Table 2.
Survival Estimates at Age One to 25
| Subtype | Article | N | Survival Estimates (95% CI) | |||||
|---|---|---|---|---|---|---|---|---|
| 1 year | 5 years | 10 years | 15 years | 20 years | 25 years | |||
| All congenital heart disease | Dastgiri et al20 | 1069 | 78.4 (75.8–80.8)a | 74.7 (73.8–75.5)b | ||||
| Jackson et al25 | 1543 | 86.1 (84.3–87.8)a | 82.0 (81.0–83.0) | 80.4 (79.5–81.7)b | ||||
| Meberg et al26 | 360 | 91.4 (88.0–94.1)a | 88.9 (85.2–91.9)a | |||||
| Moons et al28 | 921 | 96.0 (94.5–97.2)a | 95.6 (94.0–96.8)a | |||||
| Nembhard et al29 | 19 530 | 90.7 (90.2–91.1)a | ||||||
| Olsen et al30 | 6646 | 80 (79–81) | 76 (75–77)a | 75 (74–76) | 72 (70–73) | |||
| Samanek et al31 | 5030 | 80.0 (78.9–81.1) | 77.8 (76.6–79.0) | 77.4 (76.2–78.5) | 77.1 (75.9–78.3) | |||
| Tennant et al32 | 4281 | 92.3 (91.5–93.1) | 91.1 (90.2–91.9) | 90.8 (89.9–91.6) | 90.3 (89.3–91.2) | 89.5 (88.4–90.6) | ||
| Pooled estimate (95% CI) | 87.0 (82.1–91.2) | 85.4 (79.4–90.5) | 81.4 (73.8–87.9) | |||||
| Heterogeneity I2 & P–value | 99.0%, P<0.001 | 99.6%, P<0.001 | 99.5%, P<0.001 | |||||
| Ventricular septal defect | Garne23 | 195 | 96.9 (93.4, 98.9)a | |||||
| Moons et al28 | 303 | 99.3 (97.6–99.9)a | ||||||
| Nembhard et al29 | 10 382 | 93.9 (93.5–94.4)a | ||||||
| Olsen et al30 | 1559 | 94 (93–95) | 90 (89–91.7) | |||||
| Samanek et al31 | 2092 | 91.1 (89.8–92.3)a | 89.4 (88.0–90.7) | |||||
| Tennant et al32 | 1805 | 99.2 (98.7–99.5) | 99.1 (98.6–99.5) | 99.1 (98.5–99.4) | 99.1 (98.5–99.4) | 98.3 (96.6–99.1) | ||
| Pooled estimate (95% CI) | 95.5 (89.0–99.2) | 97.7 (93.5–99.8) | ||||||
| 95.5 (95.0–96.0) | ||||||||
| Heterogeneity I2 & P‐value | 99.0%, P<0.001 | 98.1%, P<0.001 | ||||||
| Pulmonary valve stenosis | Garne 23 | 33 | 97.0 (84.2–99.9)a | |||||
| Nembhard et al29 | 1170 | 91.6 (89.9–93.1)a | ||||||
| Samanek et al31 | 292 | 96.2 (94.0–98.5) | 95.6 (93.1–98.0) | 95.6 (93.1–98.0) | 95.6 (93.1–98.0) | |||
| Tennant et al32 | 382 | 98.7 (96.8–99.5) | 98.1 (96.1–99.1) | 98.1 (96.1–99.1) | 98.1 (96.1–99.1) | 98.1 (96.1–99.1) | ||
| Pooled estimate (95% CI) | 95.6 (91.1–98.6) | |||||||
| Heterogeneity I2 & P‐value | 89.6%, P<0.001 | |||||||
| Atrial septal defect | Garne23 | 78 | 98.7 (93.1, 100.0)a | |||||
| Moons et al28 | 162 | 99.4 (96.6–100.0)a | ||||||
| Nembhard et al29 | 9164 | 89.9 (89.3–90.5)a | ||||||
