Outcomes in Adult Congenital Heart Disease Patients With Down Syndrome Undergoing a Cardiac Surgical Procedure

As the life expectancy of patients with Down syndrome (DS) improves, the number of older patients with DS who require a cardiac surgical procedure for congenital heart disease will increase. Perioperative risk factors and outcomes in these patients are unknown.

In a multicenter retrospective study, teenaged and adult patients with DS who underwent a cardiac surgical procedure between 2008 and 2018 were matched by age and surgical procedure with patients who did not have DS. Demographic, medical, and surgical characteristics were compared. Outcome measures were length of stay (LOS), duration of mechanical ventilation, need for noninvasive positive pressure ventilation and reintubation, additional cardiac interventions, postoperative infections, and early postoperative mortality. Risk factors for extended hospital LOS (>10 days) were explored using multivariable logistic regression.

The study compared 121 patients with DS with 121 patients who did not have DS. Patients with DS had a longer median LOS (7 days vs 5 days; P < .001), a longer duration of mechanical ventilation (12.5 hours vs 6.7 hours; P < .001), greater need for noninvasive positive pressure ventilation or reintubation (26% vs 4%; P < .001), and a higher likelihood of postoperative infections (10% vs 2%; P = .035). There was no early mortality. Preoperative risk factors for extended LOS for patients with DS included pulmonary medication use (odds ratio [OR], 4.0; P = .046), a history of immunodeficiency (OR, 10.4; P = .004), or moderate or greater tricuspid regurgitation (OR, 12.7; P < .001).

Teenaged and adult patients with DS who underwent congenital a cardiac surgical procedure had a longer hospital LOS and more postoperative respiratory and infectious complications compared with patients who did not have DS, without increased mortality. A cardiac surgical procedure can be performed safely in older patients with DS. Management of pulmonary disease, immunodeficiency, and tricuspid regurgitation may mitigate risk.