TABLE 4.
First week mortality by case status among live births affected with ARM for all years available from 18 programs contributing to the study, ICBDSR
| Country-registry | Isolated ARMa |
Multiple ARMa |
Syndromic ARMa |
|||||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Total isolated cases |
Type of birth |
Mortality in LB |
Total multiple cases |
Type of birth |
Mortality in LB |
Total syndromic cases |
Type of birth |
Mortality in LB |
||||||||||
| ETOPFA |
SB |
LB |
Day 1 |
Day 2–6 |
ETOPFA |
SB |
LB |
Day 1 |
Day 2–6 |
ETOPFA |
SB |
LB |
Day 1 |
Day 2–6 |
||||
| n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | n (%) | |
| Argentina-RENACb | 212 (40.9) | − | 9 (4.2) | 203 (95.8) | 26 (12.8)c | 270 (52.0) | − | 23 (8.5) | 247 (91.5) | 115 (46.6)c | 37 (7.1) | − | 1 (2.7) | 36 (97.3) | 4 (11.1)c | |||
| Colombia-Bogotád | 19 (41.3) | − | 2 (10.5) | 17 (89.5) | 0 (0) | − | 15 (32.6) | − | 4 (26.7) | 11 (73.3) | 1 (9.1) | − | 12 (26.1) | − | 1 (8.3) | 11 (91.7) | 0 (0) | − |
| Colombia-Cali | 2 (100) | − | 0 (0) | 2 (100) | − | − | 0 (0) | − | 0 (0) | 0 (0) | − | − | 0 (0) | − | 0 (0) | 0 (0) | − | − |
| France-Paris | 106 (36.4) | 0 (0) | 1 (0.9) | 105 (99.1) | 2 (1.9) | 0 (0) | 134 (46.1) | 69 (51.5) | 6 (4.5) | 59 (44.0) | 6 (10.2) | 6 (10.2) | 51 (17.5) | 28 ( 54.9) | 4 (7.8) | 19 (37.3) | 0 (0) | 1 (5.3) |
| Germany-Saxony Anhalt | 100 (48.3) | 2 (2.0) | 3 (3.0) | 95 (95.0) | 0 (0) | 0 (0) | 93 (44.9) | 19 (20.4) | 8 (8.6) | 66 (71.0) | 2 (3.0) | 4 (6.1) | 14 (6.8) | 4 (28.6) | 1 (7.1) | 9 (64.3) | 1 (11.1) | 1 (11.1) |
| Israel-SMCe | 44 (83.0) | − | − | 44 (100) | 0 (0) | 1 (2.3) | 9 (17.0) | − | − | 9 (100) | 0 (0) | 0 (0) | 0 (0) | − | − | 0 (0) | 0 (0) | 0 (0) |
| Italy-Lombardy | 9 (15.5) | 0 (0) | 0 (0) | 9 (100) | 0 (0) | 0 (0) | 46 (79.3) | 8 (17.4) | 4 (8.7) | 34 (73.9) | 0 (0) | 0 (0) | 3 (5.2) | 0 (0) | 0 (0) | 3 (100) | 0 (0) | 0 (0) |
| Italy-Tuscany | 74 (53.6) | 0 (0) | 1 (1.4) | 73 (98.6 ) | 0 (0) | 1 (1.4) | 52 (37.7) | 6 (11.5) | 2 (3.9) | 44 (84.6) | 2 (4.6) | 2 (4.6) | 12 (8.7) | 8 (66.7) | 0 (0) | 4 (33.3) | 0 (0) | 0 (0) |
| Malta-MCARb | 11 (32.4) | − | 1 (9.1) | 10 (90.9) | 0 (0) | 0 (0) | 16 (47.1) | − | 5 (31.3) | 11 (68.8) | 0 (0) | 0 (0) | 7 (20.6) | − | 1 (14.3) | 6 (85.7) | 0 (0) | 0 (0) |
| Mexico-RYVEMCb | 215 (40.0) | − | 6 (2.8) | 209 (97.2) | 12 (5.7) | 2 (1.0) | 190 (35.3) | − | 20 (10.5) | 170 (89.5) | 51 (30.0) | 10 (5.9) | 133 (24.7) | − | 41 (30.8) | 92 (69.2) | 22 (23.9) | 10 (10.9) |
| Netherlands-Northern | 83 (36.9) | 2 (2.4) | 0 (0) | 81 (97.6) | 1 (1.2) | 1 (1.2) | 113 (50.2) | 23 (20.4) | 9 (8.0) | 81 (71.7) | 9 (11.1) | 16 (19.8) | 29 (12.9) | 1 (3.5) | 3 (10.3) | 25 (86.2) | 3 (12.0) | 2 (8.0) |
| Slovak Republic | 149 (56.4) | 0 (0) | 0 (0) | 149 (100) | 0 (0) | 1 (0.7) | 108 (40.9) | 0 (0) | 2 (1.9) | 106 (98.1) | 0 (0) | 20 (18.9) | 7 (2.7) | 0 (0) | 0 (0) | 7 (100) | 0 (0) | 2 (28.6) |
