Risk Stratification in the Long-QT Syndrome

Priori, Silvia G.; Schwartz, Peter J.; Napolitano, Carlo; Bloise, Raffaella; Ronchetti, E; Grillo, Massimiliano; Vicentini, Alessandro; Spazzolini, Carla; Nastoli, Janni; Bottelli, Georgia; Folli, Roberta; Cappelletti, Donata

doi:10.1056/nejmoa022147

Cited by 1,263 publications

(612 citation statements)

References 16 publications

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“…10 Indeed, we now know that these silent mutation carriers are actually rather common, accounting for 36% of LQT1 patients, 19% of LQT2 patients, and 10% of LQT3 patients. 11 Thus, the Schwartz criteria were not meant for and cannot be used to identify silent mutation carriers. Based on the growing clinical evidence, in 2006 there was an update, 12 and the Schwartz score, ie, the number associated with a high probability of LQTS, was lowered from 4 to 3.5, which increased the sensitivity without reducing the specificity, thus reducing the number of false negatives.…”

Section: Article See P 2187mentioning

confidence: 99%

QTc Behavior During Exercise and Genetic Testing for the Long-QT Syndrome

2011

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Section: Article See P 2187mentioning

confidence: 99%

QTc Behavior During Exercise and Genetic Testing for the Long-QT Syndrome

2011

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“…Sudden death occurs most often in persons under the age of 40 in patients with genetic arrhythmias [19,[21][22][23]. Nevertheless, we only found two persons with SCD before the age of 40 and, as such, the result may reflect the relative rarity of genetic arrhythmias in Denmark.…”

Section: Open Accessmentioning

confidence: 57%

“…However, this is of uncertain importance, as we tried to examine the value of ECG as a predictive tool for SCD in the general population. Besides being the cornerstone of diagnostic methods of genetic arrhythmias, the ECG also predicts the outcome of these diseases [19][20][21]. However, the predictive role of the ECG in these diseases comes from register studies.…”

Section: Open Accessmentioning

confidence: 99%

ECG is not a reliable predictor of sudden cardiac death in the general population

Theilade¹,

Pecini²,

Marott³

et al. 2013

WJCD

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Objectives: To determine the predictive value of the ECG for sudden death in the general population. Design: In the Copenhagen City Heart Study, a randomly selected population sample in Copenhagen, Denmark has been followed prospectively since 1976. From this population sample, we analyzed ECGs of individuals who had suffered sudden cardiac death (SCD) before the age of 50 years and compared them with ECGs of a randomly selected control individuals from the same population sample. Specific ECG signs that could point toward a condition associated with a risk of SCD were noted. Results: From a total of 18,974 individuals in the cohort, 207 had died at an age younger than 50 years. Among these, 24 persons with SCD were identified. The most prevalent ECG abnormality was QRS fragmentation. We found no ECGs with long or short QTc, Brugada sign or WPW. The prevalence of signs of left ventricular hypertrophy, early repolarization, or fragmentation was not different from the prevalence of these signs in the control group. Conclusion: In the Copenhagen City Heart Study, the ECG failed to predict SCD in persons who died before the age of 50 years.

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“…Ventricular arrhythmias are most often associated with QTc values of 500 milliseconds or more17, 20 and in this group (severe long QTc) the incidence was 1.7%. This is the first major study reporting incidence in acute medical admissions.…”

Section: Discussionmentioning

confidence: 79%

Incidence and outcomes of long QTc in acute medical admissions

et al. 2018

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SummaryAimsProlonged QT interval on electrocardiogram (ECG) increases the risk of ventricular arrhythmia. Patients admitted to acute medical units (AMU) may be at risk of QT prolongation from multiple, recognised risk factors. Few data exist regarding incidence or outcomes of QT prolongation in acute general medical admissions.The aims were to determine the incidence of Bazett's‐corrected QT (QTc) prolongation upon admission to AMU; the relationship between QTc and inpatient mortality, length of stay and readmission; proportion with prolonged QTc subsequently administered QT interval‐prolonging drugs.MethodsRetrospective, observational study of 1000 consecutive patients admitted to an AMU in a large urban hospital. Exclusion criteria: age <18 years, ventricular pacing, poor quality/absent ECG . QTc determined manually from ECG obtained within 4‐hours of admission. QTc prolongation considered ≥470 milliseconds (males) and ≥480 milliseconds (females). In both genders, >500 milliseconds was considered severe. Study end‐points, (a) incidence of QTc prolongation at admission; (b) inpatient mortality, length of stay and readmission rates; (c) proportion with QTc prolongation subsequently administered QT interval‐prolonging drugs.ResultsOf 1000 patients, 288 patients were excluded, therefore final sample was n = 712. Patient age (mean ± SD) was 63.1 ± 19.4 years; females 49%. QTc prolongation was present in n = 50 (7%) at admission; 1.7% had QTc interval >500 ms. Of the 50 patients admitted with prolonged QTc, 6 (12%) were subsequently administered QT interval‐prolonging drugs. QTc prolongation was not associated with worse inpatient mortality or readmission rate. Length of stay was greater in those with prolonged QTc, 7.2 (IQR 2.4‐13.2) days vs 3.3 (IQR 1.3‐10.0; P = 0.004), however, in a regression model, presence of QTc did not independently affect length of stay.Conclusions QTc interval prolongation is frequent among patients admitted to AMU. QT interval‐prolonging drugs are commonly prescribed to patients presenting with prolonged QTc but whether this affects clinical outcomes is uncertain.

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Risk Stratification in the Long-QT Syndrome

Abstract: The locus of the causative mutation affects the clinical course of the long-QT syndrome and modulates the effects of the QTc and sex on clinical manifestations. We propose an approach to risk stratification based on these variables.

Cited by 1,263 publications

References 16 publications

QTc Behavior During Exercise and Genetic Testing for the Long-QT Syndrome

QTc Behavior During Exercise and Genetic Testing for the Long-QT Syndrome

ECG is not a reliable predictor of sudden cardiac death in the general population

Incidence and outcomes of long QTc in acute medical admissions

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