TABLE 3.
Risk of cancer death in the PCCC cohort among patients without genetic abnormalities and those with a Down syndrome diagnosis (N = 55,018).
| Non-genetic | Down syndrome | |||||||
|---|---|---|---|---|---|---|---|---|
| Risk factor | At risk N = 50,069 |
Cancer deaths N = 152 |
aHR | 95% CI | At risk N = 4949 |
Cancer deaths N = 42 |
aHR | 95% CI |
| Overall | 1.0 | – | 3.63 | 2.52–5.24 | ||||
| Demographic | ||||||||
| Sex | ||||||||
| Female | 23,157 | 64 | 1.0 | – | 2574 | 20 | 1.0 | – |
| Male | 26,912 | 88 | 1.15 | 0.83–1.59 | 2375 | 22 | 1.17 | 0.64–2.15 |
| Race | ||||||||
| White | 14,693 | 52 | 1.0 | – | 1537 | 12 | 1.0 | – |
| Black | 2883 | 10 | 1.05 | 0.53–2.07 | 343 | 0 | N/A | – |
| Othera | 521 | 3 | N/A | – | 47 | 2 | N/A | – |
| Unknown | 31,972 | 87 | 0.68 | 0.44–1.04 | 3022 | 28 | 1.05 | 0.46–2.42 |
| Ethnicity | ||||||||
| Not Hispanic | 15,118 | 49 | 1.0 | – | 1627 | 11 | 1.0 | – |
| Hispanic | 1358 | 3 | N/A | – | 169 | 7 | 5.43 | 1.87–15.77 |
| Unknown | 33,593 | 100 | 1.32 | 0.86–2.05 | 3153 | 24 | 1.03 | 0.43–2.46 |
| Clinical | ||||||||
| Primary diagnosis | ||||||||
| PDA | 4583 | 14 | Ref | – | 294 | 3 | Ref | – |
| Left to right shuntb | 11,200 | 19 | 0.53 | 0.26–1.05 | 3968 | 31 | 1.02 | 0.30–3.44 |
| LHOL | 7765 | 21 | 0.74 | 0.37–1.49 | 69 | 0 | N/A | – |
| APVR | 1810 | 6 | 1.03 | 0.39–2.72 | 7 | 0 | N/A | – |
| RVOTO | 7325 | 14 | 0.58 | 0.27–1.23 | 280 | 4 | 1.38 | 0.30–6.42 |
| TGA physiology | 3423 | 8 | 0.69 | 0.28–1.73 | 7 | 0 | N/A | – |
| Truncus arteriosus | 556 | 1 | N/A | 0.09–5.52 | 5 | 0 | N/A | – |
| Miscellaneousc | 7332 | 30 | 1.25 | 0.65–2.39 | 231 | 3 | 1.09 | 0.21–5.66 |
| Single ventricle | 6075 | 39 | 2.74 | 1.43–5.24 | 88 | 1 | 1.45 | 0.14–15.38 |
| CHD severity | ||||||||
| Mild | 13,988 | 28 | Ref | – | 1667 | 8 | Ref | – |
| Moderate | 16,396 | 34 | 1.01 | 0.61–1.67 | 3036 | 29 | 2.52 | 1.13–5.58 |
| Severe 2V | 8522 | 29 | 1.82 | 1.04–3.20 | 111 | 3 | N/A | – |
| Single ventricle | 6075 | 39 | 4.68 | 2.77–7.91 | 88 | 1 | N/A | – |
| Unclassifiable | 5088 | 22 | 2.01 | 1.13–3.58 | 47 | 1 | 5.41 | 0.65–44.98 |
| Cyanotic | ||||||||
| No | 29,053 | 74 | Ref | – | 4494 | 34 | Ref | – |
| Yes | 17,483 | 66 | 1.62 | 1.13–2.32 | 427 | 8 | 1.94 | 0.84–4.50 |
| Unclassifiable | 3533 | 12 | 1.37 | 0.74–2.53 | 28 | 0 | N/A | – |
| Age at first intervention | ||||||||
| Neonate (0 to <28 days) | 12,078 | 41 | Ref | – | 321 | 7 | Ref | – |
| Infant (28 days to <1 year) | 12,271 | 30 | 0.61 | 0.38–0.97 | 3141 | 17 | 0.19 | 0.08–0.47 |
| Young child (1 to <10 years) | 18,856 | 51 | 0.61 | 0.40–0.92 | 1220 | 15 | 0.39 | 0.16–0.95 |
| Older child (10 to <21 years) | 6864 | 30 | 1.05 | 0.65–1.69 | 267 | 3 | N/A | – |
Note: N/A = not available because of wide confidence intervals and interpretability issues when <5 deaths contributed to aHRs. Numbers in bold indicate statistical significance at p < .05.
Abbreviations: aHR, adjusted hazard ratio; APVR, anomalous pulmonary venous return; CHD, congenital heart defect; CI, confidence interval; LHOL, left heart obstructive lesion; PCCC, Pediatric Cardiac Care Consortium; PDA, patent ductus arteriosus; RVOTO, right ventricular outflow tract obstruction; TGA, transposition of the great arteries.
Includes “Asian,” “Native American,” “More than one,” “Pacific Islander,” and “Other.”
Other than PDA.
Miscellaneous lesions not classifiable by a single major pathophysiology.