| South America | 511 (31.2) | − | 6 (1.2) | 505 (98.8) | 9 (1.8) | 11 (2.2) | 1,127 (68.8) | − | 153 (13.6) | 974 (86.4) | 335 (34.4) | 61 (6.3) | 0 (0) | − | − | − | − | − |
| Spain-ECEMCf | 255 (43.4) | − | 1 (0.4) | 254 (99.6) | 2 (0.8) | 0 (0) | 274 (46.7) | − | 29 (10.6) | 245 (89.4) | 45 (18.4) | 7 (2.9) | 58 (9.9) | − | 3 (5.2) | 55 (94.8) | 8 (14.5) | 2 (3.6) |
| Sweden | 688 (51.7) | 3 (0.4) | 1 (0.2) | 684 (99.4) | 4 (0.6) | 3 (0.4) | 524 (39.4) | 81 (15.5) | 8 (1.5) | 435 (83.0) | 21 (4.8) | 31 (7.1) | 118 (8.9) | 8 (6.8) | 1 (0.9) | 109 (92.4) | 6 (5.5) | 8 (7.3) |
| Ukraine-OMNI-Net | 51 (51.5) | 2 (3.9) | 1 (2.0) | 48 (94.1) | 1 (2.1) | 0 (0) | 46 (46.5) | 1 (2.2) | 7 (15.2) | 38 (82.6) | 1 (2.8) | 2 (5.6) | 2 (2.0) | 0 (0) | 0 (0) | 2 (100) | 0 (0) | 0 (0) |
| UK-Wales | 87 (33.1) | 1 (1.2) | 1 (1.2) | 85 (97.7) | 0 (0) | 0 (0) | 153 (58.2) | 65 (42.5) | 6 (3.9) | 82 (53.6) | 5 (6.1) | 5 (6.1) | 23 (8.8) | 11 (47.8) | 0 (0) | 12 (52.2) | 1 (8.3) | 2 (16.7) |
| USA-Utah | 96 (39.5) | 7 (7.3) | 1 (1.0) | 88 (91.7) | 7 (8.0) | 1 (1.1) | 117 (48.2) | 10 (8.6) | 2 (1.7) | 105 (89.7) | 5 (4.8) | 5 (4.8) | 30 (12.4) | 2 (6.7) | 0 (0) | 28 (93.3) | 2 (7.1) | 1 (3.6) |
| TOTAL | 2,712 (41.5) | 17 (1.2g) | 34 (1.3) | 2,661 (98.1) | 38 (1.4h) | 47 (1.8h,i) | 3,287 (50.3) | 282 (22.1g) | 288 (8.8) | 2,717 (82.7) | 483 (17.8h) | 284 (10.5h,i). | 536 (8.2) | 62 (21.5g) | 56 (10.4) | 418 (78.0) | 43 ( 10.3h) | 33 (7.9h,i) |
Note: Czech Republic, Iran-TROCA, Mexico-Nuevo Leon, US A-Arkansas, US A-Atlanta, USA-Texas not included in this analysis due to lack of information on case status of ARM.
Abbreviations: ECEMC, Registry of the Spanish Collaborative Study of Congenital Malformations; ECLAMC, Latin American Collaborative Study of Congenital Malformations; ETOPFA, Elective Termination of Pregnancy for Fetal Anomalies; MCAR, Malta Congenital Anomalies Registry; OMNI-Net, Ukraine Birth Defects Prevention Program; REN AC, National Network of Congenital Anomalies of Argentina; RYVEMCE, Mexican Registry and Epidemiological Surveillance of External Congenital Malformations; SMC, Soroka Medical Center; TROCA, Tabriz Registry of Congenital Anomalies; USA, United States of America.
A hyphen (–) means that the registry did not report follow-up data for that time period.
ETOPFA not allowed.
frequency and percentage refer to first week mortality.
ETOPFA not registered.
Data on liveborn children with ARM from one hospital.
Spain included information on ETOPFA from 1995 to 2014. ETOPFA not routinely registered in all the participating hospitals.
Excludes programs where ETOPFA is unavailable or does not report on ETOPFA.
Excludes programs that have no data on mortality for selected age at death.
Argentina is included under Day 2–6.