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Published on: January 16, 2019
Who is at risk for cardiac events in young patients with long QT syndrome?
Masao Yoshinaga1, Masami Nagashima, Toshimitsu Shibata
1Department of Pediatrics, Faculty of Medicine, Kagoshima University, Kagoshima, Japan. m-yoshi@m2.kufm.kagoshima-u.ac.jp
Insights
Young patients with long QT syndrome (LQTS) with a history of cardiac events face higher risks. Improving medication compliance is crucial for preventing future cardiac events in this high-risk group.
Area of Science:
- Cardiology
- Genetics
Background:
- Long QT syndrome (LQTS) is a cardiac channelopathy.
- Identifying at-risk young patients is crucial for timely intervention.
Purpose of the Study:
- To identify risk factors for cardiac events in young LQTS patients.
- To assess risk stratification in LQTS patients with and without prior cardiac events.
Main Methods:
- Retrospective analysis of 197 young LQTS patients across 36 Japanese hospitals (1997-2000).
- Stepwise regression analyses were used to predict cardiac events.
- LQTS score was evaluated for its predictive value.
Main Results:
- Patients with a history of cardiac events had a significantly higher incidence of new events (47%) compared to those without (5%).
- Noncompliance with medication and younger age at diagnosis were significant predictors in patients with a history of events.
- An LQTS score <4 had 100% negative predictive value for cardiac events in patients without a prior history.
Conclusions:
- Young LQTS patients with a prior cardiac event history are at substantially higher risk.
- Medication compliance is a critical factor for preventing recurrent cardiac events in LQTS patients.
- A low LQTS score is a reliable indicator for the absence of cardiac events in LQTS patients with no prior history.
Abstract:
The objective of this study was to determine who is at risk for cardiac events among young patients with long QT syndrome (LQTS) with or without a past history of LQTS-related cardiac events. The subjects were young patients with LQTS who had visited one of 36 hospitals from January 1997 to August 2000 in Japan. To predict the risk factors for cardiac events, stepwise regression analyses were performed for a total of 197 cases. There were 7 of 129 cases (5%) without a past history and 32 of the 68 (47%) cases with a past history of LQTS-related cardiac events that experienced new events after diagnosis (p<0.0001). Patients with a family history showed a higher incidence of symptoms both before and after diagnosis than patients with sporadic occurrence. Analyses revealed that noncompliance with medication and a lower age at diagnosis were significant predictors for the group with a past history. A negative predictive value <4 points was 100% in the group without a past history. To prevent future cardiac events, compliance with medication must be improved in those with a past history. A total LQTS score <4 points was useful to predict the absence of cardiac events in the group without a past history.
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