Objective: To describe clinical outcomes of right atrial isomerism (RAI) and to clarify the impact of our aggressive strategy.
Methods: Retrospective chart review of RAI from 1978 to 2008. Patients older than 6-months or patients who had surgery before referral were excluded.
Results: There are 114 children enrolled (68 male). Median age at initial presentation was 9 days. Major cardiac abnormalities included atrioventricular septal defect in 109 (96%), double-outlet right ventricle in 101 (89%), pulmonary atresia in 57 (50%), and extracardiac total anomalous of pulmonary venous connection (TAPVC) in 52 (46%). None was suitable for bi-ventricle repair. Among 62 RAI in the early era (1978 to 1995), no surgical intervention was planned in 12, 10 of whom died. Fourteen (23%) completed Fontan, 11 of whom alive (79%). Survival was 59% at 1 year and 31% at 5 years. Multivariate analysis revealed TAPVC repair at initial surgery and atrioventricular valve regurgitation (AVVR) as significant risk factors for mortality (p<0.05). Since mid-90s, we have changed our strategy more aggressively, which included earlier referral to surgery, repair of AVVR at initial palliation, and modification of TAPVC repair. Among 52 RAI in the modern era (1996 to present), all but one were referred to surgery. Twenty-three (43%) completed Fontan, 21 of whom alive (91%). Survival was 70% at 1 year and 54% at 5 years, which were improved significantly from the early era (p<0.05). AVVR remains as a significant risk factor for mortality.
Conclusion: Aggressive medical and surgical management has improved survival of RAI