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Universal Electrocardiographic Screening for Long QT Syndrome in Hospitalized Neonates
Bethany D Kaemingk1, Timothy J Ulrich2, Man Li3
1Division of Neonatal Medicine, Mayo Clinic, Rochester, Minnesota.
Insights
Continuous QTc monitoring in neonates did not effectively identify Long QT syndrome (LQTS) risk. This screening method resulted in a 0% positive predictive value and led to unnecessary follow-up testing.
Area of Science:
- Neonatal cardiology
- Cardiac electrophysiology
- Genetics and inherited diseases
Background:
- Long QT syndrome (LQTS) is a critical cause of sudden unexpected death in neonates.
- Routine electrocardiographic (ECG) screening is proposed for neonatal LQTS detection.
- Continuous QTc monitoring offers a potential method for early identification of at-risk infants.
Purpose of the Study:
- To evaluate the efficacy of continuous electronic QTc monitoring for screening hospitalized neonates for LQTS risk.
- To determine the positive predictive value (PPV) of this screening method.
- To assess the clinical utility and necessity of follow-up testing triggered by electronic monitoring.
Main Methods:
- A cohort of 589 hospitalized neonates underwent continuous electronic QTc monitoring.
- Screening criteria included a 24-hour average QTc interval ≥475 ms.
- Positive screens prompted 12-lead ECG and, in some cases, echocardiography.
Main Results:
- A 5.6% positive screen rate was observed, with all cases yielding negative follow-up ECGs (PPV = 0%).
- No clinically significant cardiac pathology was identified in neonates who underwent echocardiography.
- The screening method proved inefficient in identifying neonates with LQTS risk.
Conclusions:
- Continuous electronic QTc monitoring is not an efficient strategy for identifying LQTS risk in hospitalized neonates.
- The screening approach led to unnecessary diagnostic procedures, including echocardiography.
- Current electronic monitoring methods require refinement for effective neonatal LQTS screening.
Objective:
Long QT syndrome (LQTS) is a known cause of unexpected death, leading some to recommend routine neonatal electrocardiographic (ECG) screening. We used continuous electronic heart rate corrected QT interval (QTc) monitoring to screen for interval prolongation in a cohort of hospitalized neonates to identify those at a risk of having LQTS. We hypothesized that this screening method would yield an acceptable positive predictive value (PPV).
Study Design:
A cohort of 589 infants hospitalized in a level II neonatal intensive care unit were screened through continuous electronic QTc monitoring linked to an investigator-designed, computerized data sniffer. Screening was conducted from days-of-life 3 through 7 or until hospital discharge. The data sniffer alerted investigators for a 24-hour average QTc of ≥475 ms. Positively screened patients were further evaluated with 12-lead ECG.
Results:
Positive screens were obtained in 5.6% of patients, all of whom had negative follow-up ECG testing (PPV = 0%). Furthermore, one-quarter of positively screened neonates underwent echocardiography based on ECG findings, none of which identified clinically relevant pathology.
Conclusion:
Electronic monitoring of QTc in hospitalized neonates during the first week of life was not an efficient way to identify those at a risk of having LQTS. Conversely, screening triggered unnecessary testing.
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