O1014: Longitudinal Outcomes in Children With Trisomy 21 and Congenital Heart Disease after Cardiac Surgery.

Background: Congenital Heart disease (CHD) occurs in 40-60% of children with Trisomy 21, the majority requiring surgical intervention. Studies have shown excellent short and long-term survival in Trisomy 21 patients after cardiac surgery. However, the literature on morbidities and reinterventions is limited.

Methods: The study includes patients with Trisomy 21 and CHD who underwent cardiac surgery between January 2008 and December 2020 at Children’s Healthcare of Atlanta. Patients undergoing staged single ventricle palliation were excluded. The primary outcome of the study was to describe the epidemiology, longitudinal outcomes, and therapeutic interventions following surgical management of CHD in Trisomy 21 patients. Baseline characteristics and clinical outcomes were studied using descriptive statistics. Survival analysis was performed using the Kaplan-Meier survival analysis.

Results: Our study cohort comprised 261 patients, 56% being female and 13.8% being preterm. The median age was 4.9 months, and weight was 5.3 Kg at the time of surgery. The most common diagnoses were complete atrioventricular canal defect (47.1%) and ventricular septal defect (18.4%). Twenty-nine (11%) patients underwent staged biventricular repair. The median postoperative ICU length of stay was 3 days. All but 4 patients survived until hospital discharge with a survival rate of 98.5%. A majority, 93.5% of patients, were discharged on diuretic therapy, in comparison, only 30.3% were discharged on angiotensin-converting enzyme (ACE) inhibitors. By one year, 8.4% of patients remained on diuretics and 5.7% on ACE inhibitors. Pulmonary hypertension was evident in 25.7% of the patients at hospital discharge but improved in most of the patients over time. At the time of the last follow-up, only 6.7% of patients had pulmonary hypertension. Sixteen patients (6.1%) underwent permanent pacemaker placement for postoperative complete heart block. Overall, 10-year survival was 94.5% and intervention-free survival was 75%.

Conclusion: We describe longitudinal outcomes in a contemporary cohort of Trisomy 21 patients after congenital heart surgery with excellent short and long-term survival. Most of the patients do not require long-term medical therapy. Similarly, pulmonary hypertension tends to resolve. Periodic surveillance is required for prompt diagnosis of late complications and timely intervention. More research is needed to identify factors associated with the risk of re-intervention.