Table 2 Cardiac Features in RCM Subjects.

From: Molecular Characterization of Pediatric Restrictive Cardiomyopathy from Integrative Genomics

 

RCM1

RCM2

RCM3

RCM4

Echocardiography

    

LA Enlargement

+

+

+

+

Indexed LAV, ml/m^2

72

62

45

74

RA Enlargement

+

+

+

+

LV Dilation

–

–

–

–

LVEDD, mm (z-score)

3.7 (+0.2)

4.0 (+0.6)

5.1 (+0.3)

3.2 (−0.2)

LVH

-

-

-

-

IVS, mm (z-score)

0.6 (+0.1)

0.7 (+0.8)

0.8 (+0.1)

0.5 (−0.4)

PWT, mm (z-score)

0.7 (+1.0)

0.7 (+0.9)

0.7 (−0.3)

0.5 (−0.3)

Indexed LVM, g/m

36

32

32

34

LV Systolic Dysfx

–

–

–

–

LV FS, % (z-score)

35 (+0.1)

37 (+1.3)

35 (+1.0)

34 (−0.8)

LV Diastolic Dysfx

+

+

+

+

Catheterization

    

LVEDP, mmHg

18

17

14

20

RVEDP, mmHg

10

17

14

12

Mean RAP, mmHg

8

13

8

7

PA Hypertension

–

+

–

+

Mean PAP, mmHg

22

28

20

27

PWP, mmHg

16

20

15

16

Rp, indexed Woods U

0.9

2.4

1.3

4.5

Cardiac Index, L/min/m2

5.4

4.6

3.8

4.9

  1. Bold font indicates abnormal value; Dysfx, dysfunction (diastolic dysfunction as measured by patterns of mitral valve inflow); EDP end diastolic pressure; FS fractional shortening; LA left atrium; LAV left atrial volume (indexed to body surface area, normal <32 ml/m^2); LV left ventricle; LVM left ventricular mass (indexed to height ^2.7, normal <38-40 g/m); PA pulmonary artery; PAP pulmonary artery pressure; PWP pulmonary wedge pressure; RA right atrium; Rp pulmonary resistance.
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