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 syndrome 28 12 (43) 14 (50) 14 (50) 6 (21) 6 (21) 8 (1–154)
Double outlet right ventricle 11 7 (64) 6 (55) 5 (45) 9 (82) 7 (64) 5 (0–110)
Atrioventricular septal defect 10 5 (50) 5 (50) 5 (50) 8 (80) 5 (50) 5 (0–618)
Ventricular septal defect 9 2 (22) 9 (100) 0 (0) 9 (100) 2 (22) 13 (2–69)
Pulmonary atresia 8 4 (50) 1 (13) 7 (88) 6 (75) 1 (13) 10 (4–59)
Coarctation of aorta and aortic arch hypoplasia 4 1 (25) 4 (100) 0 (0) 4 (100) 2 (50) 7 (5–51)
Tricuspid valve disease and Ebstein’s anomaly 4 4 (100) 0 (0) 4 (100) 4 (100) 2 (50) 5 (2–21)
Single ventricle 4 2 (50) 3 (75) 1 (25) 3 (75) 1 (25) 290 (11–447)
Truncus arteriosus 4 2 (50) 3 (75) 1 (25) 4 (100) 2 (50) 15 (12–36)
Tetralogy of Fallot 4 0 (0) 3 (75) 1 (25) 4 (100) 1 (25) 144 (12–282)
Transposition of the great arteries 3 1 (33) 1 (33) 2 (67) 2 (67) 1 (33) 8 (8–29)
Mitral valve disease 3 1 (33) 0 (0) 3 (100) 2 (67) 0 (0) 3 (0–3)
Other† 17 11 (65) 5 (29) 12 (71) 12 (71) 3 (18) 16 (1–416)
Total 109 52 (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.