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. 2023 Jun 23;7(1):e001617. doi: 10.1136/bmjpo-2022-001617

Table 2.

Percentages of children with congenital anomalies (CA) with 95% CI according to underlying cause of death by age at death (anomalies with <10 deaths occurring are not included)

Anomaly No deaths (100%) Percentage cause of death (95% CI) Predicted proportion attributable to the CA
CA Infections Immaturity Other Missing cause of death
Neonatal deaths
 All anomalies 4199 71 (64 to 78) 1 (1 to 2) 7 (6 to 9) 11 (8 to 14) 9 (7 to 12) 93
 Spina Bifida (isolated cases with or without hydrocephalus) 19 79 (39 to 95) 0 (0 to 0) 11 (3 to 22) 5 (1 to 19) 5 (1 to 19) 97
 Hydrocephalus 138 64 (43 to 80) 1 (0 to 4) 7 (4 to 12) 14 (8 to 20) 14 (8 to 20) 97
 Congenital heart defects (CHD) 1937 79 (71 to 86) 1 (1 to 2) 3 (2 to 5) 9 (7 to 13) 7 (5 to 9) 94
 Severe CHD 1254 86 (78 to 91) 0 (0 to 1) 2 (1 to 3) 6 (4 to 9) 7 (4 to 10) 98
 Transposition of great vessels (as only severe CHD) 106 91 (66 to 98) 1 (0 to 5) 2 (0 to 7) 4 (1 to 12) 3 (1 to 10) 97
 VSD (without severe CHD) 291 75 (58 to 86) 2 (1 to 5) 5 (3 to 9) 13 (8 to 19) 5 (3 to 9) 89
 ASD (without severe CHD) 153 68 (45 to 84) 4 (1 to 8) 9 (4 to 15) 14 (7 to 22) 6 (3 to 12) 90
 AVSD 153 85 (65 to 94) 0 (0 to 0) 5 (2 to 11) 6 (2 to 14) 5 (2 to 11) 97
 Tetralogy of Fallot 63 94 (78 to 98) 0 (0 to 0) 0 (0 to 0) 5 (2 to 13) 2 (0 to 10) 94
 Coarctation of aorta 188 87 (75 to 93) 0 (0 to 0) 2 (1 to 5) 7 (4 to 12) 4 (2 to 8) 96
 Cleft lip with or without cleft palate 128 91 (71 to 98) 0 (0 to 0) 4 (1 to 13) 2 (1 to 8) 2 (0 to 7) 89
 Cleft palate 121 68 (46 to 83) 1 (0 to 5) 12 (6 to 18) 11 (6 to 17) 9 (5 to 15) 92
 Oesophageal atresia 110 82 (64 to 92) 0 (0 to 0) 5 (2 to 10) 6 (3 to 12) 7 (3 to 14) 97
 Diaphragmatic hernia (isolated cases) 186 89 (76 to 96) 0 (0 to 0) 2 (1 to 5) 2 (1 to 6) 6 (3 to 13) 99
 Gastroschisis (isolated cases) 25 92 (61 to 99) 4 (1 to 20) 0 (0 to 0) 0 (0 to 0) 4 (1 to 20) 94
 Multicystic renal dysplasia 92 67 (42 to 85) 1 (0 to 6) 9 (4 to 15) 10 (5 to 17) 13 (6 to 20) 96
 Down syndrome 102 53 (33 to 72) 4 (2 to 7) 10 (6 to 15) 26 (17 to 34) 7 (3 to 11) 82
 Trisomy 13 156 90 (76 to 96) 0 (0 to 0) 1 (0 to 5) 2 (1 to 6) 6 (3 to 13) 100
 Trisomy 18 330 93 (80 to 98) 0 (0 to 0) 1 (0 to 4) 1 (0 to 4) 5 (2 to 12) 100
Anomaly No deaths (100%) CA Infections Immaturity Other Missing cause of death Predicted proportion attributable to the CA
Postneonatal deaths
 All anomalies 2100 68 (61 to 74) 5 (4 to 6) 4 (3 to 5) 20 (16 to 22) 4 (3 to 5) 94
 Hydrocephalus 85 55 (38 to 70) 7 (4 to 11) 6 (3 to 10) 29 (22 to 34) 2 (1 to 7) 98
 Congenital heart defect 1329 75 (68 to 82) 4 (3 to 6) 3 (2 to 4) 15 (11 to 18) 3 (2 to 4) 97
 Severe CHD 773 87 (79 to 93) 3 (2 to 5) 1 (0 to 2) 6 (4 to 10) 3 (2 to 6) 99
 Transposition of great vessels (as only severe CHD) 27 100 (100 to 100) 0 (0 to 0) 0 (0 to 0) 0 (0 to 0) 0 (0 to 0) 97
