Abstract 16964: National Experience of Ebstein Anomaly Births: Demographics, Treatment, and Mortality
Introduction: Neonatal Ebstein anomaly (EA) is a rare and variable congenital heart lesion with a paucity of large series data. The objectives were to describe the characteristics, concomitant diagnoses, and mortality of neonatal EA in a national cohort.
Methods: Kids’ Inpatient Database (KID) and National Inpatient Sample (NIS) were used to analyze birth hospitalizations of neonates with EA from 1998 to 2013. Univariate logistic regression identified variables associated with mortality.
Results: 1088 neonates diagnosed with EA were identified through NIS and KID; estimated incidence was 4.8 per 100,000 in-hospital live births. Associated lesions, other than atrial septal defect, included patent ductus arteriosus in 320 (29%), pulmonary atresia/stenosis in 170 (16%), ventricular septal defect in 96 (9%), transposition of great arteries in 31 (3%) and coarctation/aortic stenosis in 25 (2%). Approximately one third (322/1088) of patients were transferred to another hospital; mortality was analyzed in the remaining 766 patients. Overall mortality was 13% (103/766). Treatments were medical only in 88% (674/766), cardiac surgery alone in 7% (50/766), catheter based only in 2% (19/766), and hybrid catheterization and surgical in 3% (23/766). Mortality without interventions was 12% (78/674) and was 11% (2/19) with catheter based treatment only. Neonates with cardiac surgery had increased mortality: 28% (14/50, OR 2.97 vs no intervention, 95% CI 1.54-5.76, p=0.001) after cardiac surgery alone and 39% (9/23, OR 4.91, 95% CI 2.06-11.73, p<0.001) after hybrid. Cardiac lesions associated with increased mortality included pulmonary atresia/stenosis (OR 4.68, 95% CI 2.92-7.49, p<0.001) and coarctation/aortic stenosis (OR 3.33, 95% CI 1.12-9.95, p=0.03).
Conclusions: This large series of neonatal EA provides new insight to this rare disease. The majority of patients did not undergo surgery or catheterization during the birth hospitalization. Mortality risk was increased by concomitant right or left sided outflow obstructions and in those requiring surgical and/or catheter based procedures prior to neonatal discharge. To further investigate this rare lesion, multi-institutional collaboration is required to assess the clinical intricacies of EA.
- Congenital heart surgery, pediatric
- Tricuspid valve
- Pediatric cardiac intensive care
- Big Data
Author Disclosures: K. Holst: None. K. Hanson: None. E. Habermann: None. P. O’Leary: None. J. Dearani: None.
- © 2016 by American Heart Association, Inc.