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Table 1 Echocardiographic measurements in nine children with Alström syndrome

From: Mutation identification and prediction for severe cardiomyopathy in Alström syndrome, and review of the literature for cardiomyopathy

 

No of patients

Mean ± SD

(min–max)

Reference values*

LV systolic functions

EF with Teicholz formula (%)

9

42.5 ± 43 (24–58)

 > 55

Speckle strain (GLS) (%)

5

− 12.74 ± 4.06 (− 8–16)

 − 17 − 24

RV systolic functions

FAC (%)

9

37 ± 7 (26–47)

 > 35

TAPSE (mm)

9

13 ± 4.6 (6–18)

 > 18

  1. EF Ejection fraction; LV left ventricular; RV right ventricular, RV FAC right ventricular fractional area change; TAPSE tricuspid annular plane systolic excursion, GLS global longitudinal strain
  2. *Recommendations for quantification methods during the performance of a pediatric echocardiogram: a report from the Pediatric Measurements Writing Group of the American Society of Echocardiography Pediatric and Congenital Heart Disease Council. J Am Soc Echocardiogr 2010; 23: 465–95
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172