Outcomes of the subgroup with 22q11.2 deletion syndrome (Table
5):
22q11.2 deletion syndrome was present in 820 (27.2%) patients with
truncus arteriosus who had a surgical repair. Patients with 22q11.2
deletion syndrome had significantly prolonged median length of stay (32
days [IQR: 19–53] compared to 22 days [IQR: 12–37], p
< 0.001) and this was associated with significant increase in
median cost of hospitalization ($382,621 [IQR $231,360–$657,527]
versus $273,713 [IQR $163,272–$522,615], p < 0.001).
Lower mortality rates were observed in the 22q11.2 deletion syndrome
group (8.3% versus 11.7%, p = 0.007). The two groups (22q11.2 deletion
and no-22q11.2 deletion) had similar baseline characteristics and
comorbidities. Low birth weight, prematurity (gestational age
<37 weeks) were present in similar frequency in both groups.
Similarly, ECMO, cardiopulmonary resuscitation, mechanical ventilation,
and cardiac catheterization procedures were utilized at a similar rate
in both groups.
Of significance, the study finds that patients with 22q11.2 deletion
syndrome had a higher incidence of non-cardiac anomalies. More airway
anomalies were present in the 22q11.2 deletion group (10.9% vs. 5.9%,
OR = 1.94, 95% CI: 1.47–2.58, p < 0.001), chromosomal
anomalies other than 22q11.2 deletion were present at higher frequency
(7.3% vs. 3.7%, p < 0.001). In association with these
comorbidities, we observed that tracheostomy and G-tube placement were
performed more frequently in the 22q11.2 deletion group (5.4% vs.
2.7%, OR = 2.05, 95% CI: 1.37–3.05, p < 0.001, and 12.9%
vs. 4.5%, OR = 3.13, 95% CI: 2.35–4.18, p = 0.001, respectively).
Kidney anomalies, acute kidney injury, venous thrombosis, necrotizing
enterocolitis, and pulmonary hypertension were present in similar
frequency in both groups (Table 5).