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Table 3 Treatment response after 1-year Tafamidis treatment

From: Tafamidis decreased cardiac amyloidosis deposition in patients with Ala97Ser hereditary transthyretin cardiomyopathy: a 12-month follow-up cohort study

 

Pre Tafamidis (N = 14)

12 m Tafamidis (N = 14)

P value

Functional status

 NYHA Fc I

1 (7%)

3 (21%)

0.083

 NYHA Fc II

12 (86%)

11 (79%)

 NYHA Fc II

1 (7%)

0

Improved: 3 (21%)

Stationary: 11 (79%)

Worse: 0 (0%)

Biochemistry

 Creatinine, mg/dL

0.76 ± 0.21

0.70 ± 0.17

0.024

 Triglyceride, mg/dL

74.5 ± 27.0

76.6 ± 38.8

0.859

 Total cholesterol, mg/dL

173.9 ± 31.6

176.0 ± 37.8

0.610

 Fasting blood glucose, mg/dL

83.5 ± 15.0

88.9 ± 23.9

0.834

 Albumin, g/dL

4.1 ± 0.2

7.9 ± 12.3

0.833

 Prealbumin*, mg/dL

27.1 ± 9.1

32.0 ± 7.7

0.046

 Log NT-ProBNP

3.0 ± 0.54

2.9 ± 0.47

0.272

 NT-ProBNP, pg/mL

2078.1 ± 3886.3

1133.4 ± 1032.6

0.221

Echocardiography

 

 IVSD, mm

15.4 ± 2.5

15.4 ± 2.8

1.000

 LVPWD, mm

14.8 ± 2.1

14.2 ± 2.5

0.119

 LVEDD, mm

45.2 ± 3.7

45.0 ± 4.5

0.936

 LVESD, mm

31.6 ± 4.8

30.9 ± 6.7

0.550

 LVEF, %

56.6 ± 11.7

58.8 ± 15.1

0.594

 LA diameter, mm

40.5 ± 4.5

40.5 ± 6.1

0.906

Cardiac MRI

 LVEF, %

54.1 ± 9.5

53.1 ± 12.5

0.683

 LVEDVi, ml/m2

69.3 ± 16.7

76.2 ± 22.3

0.177

 LVESVi, ml/m2

32.3 ± 12.0

36.4 ± 15.4

0.551

 LVMi, g/m2

93.0 ± 24.8

97.7 ± 31.2

0.331

 GRS, %

19.2 ± 8.9

18.8 ± 9.0

0.433

 GCS, %

-12.6 ± 4.3

-12.3 ± 4.5

0.363

 GLS, %

-8.7 ± 3.3

-8.9 ± 3.5

0.730

 Native T1 Mapping, ms

1159.5 ± 53.0

1157.4 ± 68.2

0.975

 ECV, %

51.5 ± 8.9

49.0 ± 9.4

0.041

  1. * Prealbumin only available in 6 patients
  2. Abbreviations: IVSD = interventricular septal diameter; LVPWD = Left ventricular posterior wall diameter; LVEDD = left ventricular end-diastolic diameter; LVESD = left ventricle end-systolic diameter; LVEF = left ventricular ejection fraction; LA diameter = left atrial diameter; LVEF = left ventricular ejection fraction; LVEDVi = LV end-diastolic volume index; LVESVi = LV end-systolic volume index; LVMi = LV mass index; GRS = global radial strain; GCS = global circumferential strain; GLS = global longitudinal strain; ECV = Extracellular volume