 VSD (without severe CHD) 289 67 (55 to 77) 5 (3 to 8) 5 (3 to 8) 20 (15 to 25) 2 (1 to 4) 96
 ASD (without severe CHD) 169 56 (41 to 69) 2 (1 to 5) 8 (5 to 11) 30 (23 to 35) 4 (2 to 7) 96
 AVSD 199 84 (67 to 93) 4 (2 to 7) 1 (0 to 3) 9 (5 to 16) 3 (1 to 6) 99
 Tetralogy of Fallot 85 98 (66 to 100) 1 (0 to 9) 0 (0 to 6) 1 (0 to 12) 0 (0 to 6) 97
 Coarctation of aorta 130 86 (67 to 95) 5 (2 to 11) 0 (0 to 0) 6 (2 to 12) 4 (1 to 10) 98
 Cleft lip with or without cleft palate 53 66 (45 to 80) 2 (0 to 9) 0 (0 to 0) 26 (17 to 33) 6 (2 to 12) 88
 Cleft palate 76 70 (49 to 84) 3 (1 to 8) 3 (1 to 8) 16 (10 to 21) 9 (5 to 15) 95
 Oesophageal atresia 45 66 (39 to 84) 5 (1 to 11) 2 (0 to 10) 18 (10 to 24) 9 (4 to 16) 97
 Diaphragmatic hernia (isolated cases) 12 83 (39 to 98) 8 (1 to 30) 0 (0 to 0) 8 (1 to 30) 0 (0 to 0) 97
 Gastroschisis (isolated cases) 20 82 (31 to 98) 5 (1 to 17) 5 (1 to 17) 5 (1 to 17) 5 (1 to 17) 94
 Multicystic renal dysplasia 11 41 (5 to 90) 0 (0 to 0) 24 (4 to 41) 36 (6 to 54) 0 (0 to 0) 72
 Down syndrome 165 71 (53 to 84) 6 (3 to 10) 5 (2 to 8) 16 (9 to 23) 2 (1 to 5) 96
 Trisomy 13 35 94 (69 to 99) 0 (0 to 0) 0 (0 to 0) 3 (0 to 15) 3 (0 to 15) 100
 Trisomy 18 136 93 (77 to 98) 0 (0 to 0) 1 (0 to 5) 1 (0 to 6) 5 (2 to 13) 100
Anomaly No deaths 1–9 years (100%) CA Infections Neoplasms Nervous Other Missing cause of death Predicted proportion attributable to the CA
Child deaths (1–9 years)
 All anomalies 1087 49 (42 to 57) 9 (8 to 10) 5 (4 to 6) 9 (8 to 11) 23 (21 to 26) 4 (3 to 5) 99
 Hydrocephalus 66 49 (25 to 73) 13 (7 to 18) 7 (3 to 11) 10 (5 to 15) 17 (10 to 21) 5 (2 to 10) 100
 Congenital heart defect 474 63 (54 to 72) 8 (6 to 10) 3 (2 to 4) 4 (3 to 5) 19 (16 to 22) 3 (2 to 5) 99
 Severe CHD 258 79 (67 to 87) 5 (3 to 8) 1 (0 to 3) 2 (1 to 4) 10 (7 to 13) 3 (2 to 6) 100
 VSD (without severe CHD) 100 48 (31 to 66) 13 (9 to 17) 6 (3 to 10) 6 (3 to 10) 24 (18 to 27) 2 (1 to 6) 99
 ASD (without severe CHD) 81 44 (28 to 61) 10 (6 to 13) 7 (4 to 11) 2 (1 to 6) 31 (25 to 32) 5 (2 to 9) 99
 AVSD 70 79 (60 to 90) 6 (2 to 12) 0 (0 to 0) 0 (0 to 0) 11 (6 to 18) 4 (2 to 10) 99
 Tetralogy of Fallot 51 71 (42 to 87) 10 (5 to 15) 2 (0 to 8) 2 (0 to 8) 12 (6 to 17) 4 (1 to 10) 100
 Coarctation of aorta 44 70 (43 to 87) 5 (1 to 12) 0 (0 to 0) 2 (0 to 10) 14 (7 to 20) 9 (4 to 16) 99
 Cleft lip with or without cleft palate 12 65 (7 to 98) 17 (1 to 46) 0 (0 to 0) 0 (0 to 0) 17 (1 to 46) 0 (0 to 0) 99
 Cleft palate 41 67 (34 to 89) 5 (1 to 12) 0 (0 to 0) 5 (1 to 12) 16 (7 to 27) 7 (2 to 15) 91
 Oesophageal atresia 23 47 (10 to 88) 11 (2 to 19) 0 (0 to 0) 11 (2 to 19) 26 (7 to 37) 5 (1 to 15) 99
 Down syndrome 98 42 (28 to 56) 14 (10 to 18) 17 (13 to 21) 0 (0 to 0) 24 (20 to 28) 2 (1 to 6) 99
 Trisomy 18 17 94 (24 to 100) 6 (0 to 76) 0 (0 to 0) 0 (0 to 0) 0 (0 to 0) 0 (0 to 0) 99

ASD, atrial septal defect; AVSD, atrioventricular septal defect; VSD, ventricular septal defect.