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May 1, 1999Archives of Disease in ChildhoodOpen Access

Prolonged QT interval in Rett syndrome

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Key result

Rett syndrome is linked to longer QTc, with QT prolongation affecting ~26% of girls.

  • n=34

Why the study?

Prolonged QT interval has been described in Rett syndrome, but its prevalence and association with cardiac tachyarrhythmias needed prospective investigation.

Do girls with Rett syndrome have prolonged QT intervals compared to healthy controls?

Population

34 girls with Rett syndrome

Comparison

Patients with Rett syndrome vs healthy controls of similar age

Design

Prospective cohort study with electrocardiography and 24 hour Holter monitoring

Authors

CECarolyn EllawayThe University of SydneyGSGary F. ShollerThe University of SydneyHLHelen LeonardThe Kids Research Institute Australia

Discussion

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Implication

Rett syndrome was associated with QT prolongation; hypothesis-generating for arrhythmia risk and requires prospective validation.

Study Design

Type

Cohort (n=34)

Structured PICO

Do girls with Rett syndrome have prolonged QT intervals compared to healthy controls?

P
Population
34 girls with Rett syndrome and a group of healthy controls of similar age evaluated for QT prolongation and cardiac tachyarrhythmias.
E
Exposure
Electrocardiography and 24 hour Holter monitoring
C
Comparator
Healthy controls of a similar age range
O
Outcome
QT prolongation (corrected QT values) and presence of cardiac tachyarrhythmiassurrogate

Girls with Rett syndrome have a significantly higher prevalence of prolonged QT intervals compared to healthy controls, highlighting the need for cardiac screening in this population.

Cite This Study

Ellaway et al. (1999) conducted a cohort in Rett syndrome (n=34). Rett syndrome vs. Healthy controls was evaluated on Corrected QT value. Girls with Rett syndrome had significantly longer corrected QT values compared to healthy controls, with prolonged QT observed in 9 of 34 patients.

synapsesocial.com/papers/6aaa06680a88a9c60a481d48https://doi.org/10.1136/adc.80.5.470
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Also Consider

Synapse has enriched 4 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Prolongation of the QT Interval and the Sudden Infant Death Syndrome1999 · 57 citations
  2. 2Anesthetic Implications of Prolonged QT Interval Syndromes1985 · 37 citations
  3. 3Diagnostic criteria for rett syndrome1988 · 404 citations
  4. 4Prolongation of the QT Interval and the Sudden Infant Death Syndrome1998 · 57 citations