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Table 1 Baseline clinical, echocardiographic and electrocardiographic features of 32 patients with MPS IVA

From: Cardiac features and effects of enzyme replacement therapy in Taiwanese patients with Mucopolysaccharidosis IVA

No.

Gender

Age (years)

LVMI (z score)

RVDd (z score)

IVSd (z score)

IVSs (z score)

LVIDd (z score)

LVIDs (z score)

LVPWd (z score)

LVPWs (z score)

AoD (z score)

LAD (z score)

EF

SF (%)

Reversed E/A ratio

MS

MR

AS

AR

MVP

Thick IVS

Electrocardiographic features

1

M

1.1

0.40

-0.07

1.83

-0.32

-0.18

-0.70

1.09

-1.22

0.52

-0.86

0.74

42%

-

0

1

0

0

+

-

Normal

2

M

1.3

-1.83

1.56

-0.16

-1.03

1.08

1.85

-1.61

-0.17

1.36

-0.10

0.60

31%

-

0

0

0

0

-

-

Sinus tachycardia

3

M

2.0

-0.11

2.18

2.25

0.34

-0.32

-1.55

-0.25

0.26

3.56

0.14

0.80

47%

-

0

0

0

0

-

-

NA

4

F

3.4

0.33

3.18

1.28

0.36

0.46

-0.58

-0.07

-1.36

1.71

-1.40

0.76

43%

-

0

0

0

0

-

-

Sinus tachycardia, RAD

5

F

3.7

-1.40

0.31

0.20

-0.23

-0.19

-0.60

-0.97

-1.06

1.31

1.36

0.74

41%

-

0

0

0

0

-

-

Normal

6

M

5.3

0.69

2.71

0.99

0.29

1.22

0.05

0.21

2.00

3.00

1.64

0.76

43%

-

1

0

1

0

-

-

Sinus tachycardia, RAD

7

M

5.8

1.34

0.50

2.24

-0.03

0.14

0.49

1.73

-2.64

1.64

-1.30

0.65

34%

-

0

0

0

0

-

-

Sinus tachycardia, septal hypertrophy

8

M

6.0

-0.43

0.98

0.14

-1.19

1.64

1.15

-0.71

-0.92

5.26

-0.05

0.71

40%

-

0

0

0

0

-

-

LAD

9

F

6.4

1.13

0.59

3.77

-0.52

-0.90

-0.26

1.60

-1.33

1.73

0.26

0.66

35%

-

0

0

0

0

-

-

Sinus arrhythmia

10

F

6.5

0.42

3.73

2.48

1.55

0.37

0.15

0.16

0.64

3.06

-0.24

0.69

38%

-

1

1

0

0

+

-

Normal

11

M

6.5

0.36

4.59

4.89

2.13

-1.00

-0.09

-0.07

-2.24

3.86

0.30

0.62

32%

-

0

0

0

0

-

-

RAD

12

F

8.0

0.17

3.50

0.45

0.03

0.83

0.31

-0.28

-0.82

3.94

1.12

0.71

40%

-

1

0

1

0

-

-

Sinus arrhythmia, low voltage

13

M

8.9

2.61

NA

5.47

1.56

1.43

-0.36

-0.19

1.52

4.00

4.80

0.79

46%

-

1

1.5

1

0

-

+

Sinus arrhythmia

14

M

9.0

-0.82

0.45

3.00

0.79

-1.29

-1.00

1.51

-0.82

2.18

1.88

0.69

37%

+

0

0

0

0

-

+

Sinus tachycardia, LAD

15

F

10.7

-0.72

0.59

3.51

1.05

-2.46

-2.92

1.06

-1.71

1.82

1.44

0.80

47%

-

1

0

1

0

+

+

Sinus tachycardia

16

M

10.7

1.62

-0.84

0.37

2.65

4.20

2.13

-1.45

-0.12

4.47

-0.21

0.76

45%

-

1

0

0

0

-

+

Sinus arrhythmia

17

F

10.8

-1.12

3.11

0.39

-0.46

-0.06

-0.08

-0.59

-0.14

0.94

-1.00

0.68

37%

-

0

0

0

0

-

-

Normal

18

F

11.1

-2.08

1.30

0.37

-1.39

-0.79

0.50

-0.75

-2.01

1.39

0.71

0.55

28%

-

0

0

0

0

-

-

Normal

19

F

11.5

-2.52

2.23

0.21

-0.27

