Table 1

Characteristics of children with severe congenital heart defects and death in a setting of palliative care,. Norway 2004–2016

IPCCC diagnosesnPrenatal detection, n (%)Refrainment from adequate cardiac surgery, n (%)Not offered cardiac surgery, n (%)Comorbidity, n (%)Prematurity,*
n (%)
Median time to death (days (range))
Hypoplastic left heart syndrome2812 (43)14 (50)14 (50)6 (21)6 (21)8(1–154)
Double outlet right ventricle117 (64)6 (55)5 (45)9 (82)7 (64)5(0–110)
Atrioventricular septal defect105 (50)5 (50)5 (50)8 (80)5 (50)5(0–618)
Ventricular septal defect92 (22)9 (100)0 (0)9 (100)2 (22)13(2–69)
Pulmonary atresia84 (50)1 (13)7 (88)6 (75)1 (13)10(4–59)
Coarctation of aorta and aortic arch hypoplasia41 (25)4 (100)0 (0)4 (100)2 (50)7(5–51)
Tricuspid valve disease and Ebstein’s anomaly44 (100)0 (0)4 (100)4 (100)2 (50)5(2–21)
Single ventricle42 (50)3 (75)1 (25)3 (75)1 (25)290(11–447)
Truncus arteriosus42 (50)3 (75)1 (25)4 (100)2 (50)15(12–36)
Tetralogy of Fallot40 (0)3 (75)1 (25)4 (100)1 (25)144(12–282)
Transposition of the great arteries31 (33)1 (33)2 (67)2 (67)1 (33)8(8–29)
Mitral valve disease31 (33)0 (0)3 (100)2 (67)0 (0)3(0–3)
Other†1711 (65)5 (29)12 (71)12 (71)3 (18)16(1–416)
Total10952 (48)54 (50)55 (50)73 (67)33 (30)9(0–618)
  • *Gestational age <37 weeks.

  • †Heterotaxia, aortic valve disease, anomalous pulmonary venous connection, congenitally corrected transposition of the great arteries, Shone’s syndrome, pulmonary venous stenosis, coronary artery anomaly and other complex defects.

  • IPCCC, International Paediatric and Congenital Cardiac Code.