Table 1.
Demographic, anatomic, and surgical characteristics of the cohorts. All variables listed were considered in modelling as potential correlates of Fontan Functional Score, unless otherwise noted in Methods.
| Study Cohort |
Modelling Cohort |
Validation Cohort |
|||
|---|---|---|---|---|---|
| Variables | Number missing | Mean ± SD, Median (Interquartile Range) or % | Mean ± SD, Median (Interquartile Range) or % | Mean ± SD, Median (Interquartile Range) or % | p value* |
| Number of subjects | 476 | 405 | 71 | ||
| Fontan Functional Score | 49.5 ± 17.3 | 49.4 ± 17.7 | 50.0 ± 14.9 | 0.803 | |
| Age at enrollment, year | 12.0 ± 3.4 | 12.0 ± 3.4 | 12.1 ± 3.3 | 0.825 | |
| Race | 1 | 0.133 | |||
| White | 382 (80.4%) | 319 (79.0%) | 63 (88.7%) | ||
| Black | 47 (9.9%) | 44 (10.9%) | 3 (4.2%) | ||
| Other | 46 (9.7%) | 41 (10.1%) | 5 (7.0%) | ||
| Male | 287 (60.3%) | 244 (60.2%) | 43 (60.6%) | 1.000 | |
| Parental highest grade of school | 6 | 0.391 | |||
| Some high school or less | 29 (6.2%) | 22 (5.5%) | 7 (10.3%) | ||
| High school graduate or GED | 117 (24.9%) | 105 (26.1%) | 12 (17.6%) | ||
| Vocational school, some college | 159 (33.8%) | 136 (33.8%) | 23 (33.8%) | ||
| 4 year college graduate | 110 (23.4%) | 93 (23.1%) | 17 (25.0%) | ||
| Graduate degree | 55 (11.7%) | 46 (11.4%) | 9 (13.2%) | ||
| Household income | 61 | 0.174 | |||
| <$20 000 | 52 (12.5%) | 46 (12.9%) | 6 (10.2%) | ||
| $20 000–39 000 | 76 (18.3%) | 70 (19.7%) | 6 (10.2%) | ||
| $40 000–59 000 | 69 (16.6%) | 53 (14.9%) | 16 (27.1%) | ||
| $60 000–79 000 | 63 (15.2%) | 54 (15.2%) | 9 (15.3%) | ||
| $80 000–99 000 | 58 (14.0%) | 51 (14.3%) | 7 (11.9%) | ||
| >$100 000 | 97 (23.4%) | 82 (23.0%) | 15 (25.4%) | ||
| Pulmonary atresia | 29 (6.1%) | 25 (6.2%) | 4 (5.6%) | 1.000 | |
| Hypoplastic left heart syndrome | 100 (21.0%) | 83 (20.5%) | 17 (23.9%) | 0.529 | |
| Common atrioventricular valve/Heterotaxy/Unbalanced atrioventricular septal defect | 52 (10.9%) | 42 (10.4%) | 10 (14.1%) | 0.355 | |
| Tricuspid atresia | 114 (24.0%) | 97 (24.0%) | 17 (23.9%) | 1.000 | |
| Totally anomalous pulmonary venous return | 8 (1.7%) | 5 (1.2%) | 3 (4.2%) | 0.103 | |
| Ventricular type | 0.671 | ||||
| Left | 244 (51.3%) | 211 (52.1%) | 33 (46.5%) | ||
| Right | 162 (34.0%) | 135 (33.3%) | 27 (38.0%) | ||
| Mixed | 70 (14.7%) | 59 (14.6%) | 11 (15.5%) | ||
| Pre-Fontan mean pulmonary arterial pressure, mmHg | 43 | 11.4 ± 3.3 | 11.4 ± 3.3 | 11.9 ± 3.3 | 0.200 |
| Pre-Fontan end-diastolic pressure, mmHg | 22 | 7.8 ± 3.3 | 7.8 ± 3.4 | 8.0 ± 2.6 | 0.562 |
| Pre-Fontan oxygen saturation, percent | 13 | 84 ± 5.2 | 84.0 ± 5.0 | 84.3 ± 6.4 | 0.694 |
| Age at volume unloading surgery (either superior cavopulmonary anatomosis or Fontan), year | 6 | 1.0 (0.6, 2.3) | 1.0 (0.6, 2.3) | 1.1 (0.6, 2.2) | 0.949 |
| Superior cavopulmonary anatomosis | 351 (73.7%) | 297 (73.3%) | 54 (76.1%) | 0.770 | |
| Number of pulmonary arterial surgeries | 0.4 ± 0.7 | 0.4 ± 0.7 | 0.5 ± 0.7 | 0.819 | |
| Moderate/Severe atrioventricular valvar regurgitation | 48 | 20 (4.7%) | 17 (4.7%) | 3 (4.4%) | 1.000 |
| Moderate/Severe ventricular dysfunction | 31 | 6 (1.4%) | 5 (1.4%) | 1 (1.5%) | 1.000 |
| Age at Fontan, year | 2.9 (2.1, 4.0) | 2.9 (2.1, 3.9) | 3.0 (1.9, 5.0) | 0.657 | |
| Weight for age z score at Fontan | 8 | −1.0 ± 1.2 | −1.0 ± 1.2 | −0.9 ± 1.2 | 0.457 |
| Fenestration | 319 (67.0%) | 262 (64.7%) | 57 (80.3%) | 0.009 | |
| Cardpulmonary bypass time, minutes | 37 | 113.9 ± 47.4 | 114.0 ± 48.1 | 113.0 ± 43.4 | 0.865 |
| Type of Fontan | 0.346 | ||||
| Atriopulmonary connection | 67 (14.1%) | 61 (15.1%) | 6 (8.5%) | ||
| Total cavopulmonary connection intracardiac lateral tunnel | 287 (60.3%) | 244 (60.2%) | 43 (60.6%) | ||
| Total cavopulmonary connection extracardiac lateral tunnel | 53 (11.1%) | 41 (10.1%) | 12 (16.9%) | ||
| Total cavopulmonary connection extracardiac conduit | 60 (12.6%) | 51 (12.6%) | 9 (12.7%) | ||
| Other | 9 (1.9%) | 8 (2.0%) | 1 (1.4%) | ||
| Post-operation complication: Prolonged pleural/pericardial effusions/chylothorax | 177 (37.6%) | 149 (37.3%) | 28 (39.4%) | 0.791 | |
| Length of hospital stay, days | 9 | 11 (9, 18) | 12 (9, 18) | 11 (8, 18) | 0.454 |
| Post-Fontan arrhythmia | 101 (21.2%) | 86 (21.2%) | 15 (21.1%) | 1.000 | |
| Post-Fontan surgeries ≥2 | 13 (2.7%) | 12 (3.0%) | 1 (1.4%) | 0.702 | |
| Number of post-Fontan catheterization interventions | 0.8 ± 1.1 | 0.7 ± 1.1 | 1.0 ± 1.2 | 0.107 | |
| Aortic arch repair | 1 (0.2%) | 0 (0.0%) | 1 (1.4%) | 0.149 | |
| Pulmonary arterial surgeries | 4 (0.8%) | 4 (0.9%) | 0 (0.0%) | 1.000 | |
| Number of years since Fontan | 8.7 ± 3.4 | 8.8 ± 3.4 | 8.4 ± 3.7 | 0.398 | |
| Currently on pacemaker | 64 (13.5%) | 51 (12.6%) | 13 (18.3%) | 0.191 | |
*
Test of significance for differences between model sample (405 subjects) and validation sample (71 subjects).