-1.46

-2.40

-0.29

-0.32

1.36

-1.30

0.80

47%

-

0

0

0

0

-

-

Sinus arrhythmia

20

F

13.3

0.61

0.41

0.68

0.57

0.40

-0.19

0.86

0.48

4.53

1.20

0.73

41%

-

1

0

1

0

-

-

Sinus arrhythmia

21

F

13.6

-0.14

2.45

1.73

1.45

-0.50

-0.26

0.59

0.16

3.47

0.26

0.69

38%

-

1

1

1

0

+

-

Sinus arrhythmia

22

F

15.1

1.08

0.60

2.50

-0.46

0.48

-0.44

-0.18

0.76

4.18

0.70

0.74

42%

-

0

0

1

0

+

-

Sinus tachycardia

23

F

15.3

2.01

NA

2.45

1.94

2.32

0.16

-0.18

-0.58

4.71

0.03

0.78

46%

-

0

0

0

0

-

+

Sinus arrhythmia

24

M

15.7

0.45

0.73

1.73

1.45

1.54

0.04

0.37

0.41

3.64

-0.75

0.77

44%

-

0

0

0

0

-

-

NA

25

M

15.8

5.49

-0.13

4.63

6.26

4.03

1.08

1.84

0.76

4.65

-0.10

0.79

47%

-

0

0

0

0

-

-

NA

26

F

15.8

3.36

2.23

6.60

1.84

0.67

-0.21

1.37

1.67

6.40

0.34

0.76

43%

+

1

1

1

1

+

+

Sinus tachycardia, LAE

27

M

15.9

1.58

-0.32

1.60

0.88

3.20

1.75

-0.22

0.13

2.48

1.59

0.68

39%

-

0

0

0

0

-

-

NA

28

F

16.1

-0.72

1.20

1.14

-1.47

-0.27

-0.29

-0.75

-1.45

3.76

-0.68

0.67

37%

-

0

0

0

0

-

-

Sinus tachycardia

29

F

18.4

5.14

-2.50

15.07

5.20

-0.95

-0.65

4.71

1.62

2.82

0.04

0.68

36%

-

0

0.5

1

0

-

+

NA

30

M

21.3

2.08

-2.68

2.13

4.67

2.15

-0.96

-0.24

0.69

6.47

-1.16

0.85

54%

+

1

0

1

0

+

+

Sinus tachycardia, RAE

31

M

25.3

3.43

-0.13

4.51

2.29

1.02

-0.61

0.65

2.37

3.44

-1.90

0.78

46%

-

1

0

1

1.5

-

+

Sinus arrhythmia, IRBBB

32

M

29.1

7.65

3.57

8.00

2.84

1.03

-0.60

3.39

2.41

6.53

0.17

0.78

46%

+

1

1

2

2

-

+

Normal

  1. MPS mucopolysaccharidosis, LVMI left ventricular mass index, RVDd right ventricular end diastolic dimension, IVSd interventricular septum thickness in diastole, IVSs interventricular septum thickness in systole, LVIDd left ventricular internal diameter in diastole, LVIDs left ventricular internal diameter in systole, LVPWd left ventricular posterior wall thickness in diastole, LVPWs left ventricular posterior wall thickness in systole, AoD aortic diameter, LAD left atrial dimension, EF ejection fraction, SF shortening fraction, E/A ratio between early and late (atrial) ventricular filling velocity, MS mitral stenosis, MR mitral regurgitation, AS aortic stenosis, AR aortic regurgitation, MVP mitral valve prolapse, IVS interventricular septum, NA not available, RAD right axis deviation, LAD left axis deviation, LAE left atrial enlargement, RAE right atrial enlargement, IRBBB irregular bundle branch block. Severity of valvular stenosis and regurgitation (MS, MR, AS, AR) were estimated and graded on the following scores: 0 (none), 1 (mild), 2 (moderate), and 3 (severe)
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172