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Table 1 Cardiac and extracardiac abnormalities in the patients with Htx

From: Rare copy number variants analysis identifies novel candidate genes in heterotaxy syndrome patients with congenital heart defects

 

Number of patients (%)

Sex

 Male

40 (63.5%)

 Female

23 (36.5%)

Cardiac position

 Levocardia

15 (23.8%)

 Dextrocardia

35 (55.6%)

 Mesocardia

13 (20.6%)

Atrial arrangement

 Atrial situs inversus

20 (31.7%)

 Isomerism of right atrial appendages

33 (52.4%)

 Isomerism of left atrial appendages

7 (11.1%)

Ventricular arrangement

 Ventricular situs solitus

14 (22.2%)

 Ventricular situs inversus

16 (25.4%)

 Single ventricle (morphologic right)

23 (36.5%)

 Single ventricle (morphologic left)

3 (4.8%)

 Single ventricle (morphologic indeterminate)

5 (7.9%)

 Other abnormal ventricle arrangement

2 (3.2%)

Bronchi

 Bilateral right bronchi (short)

34 (54.0%)

 Bilateral left bronchi (long)

7 (11.1%)

 Bronchial inversus

22 (34.9%)

Spleen

 Polysplenia

6 (9.5%)

 Asplenia

29 (46.0%)

 Single right spleen

21 (33.3%)

 Single left spleen

7 (11.1%)

Stomach

 Right-sided stomach

38 (60.3%)

 Left-sided stomach

19 (30.2%)

 Stomach centrally situated

4 (6.3%)

 Unknown

2 (3.2%)

Liver

 Left-sided liver

23 (36.5%)

 Liver centrally situated

31 (49.2%)

Aortic arch

 Left aortic arch

17 (27.0%)

 Right aortic arch

45 (71.4%)

 Aortic arch centrally descending

1 (1.6%)

SVC

 Right SVC

8 (12.7%)

 Left SVC

33 (52.4%)

 Bilateral SVC

22 (34.9%)

IVC

 Interrupted IVC, hemiazygos vein continuation

1 (1.6%)

 Interrupted IVC, azygos vein continuation

6 (9.5%)

Relationship of IVC and descending aorta

 IVC right of spine and descending aorta left of spine

4 (6.3%)

 IVC left of spine and descending aorta right of spine

17 (27.0%)

 IVC and descending aorta same side

32 (50.8%)

 IVC left of spine and descending aorta anterior of spine

2 (3.2%)

 IVC anterior of spine and descending aorta left of spine

1 (1.6%)

  1. SVC superior vena cava, IVC inferior vena cava