| Olsen et al30 | 361 | 93 (90–95.3) | 91 (88–95.6) | 84 (72–91) | ||||
| Samanek et al31 | 436 | 94.0 (92.4–96.3) | 92.9 (90.1–95.1)a | 92.9 (90.1–95.1)a | ||||
| Tennant et al32 | 365 | 97.3 (95.0–98.5) | 97.0 (94.6–98.3) | 97.0 (94.6–98.3) | 96.3 (93.3–98.0) | 96.3 (93.3–98.0) | ||
| Pooled estimate (95% CI) | 94.9 (92–97.2) | 96.8 (90.8–99.7) | 94.0 (89.9–97.1) | |||||
| 94.8 (93.5–96.0) | 95.4%, P<0.001 | 94.3 (92.7–95.6) | ||||||
| Heterogeneity I2 & P‐value | 77.4%, P<0.001 | 81.6%, P=0.004 | ||||||
| Aortic valve atresia/stenosis | Garne23 | 24 | 87.5 (67.6, 97.3)a | |||||
| Moons et al28 | 36 | 100.0 (90.3–100.0)a | ||||||
| Samanek31 | 391 | 90.3 (87.3–93.3) | 88.4 (85.1–91.7) | |||||
| Tennant et al32 | 171 | 92.4 (87.3–95.5) | 91.2 (85.9–94.6) | 91.2 (85.9–94.6) | 89.3 (83.2–3.3) | 89.3 (83.2–3.3) | ||
| Wang et al33 | 877 | 74.1 (71.0–77.0) | 73.4 (70.1–76.4) | |||||
| Wang et al35 | 2646 | 83.6 (82.1–84.9) | 81.5 (79.7–83.2) | |||||
| Pooled estimate (95% CI) | 88.7 (82.4–93.8) | 92.1 (81.3–98.4) | 84.4 (73.1–93.1) | |||||
| 85.0 (83.7–86.2) | 92.7%, P<0.001 | 82.2 (80.3–84.0) | ||||||
| Heterogeneity I2 & P‐value | 91.3%, P<0.001 | 96.8%, P<0.001 | ||||||
| Atrioventricular septal defect | Frid et al22 | 502 | 77.1 (73.2–80.7)a | 66.5 (62.2–70.7)a | 64.3 (59.9–68.5)a | 63.1 (58.8–67.4)a | ||
| Garne23 | 20 | 50 (27.2–72.8)a | ||||||
| Miller et al27 | 338 | 57.9 (49.7–65.3) | ||||||
| Moons et al28 | 37 | 91.9 (78.1–98.3)a | ||||||
| Olsen et al30 | 354 | 75 (70–79) | 65 (59–70) | 59 (51–65) | ||||
| Samanek et al31 | 201 | 62.2 (55.4–69.0) | 54.7 (47.7–61.8) | 54.2 (47.1–61.2) | 54.2 (47.1–61.2) | |||
| Tennant et al32 | 94 | 84.0 (74.9–90.1) | 80.9 (71.3–87.5) | 79.7 (70.1–86.6) | 79.7 (70.1–86.6) | 79.7 (70.1–86.6) | ||
| Wang et al33 | 1004 | 59.5 (56.3–62.6) | 58.1 (56.5–61.4) | 56.6 (52.8–60.2) | ||||
| Wang et al34 | 4884 | 80.1 (79.0–81.2) | 76.7 (75.3–78.1) | |||||
| Pooled estimate (95% CI) | 75.9 (70.5–81.0) | 71.2 (61.9–79.6) | 64.0 (57.2–70.5) | 63.4 (56.3–70.3) | ||||
| Heterogeneity I2 & P‐value | 89.0%, P<0.001 | 92.7%, P<0.001 | 81.4%, P<0.001 | 85.9%, P<0.001 | ||||
| Coarctation of aorta | Garne23 | 12 | 58.3 (27.7–84.8)a | |||||
| Moons et al28 | 46 | 91.3 (79.2–97.6)a | ||||||
| Nembhard et al29 | 1145 | 78.6 (76.1–80.9) | ||||||
| Olsen et al30 | 334 | 84 (79–87) | 82 (77–85) | 78 (61–82) | ||||
