Description

Figliozzi et al reported a nomogram for predicting cardiac involvement in Fabry disease. This can help to identify a patient who may require a more complete cardiac assessment. The authors are from mulitple institutions in Italy, the United States and Switzerland.


Patient selection: Fabry disease

 

Parameters:

(1) QRS in msec, from 80 to 160

(2) QT interval in msec, from 320 to 560

(3) Sokolow Lyon index, from 1 to 6.5

(4) P wave to PR segment ratio, from 0 to 5

 

points for QRS =

= (0.896 * (QRS)) - 71.7

 

points for QT interval =

= (0.304 * (QT)) - 97.3

 

points for Sokolow-Lyon index =

= (16.2 * (SLI)) - 16.2

 

points for P wave to PR segment ratio =

= 20 * (ratio)

 

total score =

= SUM(points for all of the parameters)

 

Interpretation:

• minimum score: 0

• maximum score: 333.9

 

value of X =

= (0.073 * (score)) - 7.27

 

probability of Fabry involvement of the heart =

= 1 / (1 + EXP((-1) * X))

 

Performance:

• The area under the ROC curve is 0.90 in the test group and 0.81 in validation.


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