Description

Schwartz et al updated the criteria for the long QT syndrome in 1993. This can help diagnose a patient with the idiopathic long QT syndrome.


 

Patient preparation: The patient must not be taking a medication known to prolong the QT interval. A person should not be evaluated while suffering from a condition affecting the QT interval.

 

Parameters:

(1) clinical history of syncope

(2) clinical history of congenital deafness

(3) definite family history of long QT syndrome

(4) unexplained sudden death in an immediate family member not included in #3

(5) long corrected QT interval on ECG

(6) torsade de pointes

(7) T wave alternans

(8) notched T wave in 3 leads

(9) low heart rate for age

Parameter

Finding

Points

clinical history of syncope

none

0

 

without stress

1

 

with stress

2

congenital deafness

absent

0

 

present

0.5

family history of long QT syndrome

absent

0

 

present

1

unexplained sudden death in an immediate family member before age 30

absent

0

 

present

0.5

corrected QT interval

< 450 msec

0

 

450 msec (in males)

1

 

460-470 msec

2

 

>= 480 msec

3

torsade de pointes

absent

0

 

present

2

T wave alternans

absent

0

 

present

1

leads with notched T waves

>= 3

1

 

< 3

0

heart rate for age

low (< 2nd percentile for age)

0.5

 

not low

0

 

where:

• Torsade de pointes and syncope are mutually exclusive.

• The same family member cannot be used to satisfy a family history of LQTS and sudden death.

• The heart rates for age in children are given by Davignon et al (1980).

• The ranges for corrected QT interval given in the original table do not cover a number of situations: (a) female with interval 450 msec; (b) range 451-459; (c) range 471-479. I have set (a) to 0 points; (b) to 1 point; (c) to 2 points in the implementation.

 

total score =

= MAX((points for syncope), (points for torsade on ECG)) + SUM(points for remaining parameters)

 

Interpretation:

• minimum score: 0

• maximum score: 9.5

 

Total Score

Probability of LQTS

<= 1

low

2 to 3

intermediate

>= 4

high

 

where:

• This scheme leaves out scores 1.5 and 3.5. In the implementation I have made 1.5 to 3.5 as intermediate risk.

 


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