| Samanek et al31 | 266 | 68.0 (62.3–73.8) | 65.4 (59.6–71.3) | 65.0 (59.2–70.9) | 65.0 (59.2–70.8) | |||
| Tennant et al32 | 189 | 91.5 (86.6–94.7) | 91.5 (86.6–94.7) | 90.9 (85.8–94.3) | 90.9 (85.8–94.3) | 89.6 (83.7–93.5) | ||
| Wang et al33 | 2529 | 79.4 (77.8–81.0) | 77.0 (75.4–78.6) | 76.0 (74.3–77.7) | 75.2 (73.3–77.0) | |||
| Wang et al34 | 6365 | 84.5 (83.6–85.4) | 81.9 (80.7–83.0) | |||||
| Pooled estimate (95% CI) | 82.7 (75.4–89.0) | 81.0 (70.7–89.4) | 80.3 (65.0–92.0) | 78.2 (65.9–88.4) | ||||
| 79.5 (76.6–82.2) | 76.2 (74.6–77.7) | |||||||
| Heterogeneity I2 & P‐value | 93.7%, P<0.001 | 93.0%, P<0.001 | 87.3%, P<0.001 | 95.6%, P<0.001 | ||||
| Common arterial trunk | Moons et al28 | 7 | 85.7 (42.1–99.6)a | |||||
| Olsen et al30 | 78 | 45 (34–55) | 45 (34–55) | 45 (34–55) | 45 (34–55) | 45 (34–55) | 45(34–55) | |
| Samanek et al31 | 55 | 12.7 (3.7–21.7) | 10.5 (4.1–22.2)a | 7.3 (0–15.4) | 7.3 (0–15.4) | |||
| Tennant et al32 | 36 | 36.1 (21.0–51.4) | 36.1 (21.0–51.4) | 36.1 (21.0–51.4) | ||||
| Wang et al33 | 460 | 59.2 (54.4–63.6) | 55.2 (49.5–60.5) | |||||
| Wang et al35 | 956 | 75.1 (72.7–77.7) | ||||||
| Pooled estimate (95% CI) | 41.8 (14.1–72.6) | 47.4 (21.8–73.8) | 28.9 (16.3–43.3) | 36.5 (14.6–62) | ||||
| 35.4 (30.0–41.0) | 54.4(50.2–58.6) | |||||||
| Heterogeneity I2 & P‐value | 97.6%, P<0.001 | 96.3%, P<0.001 | 87.3%, P<0.001 | 94.5%, P<0.001 | ||||
| Pulmonary valve atresia (with IVS) | Fixler et al21 | 118 | 59.3 (49.9–67.6) | 55.7 (45.8–64.4) | ||||
| Idorn et al24 | 75 | 41.7 (30.1–53.3)a | 37.5 (26.4–49.2)a | 35.3 (24.0–46.5)a | 37.5 (26.4–49.2)a | 35.3 (24.0–46.5)a | 37.5 (26.4–49.2)a | |
| Moons et al28 | 6 | 83.3 (36.5–99.1)a | ||||||
| Samanek et al31 | 53 | 18.9 (8.1–29.6) | 7.6 (0.3–14.8) | 7.6 (0.3–14.8) | 7.6 (0.3–14.8) | |||
| Pooled estimate (95% CI) | 39.7 (18.5–63.3) | 41.1 (17.2–67.6) | ||||||
| 45.5 (39.2–52.0) | ||||||||
| Heterogeneity I2 & P‐value | 92.1%, P<0.001 | 92.0%, P<0.001 | ||||||
| Pulmonary atresia | Garne et al23 | 5 | 60.0 (14.7–94.7)a | |||||
| Pulmonary valve atresia (with VSD) | Moons et al28 | 6 | 67 (19–96)a | 50 (11.8–88.2)a | ||||
| Samanek et al31 | 55 | 61.8 (48.7–74.9) | 54.5 (41.1–68.0) | 45.2 (30.8–59.6) | 45.2 (30.8–59.6) | |||
| Tetralogy of Fallot | Garne23 | 7 | 82.6 (61.2–95.0)a | |||||
| Moons et al28 | 52 | 83 (70–92)a | 82.7 (69.7–91.8)a | |||||
