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Table 2 Clinical demographics and investigation data: Fabry versus HCM

From: Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy

 

Fabry

HCM: primary prevention

p valuec

Primary prevention

Secondary prevention

p valueb

Sample size (n, %)

36 (72)

14 (28)

N/A

64

N/A

Follow-up duration (years)

3.8 ± 2.6

5.8 ± 3.9

0.036

6.4 ± 2.9

 < 0.001

Age (years)

58 ± 12

57 ± 12

0.922

56 ± 19

0.516

Male gender (n, %)

8 (16)

2 (4)

0.704

21 (33)

0.373

On ERT (n, %)

23 (46)

11 (22)

0.501

–

N/A

Classical mutation (n, %)

16 (32)

4 (8)

0.353

–

N/A

BMI (kg/m2)

27.0 ± 6.0

31.6 ± 7.2

0.087

28.4 ± 6.2

0.411

HR (bpm)

64 ± 16

55 ± 8

0.265

66 ± 12

0.633

SBP (mmHg)

122 ± 22

124 ± 19

0.824

128 ± 22

0.436

DBP (mmHg)

71 ± 16

73 ± 7

0.785

76 ± 12

0.292

MSSI

16.7 ± 9.4

11.9 ± 7.1

0.104

–

N/A

Comorbidities

IHD (n, %)

2 (4)

0 (0)

1.000

3 (5)

1.000

CKD stage 3–5 (n, %)

8 (16)

0 (0)

0.087

0 (0)

< 0.005

HTN (n, %)

8 (16)

2 (4)

0.704

16 (25)

0.946

DM (n, %)

3 (6)

2 (4)

0.611

4 (6)

0.700

Stroke/TIA (n, %)

7 (14)

0 (0)

0.169

2 (3)

0.010

ECG

n = 48

  

n = 53

 

Abnormal (n, %)

33 (69)

13 (27)

1.000

43 (81)

0.114

AF/PAF (n, %)

3 (6)

0 (0)

0.550

3 (6)

0.664

PR interval (ms)

172 ± 36

145 ± 30

0.051

178 ± 39

0.557

QRS duration (ms)

135 ± 32

134 ± 32

0.939

116 ± 31

0.006

Echocardiography

n = 48

  

n = 62

 

LVH (n, %)

32 (67)

12 (25)

1.000

55 (89)

0.485

LA dilated (n, %)

24 (50)

10 (21)

1.000

30 (48)

0.060

CMR

n = 27

  

n = 28

 

LVEDV (ml)

158.2 ± 75.0

143.0 ± 18.3

0.791

145.0 ± 53.2

0.564

LVESV (ml)

73.0 ± 80.3

26.0

0.594

54.5 ± 44.9

0.386

LVEF (%)a

58 (53–65)

55 (43–65)

0.747

57 (55–64)

0.904

LVMi (g/m2)

143.6 ± 38.4

164.0 ± 45.0

0.379

102.4 ± 35.7

0.009

MWT (mm)

21.8 ± 5.2

21.3 ± 4.0

0.867

21.6 ± 5.5

0.965

LGE (n, %)d

14/16 (88)

5/9 (56)

0.072

25 (89)

1.000

 Extensive (> 3 AHA segments)

11/16 (69)

3/9 (33)

0.115

14 (50)

0.373

 Mild (1–2 AHA segment e.g. BIFL)

3/16 (19)

2/9 (22)

1.000

9 (32)

0.487

 RV insertion point

0 (0)

0 (0)

N/A

2 (7)

0.526

Biomarkers

High sensitive troponin T (ng/L)a

121 (51–154)

90 (44–272)

0.927

19 (13–38)

 < 0.001

NT-pro BNP (ng/l)a

1708 (626–4068)

1319 (719–1894)

0.667

888 (353–2070)

0.081

  1. Bold italics indicate statistically significant results (p < 0.05)
  2. Italics indicate the level of significance
  3. aNon-parametric data so presented as median (IQR)
  4. bp value comparing primary and secondary prevention device implantation in FD
  5. cp value comparing primary prevention device implantation in FD and HCM
  6. dProportions taken from patients who had a CMR and were given Gadolinium contrast
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172