Skip to main content

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