| Olsen et al30 | 381 | 83 (79–87) | 70 (65–74) | 67 (58–74) | ||||
| Samanek et al31 | 169 | 84.6 (79.0–90.2) | 76.6 (70.1–83.2) | 76.6 (70.1–83.2) | ||||
| Tennant et al32 | 190 | 90.5 (85.4–93.9) | 83.7 (77.6–88.2) | 83.1 (76.9–87.7) | 83.1 (76.9–87.7) | 80.8 (72.8–86.6) | ||
| Wang et al34 | 5208 | 87.1 (86.1–87.9) | 84.7 (83.5–85.8) | |||||
| Wang et al34 | 1739 | 86.9 (85.3–88.4) | ||||||
| Pooled estimate (95% CI) | 86.3 (83.7–88.6) | 84.6 (83.5–85.7) | 81.4 (77.5–85) | |||||
| 81.6 (78.6–84.4) | ||||||||
| Heterogeneity I2 & P‐value | 0.0%, P=0.097 | 0.0%, P=0.957 | 36.1%, P=0.209 | |||||
| Total anomalous pulmonary venous return | Garne23 | 5 | 20 (0.5–71.6)a | |||||
| Samanek et al31 | 40 | 52.5 (36.7–8.23) | 50.0 (34.2–65.8) | 50.0 (34.2–65.8) | 50.0 (34.2–65.8) | |||
| Tennant et al32 | 54 | 72.2 (58.2–82.2) | 72.2 (58.2–82.2) | 72.2 (58.2–82.2) | 72.2 (58.2–82.2) | 72.2 (58.2–82.2) | ||
| Pooled estimate (95% CI) | 53.7 (30–76.6) | |||||||
| 61.2 (51.2–70.6) | ||||||||
| Heterogeneity I2 & P‐value | 76.6%, P=0.014 | |||||||
| Transposition of the great vessels | Garne23 | 21 | 76.2 (52.8, 91.8)a | |||||
| Moons et al28 | 29 | 100.0 (88.1–100.0)a | ||||||
| Olsen et al30 | 461 | 74 (70–78) | 62 (38–67) | 50 (41–59) | ||||
| Samanek et al31 | 271 | 61.6 (56.7–67.5) | 56.5 (50.3–62.4)a | 53.9 (46.8–60.9) | 53.9 (46.8–60.9) | |||
| Tennant et al32 | 189 | 82.5 (76.3–87.3) | 81.0 (74.6–85.9) | 80.3 (73.8–85.3) | 78.4 (71.6–83.9) | 74.1 (64.4–81.5) | ||
| Wang et al34 | 1840 | 74.5 (72.4–76.4) | ||||||
| Wang et al34 | 4330 | 83.7 (82.6–84.4) | 81.1 (79.7–82.4) | |||||
| Pooled estimate (95% CI) | 76.0 (65.5–85.1) | 81.9 (68.9–91.9) | 66.1 (46.0–83.5) | |||||
| 67.1 (62.5–71.5) | ||||||||
| Heterogeneity I2 & P‐value | 96.9%, P<0.001 | 95.9%, P<0.001 | 93.6%, P<0.001 | |||||
| Tricuspid atresia | Fixler et al21 | 67 | 76.1 (64.0–84.6) | 74.6 (62.4–83.4) | ||||
| Idorn et al24 | 106 | 68.0 (58.2–76.7)a | 61.7 (51.4–70.6)a | 60.5 (50.4–69.7)a | 57.4 (47.6–67.1)a | 57.4 (47.6–67.1)a | 57.4 (47.6–67.1)a | |
| Moons et al28 | 4 | 100 (39.8–100.0)a | 100 (39.8–100.0)a | |||||
| Samanek et al31 | 39 | 46.2 (30.2–62.1) | 35.9 (20.5–51.3) | 35.9 (20.5–51.3) | ||||
| Tennant et al32 | 24 | 83.3 (61.5–93.4) | 66.7 (44.3–81.7) | 62.5 (40.3–78.4) | 62.5 (40.3–78.4) | |||
