Improving outcomes of the surgical management of right atrial isomerism

Abstract

Background: Patients with right atrial isomerism (RAI) have several cardiac malformations historically resulting in significant morbidity and mortality. We sought to assess whether current surgical strategies have improved the outcomes of patients with RAI.

Methods: A retrospective review of our database from 1997 to 2010 identified 60 consecutive patients with RAI who underwent initial palliation at Mt. Fuji Shizuoka Children's Hospital. All of the patients had a functional single ventricle. Of the 60 patients, 33 patients (51.7%) had obstructed total anomalous pulmonary venous drainage (TAPVD), 23 patients (40.3%) had significant (more than moderate) atrioventricular valvular regurgitation (AVVR), 7 patients (12.3%) had hiatus hernia, and 4 patients (6.7%) had major aortopulmonary collateral arteries. Pulmonary outflow atresia was present in 39 of the patients (65%), and 46 patients had systemic-to-pulmonary artery shunts. Of the 33 patients with TAPVD, 18 underwent repair of the condition at initial palliation, 9 others at the time of a Glenn operation, 2 more through a Fontan operation, and 4 at the interstage between palliative surgeries. Eight of the 23 patients with significant AVVR underwent atrioventricular valve repair at initial palliation, 8 others at the time of Glenn operation, 4 others at the time of Fontan operation, and 3 during the interstage between operations. An initial neonatal surgical procedure was performed in 30 patients (50%). The mean follow-up period for patients in the study was 53.1 months.

Results: Operative mortality after initial palliation was 15.4% (4 of 26 patients) before 2003 and 17.6% (6 of 34 patients) after 2004 (p=NS). Five-year survival was 53.8% before 2003 and 81.7% after 2004 (p=0.035, log-rank test). A multivariate analysis identified persistent AVVR of more than moderate degree (p=0.04) as a factor associated with late mortality.

Conclusions: The outcomes of surgery for RAI are improving. Neonatal palliative surgery for RAI carries a high operative risk of early mortality, and persistent significant AVVR remains a risk factor for late mortality.