Table 1.
Characteristics of the 315 adults with congenital heart disease (CHD) studied, comparing those with and without 22q11.2 deletion syndrome (22q11.2DS)
| Variables | 22q11.2DS (n=86) |
No 22q11.2DS* (n=229) |
p Value |
|---|---|---|---|
| Female sex (%) | 39 (45.4%) | 108 (47.2%) | .8009* |
| Mean age in years at time of thoracic radiograph (SD) | 22.7 (5.0) | 26.9 (6.4) | <.0001 |
| CHD severity class† | |||
| Mild-moderate | 56 (65.1%) | 139 (60.7%) | 0.5163* |
| Severe | 30 (34.9%) | 90 (39.3%) | |
| Cardiac surgery before age 12 years‡ | |||
| Sternotomy only | 55 (64.0%) | 124 (54.2%) | 0.1270 |
| Thoracotomy (with/without sternotomy) | 15 (17.4%) | 83 (36.2%) | .0016 |
| No cardiac surgery or uncertain cardiac surgical approach | 16 (18.6%) | 22 (9.6%) | .0339 |
Significant findings are indicated in bold font.
By design, the no 22q11.2DS group was matched a priori to the 22q11.2DS group by sex and CHD severity class.
CHD severity class was determined following the 2018 guidelines from the American Heart Association and American College of Cardiology [22]; mild and moderate severity classes were combined given small numbers for the mild subgroup.
The surgical approach was determined based on the medical records. The patients could either fall in the sternotomy only group (first category), the lateral thoracotomy group with or without a sternotomy (second category) or in the group in which it was uncertain whether the patients had surgery paediatric cardiac and/or it was uncertain what kind of surgical approach was used (third category).