| Pooled estimate (95% CI) | 71.4 (57.2–83.7) | 53.7 (30.0–76.6) | 53.1 (36.5–69.2) | 53.3 (37.2–69.1) | ||||
| 55.1 (47.3–62.9) | 56.2 (49.1–63.1) | |||||||
| Heterogeneity I2 & P‐value | 74.4%, P=0.004 | 93.9%, P<0.001 | 72.4%, P=0.027 | 72.9%, P=0.025 | ||||
| Hypoplastic left heart | Garne | 22 | 4.5 (0.1–22.8)a | |||||
| Idorn et al24 | 252 | 12.5 (8.9–17.5)a | 10.4 (6.9–14.8)a | 10.4 (6.9–14.8)a | 8.8 (5.6–12.9)a | |||
| Moons et al28 | 10 | 50 (18.7–81.3)a | 40.0 (12.2–73.8)a | |||||
| Samanek et al31 | 172 | 0 (0.0–2.1)a | 0 (0.0–2.1)a | 0 (0.0–2.1)a | 0 (0.0–2.1)a | |||
| Tennant et al32 | 73 | 4.1 (1.1–10.5) | 2.9 (0.5–8.9) | |||||
| Wang et al34 | 33.1 (30.6–35.7) | |||||||
| Wang et al34 | 2976 | 55.2 (53.4–56.9) | 50.6 (48.4–52.7) | |||||
| Pooled estimate (95% CI) | 17.4 (0.0–54.5) | 12.5 (0.0–41.4) | ||||||
| Heterogeneity I2 & P‐value | 99.5%, P<0.001 | 99.1%, P=0.036 | ||||||
| Single ventricle | Fixler et al21 | 286 | 64.7 (58.8–69.9) | 56.1 (49.9–61.7) | ||||
| Garne23 | 16 | 56.3 (29.9– 80.2)a | ||||||
| Moons et al28 | 9 | 56 (21–86)a | 55.6 (21.2–86.3)a | |||||
| Tennant et al32 | 31 | 83.9 (65.5–93.0) | 74.2 (55.0–86.2) | 74.2 (55.0–86.2) | 64.5 (43.1–80.0) | |||
| Pooled estimate (95% CI) | 70.4 (54.1–84.4) | 59.8 (50.4–68.8) | ||||||
| 69.5 (63.3–75.3) | ||||||||
| Heterogeneity I2 & P‐value | 65.0%, P=0.057 | 26.9%, P=0.250 | ||||||
| Ebstein's anomaly | Garne23 | 5 | 60.0 (14.7–94.7)a | |||||
| Moons et al28 | 3 | 100 (29.2–100.0)a | ||||||
| Nembhard et al29 | 160 | 68.8 (61.0–75.8)a | ||||||
| Samanek et al31 | 22 | 67.9 (50.2–86.5) | 64.3 (46.2–82.4) | 64.3(46.2–82.4) | 64.3(46.2–82.4) | |||
| Tennant et al32 | 55 | 67.3 (53.2–78.0) | 58.0 (43.8–69.7) | 58.0 (43.8–69.7) | 54.6 (39.7–67.2) | 54.6 (39.7–67.2) | ||
| Pooled estimate (95% CI) | 65.6 (57.5–73.2) | |||||||
| Heterogeneity I2 & P‐value | 18.0%, P=0.300 | |||||||
Pooled estimated are calculated using random effects meta‐analysis. But where there are ≤3 studies, pooled estimates are also calculated using fixed effects meta‐analysis with these results being shown in italics. AVA/S in Wang et al's studies refers to aortic valve stenosis only. IVS indicates intact ventricular septum; VSD, ventricular septal defect.
a
Indicates that 95% CIs were not reported in the study, but 95% binomial exact 95% CIs were calculated by the authors.
b
95% CIs obtained from author