Table 1 Baseline characteristics.

From: Sarcomere gene variants did not improve cardiac function in pediatric patients with dilated cardiomyopathy from Japanese cohorts

 

Total (n = 123)

Age < 2 years old

(n = 86)

Age ≥ 2 years old

(n = 37)

P value

Male

62 (50.4%)

42 (48.8%)

20 (54.1%)

0.6950

Age at diagnosis (m)

8 (1–51)

3 (0–9)

113 (78–152)

 < 0.0001

Variant

45 (36.6%)

30 (34.9%)

15 (40.5%)

0.5486

Family history of CM

18 (15%)

10 (11.9%)

8 (22.2%)

0.1684

Outcome

    

HF requiring hospitalization

22 (20.4%)

16 (20.8%)

6 (19.4%)

1.0000

MACE

23 (18.7%)

17 (19.8%)

6 (16.2%)

0.8023

Death

18 (14.6%)

13 (15.1%)

5 (13.5%)

1.0000

Heart transplantation

6 (4.9%)

5 (5.8%)

1 (2.7%)

0.6672

ICD

0 (0%)

0 (0%)

0 (0%)

1.0000

Reason for diagnosis

    

Symptom

89 (72.4%)

66 (76.7%)

23 (62.2%)

0.1242

Fetal screening

10 (8.1%)

10 (11.6%)

0 (0%)

0.0316

Infantile screening

2 (1.6%)

2 (2.3%)

0 (0%)

1.0000

School screening

9 (7.3%)

0 (0%)

9 (24.3%)

 < 0.0001

Family screening

2 (1.6%)

1 (1.2%)

1 (2.7%)

0.5129

Perinatal abnormality

14 (13.3%)

14 (17.7%)

0 (0%)

0.0192

Treatment

111 (91.7%)

77 (91.7%)

34 (91.9%)

1.0000

Oral medication

103 (92.8%)

71 (92.2%)

32 (94.1%)

1.0000

ACE inhibitor

75 (61%)

52 (60.5%)

23 (62.2%)

1.0000

ARB

7 (5.7%)

5 (5.8%)

2 (5.4%)

1.0000

β blocker

77 (62.6%)

52 (60.5%)

25 (67.6%)

0.5439

Mineralocorticoid

83 (67.5%)

62 (72.1%)

21 (56.8%)

0.1410

Pacemaker implantation

1 (0.8%)

1 (1.3%)

2 (5.9%)

0.2289

Chest X-ray

    

Cardio thoracic ratio (%)

58.0 (51.5–64.0)

60.0 (54.8–65.0)

55.1 (45.9–59.3)

0.0017

Pulmonary congestion

46 (44.7%)

36 (49.3%)

10 (33.3%)

0.1907

Laboratory testing

    

BNP (pg/mL)

191.4 (22.0–1492)

243.2 (38.6–2000)

140.7 (13.8–606.7)

0.1662

Echocardiography

    

LVEF

33.5 (23.0–49.5)

33.6 (26.0–48.4)

32.0 (18.5–54.0)

0.6731

LVDD Z-score

5.1 (2.6–8.0)

6.7 (3.2–8.8)

3.7 (1.7–6.2)

0.0014

E/A

1.51 (1.17–1.97)

1.36 (1.11–1.88)

1.68 (1.47–2.18)

0.0183

E/e'

11.86 (8.95–16.79)

14.14 (9.26–17.86)

9.42 (7.17–13.95)

0.0065

LV Tei index

0.35 (0.30–0.54)

0.49 (0.31–0.76)

0.34 (0.25–0.42)

0.0828

Moderate and severe MR

61 (53.1%)

45 (56.3%)

24 (68.5%)

0.3010

Moderate and severe TR

23 (21.1%)

15 (19.5%)

8 (25.1%)

0.6076

TRPG

24.5 (19.1–33.3)

25.1 (19.9–34.3)

21.6 (16.8–32.4)

0.2279

  1. CM; cardiomyopathy, MACE; major adverse cardiac event, ICD; implantable Cardioverter Defibrillator, ACE; angiotensin converting enzyme, ARB; angiotensin receptor blocker, HF; heart failure, MACE; major adverse cardiac events, BNP; brain natriuretic peptide, LVEF; left ventricular ejection fraction, LVDD; left ventricular diastolic diameter, MR; mitral valve regurgitation, TR; tricuspid valve regurgitation, TRPG; pressure gradient of tricuspid regurgitation.
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