Classification and mechanism of initiation in patients with congenital long QT syndrome
Abstract
AIMS: To examine the initiating mode of Torsade de Pointes (TdP) in patients with congenital long QT syndrome (LQTS). METHODS AND RESULTS: We evaluated 111 episodes of TdP recorded on the electrocardiograms of 24 patients with congenital LQTS, and clarified the initiating mode, the three consecutive preceding RR intervals defined as C(2), C(1), and C(0), the timing of initiating premature ventricular contraction (PVC) and the cycle length (CL) of TdP. Three different initiating patterns were observed: (1) a "short-long-short" sequence (SLS) pattern (23 patients, 72 TdP, 65%) defined as one or more short-long cardiac cycles followed by an initiating short-coupled PVC (C(1)>C(2) and C(0)), (2) an "increased sinus rate" (ISR) pattern (8 patients, 28 TdP, 25%) defined as a gradual increase in sinus rate with or without T-wave alternans (C(2)>/=C(1)>/=C(0)), and (3) a "changed depolarization" (CD) pattern (5 patients, 11 TdP, 10%) defined as a sudden long-coupled PVC or fusion beat followed by short-coupled PVC. The C(0) was shorter in ISR than SLS and CD (mean C(0): 488 vs. 587 and 603 ms, respectively; P<0.05). Therefore, the initiating PVC appeared near the T-wave peak of the last beat before onset in ISR, while it occurred after the T-wave peak in SLS and CD. The CL of TdP was shorter in ISR than in SLS (256 vs. 295 ms, P<0.05). CONCLUSIONS: Our data show the existence of three predominant initiating modes of TdP in patients with congenital LQTS and suggests a differential mechanism of initiation of TdP for each mode.
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Significant QTc prolongation (to intervals above 470-500 ms) is known to result in a risk of potentially fatal arrhythmias, such as Torsade de Pointes.
“Similarly, significant QTc prolongation (to intervals above 470–500 ms) is known to result in a risk of potentially fatal arrhythmias, such as Torsade de Pointes [100].”
From European guidelines on managing adverse effects of medication for ADHD (2011) · cites this paper as [100]
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- Authors
- NODA, T., SHIMIZU, W., SATOMI, K., SUYAMA, K., KURITA, T., AIHARA, N., & KAMAKURA, S.
- Corresponding author
- NODA, T
- Venue
- European Heart Journal
- Publisher
- Oxford University Press (OUP)
- Published
- 2004-12
- Volume
- 25
- Issue
- 23
- Pages
- 2149-2154
- ISSN
- 0195-668X (ISSN-L)
- OpenAlex
- W2175776907
- Cited by
- 83
- References
- 29
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 3 · 2023: 1 · 2022: 4 · 2021: 7 · 2020: 1 · 2019: 7 · 2017: 3 · 2016: 2 · 2015: 5 · 2014: 3 · 2013: 4 · 2012: 2
- FWCI
- 3.92
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- European Heart Journal (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Arrhythmias and Treatments
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac pacing and defibrillation studies
- Keywords
- congenital long QT syndrome, torsades de pointes, premature ventricular contractions, T-wave alternans, cycle length
- MeSH terms
- Adolescent, Adult, Child, Child, Preschool, Electrocardiography, Female, Humans, Infant, Long QT Syndrome, Male, Middle Aged, Torsades de Pointes, Ventricular Premature Complexes
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