Short QT syndrome is an inherited channelopathy that affects the movement of ions in the heart. It is characterized by an abnormally short QT interval on electrocardiograms. The syndrome can cause sudden cardiac arrest. Diagnosis involves assessing personal and family history of cardiac events, performing an ECG to measure QT intervals, and genetic testing. Treatment may involve implantation of an ICD to prevent sudden cardiac death in high-risk patients.

1. Short QT
syndrome
Tara Rajendran
09/19/2017,Tuesday
Florida International University

2. inherited channelopathy (disorder which affects the moveme
In contrast to long QT syndrome, another channelopathy, io

3. ETIOLOGY
Since its first report in 2000, significant progress has been m

4. Diagnostic Algorithm
Personal History
Family History
Take a 12 lead ECG
Look at genetic report

5. Personal History
FEATURES SCORES
History of sudden cardiac arrest 2
Documented PMVT or VF 2
Unexplained syncope 1
Atrial fibrillation 1

6. Family History
FEATURES SCORE
First- or second-degree relative with
high probability of SQT
2
First- or second-degree relative with
autopsy-negative SCD
1
Sudden infant death syndrome 1

7. 12 lead ECG
Corrected QT interval in ms score
<370 1
<350 2
<330 3
J point peak to T-wave interval<120
msec
1

8. GENETIC STUDY
GENOTYPE SCORE
Geno type positive 2
Mutation of undetermined significance
in a culprit gene
1

9. Interpretation
A score > 4 indicates high Probability
A score > 3 indicates intermediate
probability
A score > 2 indicates low probability
This scoring system requires at least 1 point from ECG
section before evaluating other data

10. TREATMENT
implantation of an implantable cardioverter-defibrillator (ICD)
as a preventive action
h short QT syndrome due to their effects on prolonging the action p

11. THANK YOU