Table 3, online only.
Characteristics of T21 patients and matched euploidic comparators
| Euploidic comparators | T21 | p-value | |
|---|---|---|---|
| N = 2,185 | N = 2,185 | ||
| Males N (%) | 1,055 (48.3) | 1,064 (48.7) | 0.809 |
| Surgical era N (%) | 0.674 | ||
| Early 1982–1992 | 629 (28.8) | 632 (28.9) | |
| Mid 1993–1997 | 720 (32.9) | 694 (31.8) | |
| Late 1998–2003 | 836 (38.3) | 859 (39.3) | |
| Diagnosis category N (%) | NC | ||
| ASD | 237 (10.9) | 237 (10.9) | |
| Incomplete AVC | 320 (14.6) | 320 (14.6) | |
| VSD simple | 929 (42.5) | 929 (42.5) | |
| VSD complex | 58 (2.6) | 58 (2.6) | |
| CAVC | 391 (17.9) | 391 (17.9) | |
| CAVC+ aortic arch obstruction | 37 (1.7) | 37 (1.7) | |
| TOF | 192 (8.8) | 192 (8.8) | |
| CAVC + TOF | 21 (1.0) | 21 (1.0) | |
| CHD complexity N (%) | 0.457 | ||
| Mild | 1,166 (53.4) | 1,157 (52.9) | |
| Moderate | 921 (42.1) | 945 (43.2) | |
| Severe | 98 (4.5) | 83 (3.8) | |
| Staged repair N (%) | 164 (7.5) | 137 (6.3) | 0.120 |
| Age at 1st surgery (Yrs) median (IQR) | 0.96 (0.39–3.51) | 0.61 (0.37–1.38) | <0.001 |
| Age at corrective surgery (Yrs) median (IQR) | 1.13 (0.46–3.71) | 0.67 (0.40–1.56) | < 0.001 |
| ECLS at surgery N (%) | 4 (0.2) | 1 (<0.1) | 0.375 |
| First permanent pacemaker N (%) | 61 (2.8) | 66 (3.0) | 0.719 |
| Postop < 6 months SND or heart block | 53 (86.9) | 50 (75.8) | 0.115 |
| Other indication1 | 8 (13.1) | 16 (24.2) | |
| Heart or Lung Transplant N (%) | 4 (0.2) | 1 (< 0.1) | 0.375 |
| Any LAVV reop after iAVC N (%) | 24 (7.5) | 17 (5.3) | 0.333 |
| Any LAVV reop after CAVC N (%) | 61 (14.2) | 32 (7.4) | 0.001 |
| Any reoperation N (%) | 304 (13.9) | 316 (14.5) | 0.633 |
| 30-year survival % (95% CI) | 93.1 (91.5–94.3) | 88.0 (86.0–89.7) | <0.001 |
| Age at death (Yrs) median (IQR) | 7.07 (0.93–21.57) | 6.76 (1.27–18.53) | 0.876 |
| Follow-up duration (Yrs) median (IQR) | 23.14 (19.7–27.29) | 22.70 (19.17–27.00) | 0.020 |
| Cause of death N (%) | 0.013 | ||
| CHD-related | 50 (40.7) | 58 (27.2) | |
| Other cardiac-related | 16 (13.1) | 51 (23.9) | |
| Not cardiac-related | 57 (46.3) | 104 (48.8) | |
| # Contributing COD/death event | |||
| Mean (SD) | 2.82 (1.52) | 3.31 (1.54) | 0.005 |
Given are frequency (N) and percent (%) for categorical variables, median and interquartile range (IQR) or mean and standard deviation (SD) for continuous variables. 30-year survival is Kaplan-Meier survival estimate. P values represent Fisher’s exact test for categorical variables, log-rank test, t-test or Wilcoxon rank-sum test for continuous variables.
Other indication included late (>6 months postoperative) sinus node dysfunction or heart block in 14 T21 patients and 5 euploidic comparators, preoperative sinus node dysfunction or heart block in 4, biventricular pacing in 1.
NC= not calculated, two groups were matched 1:1 on diagnosis category; ASD = atrial septal defect; iAVC = incomplete atrioventricular canal (partial and transitional; VSD = ventricular septal defect; CAVC = common atrioventricular canal; TOF = tetralogy of Fallot; ECLS = extracorporeal life support; LAVV = left atrioventricular valve; reop = reoperation including repair or replacement; SND = sinus node dysfunction; CHD = congenital heart disease; COD = cause of death