.00003-P = 0.000002). Separation of HCM-CA from the HCM-cohort is obtained by limb-lead QRS-amplitude sum >or=7.7 mV (odds ratio 18.8, sensitivity 87%, negative predictive value (NPV) 94%, P < 0.0001), 12-lead amplitude-duration product >or=2.2 mV s (odds ratio 31.0, sensitivity 92%, NPV 97%, P < 0.0001), and limb-lead amplitude-duration product >or=0.70 mV s (odds ratio 31.5, sensitivity 93%, NPV 96%, P < 0.0001). Sensitivity in HCM-patients <40 years is 90, 100, and 100% for those ECG-variables, respectively. Qualitative analysis showed correlation with cardiac arrest for pathological T-wave-inversion (P = 0.0003), ST-depression (P = 0.0010), and dominant S-wave in V(4) (P = 0.0048). A risk score is proposed; a score >or=6 gives a sensitivity of 85% but a higher positive predictive value than above measures. Optimal separation between HCM-CA <40 years and athletes is obtained by a risk score >or=6 (odds ratio 345, sensitivity 85%, specificity 100%, P < 0.0001).

Conclusion

Twelve-lead ECG is a powerful instrument for risk-stratification in HCM.

SUBMITTER: Ostman-Smith I

PROVIDER: S-EPMC2821628 | biostudies-literature | 2010 Feb

REPOSITORIES: biostudies-literature

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Publications

Electrocardiographic amplitudes: a new risk factor for sudden death in hypertrophic cardiomyopathy.

Ostman-Smith Ingegerd I Wisten Aase A Nylander Eva E Bratt Ewa-Lena EL Granelli Anne de-Wahl Ad Oulhaj Abderrahim A Ljungström Erik E

European heart journal 20091105 4

<h4>Aims</h4>Assessment of ECG-features as predictors of sudden death in adults with hypertrophic cardiomyopathy (HCM).<h4>Methods and results</h4>ECG-amplitude sums were measured in 44 normals, 34 athletes, a hospital-cohort of 87 HCM-patients, and 29 HCM-patients with sudden death or cardiac arrest (HCM-CA). HCM-patients with sudden death or cardiac arrest had substantially higher ECG-amplitudes than the HCM-cohort for limb-lead and 12-lead QRS-amplitude sums, and amplitude-duration products ( ...[more]

PMID: 19897498

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Project description:BackgroundHypertrophic cardiomyopathy is the leading cause of sudden cardiac death (SCD) in children and young adults. Our objective was to develop and validate a SCD risk prediction model in pediatric hypertrophic cardiomyopathy to guide SCD prevention strategies.MethodsIn an international multicenter observational cohort study, phenotype-positive patients with isolated hypertrophic cardiomyopathy <18 years of age at diagnosis were eligible. The primary outcome variable was the time from diagnosis to a composite of SCD events at 5-year follow-up: SCD, resuscitated sudden cardiac arrest, and aborted SCD, that is, appropriate shock following primary prevention implantable cardioverter defibrillators. Competing risk models with cause-specific hazard regression were used to identify and quantify clinical and genetic factors associated with SCD. The cause-specific regression model was implemented using boosting, and tuned with 10 repeated 4-fold cross-validations. The final model was fitted using all data with the tuned hyperparameter value that maximizes the c-statistic, and its performance was characterized by using the c-statistic for competing risk models. The final model was validated in an independent external cohort (SHaRe [Sarcomeric Human Cardiomyopathy Registry], n=285).ResultsOverall, 572 patients met eligibility criteria with 2855 patient-years of follow-up. The 5-year cumulative proportion of SCD events was 9.1% (14 SCD, 25 resuscitated sudden cardiac arrests, and 14 aborted SCD). Risk predictors included age at diagnosis, documented nonsustained ventricular tachycardia, unexplained syncope, septal diameter z-score, left ventricular posterior wall diameter z score, left atrial diameter z score, peak left ventricular outflow tract gradient, and presence of a pathogenic variant. Unlike in adults, left ventricular outflow tract gradient had an inverse association, and family history of SCD had no association with SCD. Clinical and clinical/genetic models were developed to predict 5-year freedom from SCD. Both models adequately discriminated between patients with and without SCD events with a c-statistic of 0.75 and 0.76, respectively, and demonstrated good agreement between predicted and observed events in the primary and validation cohorts (validation c-statistic 0.71 and 0.72, respectively).ConclusionOur study provides a validated SCD risk prediction model with >70% prediction accuracy and incorporates risk factors that are unique to pediatric hypertrophic cardiomyopathy. An individualized risk prediction model has the potential to improve the application of clinical practice guidelines and shared decision making for implantable cardioverter defibrillator insertion. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT0403679.

Project description:ImportanceSudden cardiac death (SCD) is the most common mode of death in childhood hypertrophic cardiomyopathy (HCM), but there is no validated algorithm to identify those at highest risk.ObjectiveTo develop and validate an SCD risk prediction model that provides individualized risk estimates.Design, setting, and participantsA prognostic model was developed from a retrospective, multicenter, longitudinal cohort study of 1024 consecutively evaluated patients aged 16 years or younger with HCM. The study was conducted from January 1, 1970, to December 31, 2017.ExposuresThe model was developed using preselected predictor variables (unexplained syncope, maximal left-ventricular wall thickness, left atrial diameter, left-ventricular outflow tract gradient, and nonsustained ventricular tachycardia) identified from the literature and internally validated using bootstrapping.Main outcomes and measuresA composite outcome of SCD or an equivalent event (aborted cardiac arrest, appropriate implantable cardioverter defibrillator therapy, or sustained ventricular tachycardia associated with hemodynamic compromise).ResultsOf the 1024 patients included in the study, 699 were boys (68.3%); mean (interquartile range [IQR]) age was 11 (7-14) years. Over a median follow-up of 5.3 years (IQR, 2.6-8.3; total patient years, 5984), 89 patients (8.7%) died suddenly or had an equivalent event (annual event rate, 1.49; 95% CI, 1.15-1.92). The pediatric model was developed using preselected variables to predict the risk of SCD. The model's ability to predict risk at 5 years was validated; the C statistic was 0.69 (95% CI, 0.66-0.72), and the calibration slope was 0.98 (95% CI, 0.59-1.38). For every 10 implantable cardioverter defibrillators implanted in patients with 6% or more of a 5-year SCD risk, 1 patient may potentially be saved from SCD at 5 years.Conclusions and relevanceThis new, validated risk stratification model for SCD in childhood HCM may provide individualized estimates of risk at 5 years using readily obtained clinical risk factors. External validation studies are required to demonstrate the accuracy of this model's predictions in diverse patient populations.

Dataset Information

Electrocardiographic amplitudes: a new risk factor for sudden death in hypertrophic cardiomyopathy.

Aims

Methods and results

Conclusion

Publications

Electrocardiographic amplitudes: a new risk factor for sudden death in hypertrophic cardiomyopathy.

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