Abstract 19273: Short and Long Term Outcomes in Children With Trisomy 21 Following Interventions for Congenital Heart Disease (CHD): A Study From the Pediatric Cardiac Care Consortium (PCCC).
Methods: Retrospective review of the PCCC for 7809 patients (4081 females) with Trisomy 21 who underwent transcatheter or surgical intervention for CHD from 1982 to 2007. Short term outcomes included in-hospital mortality, need for extracorporeal life support (ECLS), and need for permanent pacemaker. Outcomes of 30 day mortality, long term survival, and transplant status were obtained for patients with available direct identifiers up to April 2003 by linkage to the National Death Index (NDI) and the United Network for Organ Sharing.
Results: The most common surgical procedures were endocardial cushion defect repair (3197/8511, 37.6%), ventricular septal defect repair (1728/8511, 20.3%), and Tetralogy of Fallot repair (589/8511, 6.9%). In-hospital mortality was 5% (334/6694) for corrective procedures, 28.7% (72/251) for palliative procedures, and 34% (38/112) single ventricle procedures. Forty of 7057 patients required ECLS and 140 (2%) initial pacemaker implantation. Six patients underwent transplant; 5 cardiac, 1 lung. Transcatheter interventions were performed in 593 patients; the most common interventions were patent ductus arteriosus occlusion (395/833, 47.4%), pulmonary arterioplasty/stent (137/833, 16.4%), and atrial septal defect closure (133/833, 15.9%). Catheterization mortality was 27/3979 (0.7%). Late deaths occurred in 468 patients out of 4294 discharged alive and submitted to NDI (including 15 with 30 day mortality). Survival by treatment pathway is shown in the Figure. The most common causes of late mortality were cardiac (199/453, 43.9%), infection (71/453, 15.7%), and pulmonary hypertension (46/453, 10.2%).
Conclusions: Long term survival conditioned after CHD interventions in patients with Trisomy 21 approaches 92% (95% CI 91.3-92.9%) for corrective procedures, 64% (95% CI 55.9-72.9%) for palliative procedures, and 66% (95% CI 50.5-81.5%) for single ventricle procedures. Causes of late deaths are predominately cardiac.
Author Disclosures: J.K. Peterson: None. K.G. Catton: None. L. Kochilas: None. S.P. Setty: None.
- © 2016 by American Heart Association, Inc.