Biventricular repair with the Yasui operation (Norwood/Rastelli) for systemic outflow tract obstruction with two adequate ventricles

Abstract

Background: The Yasui procedure is employed in neonates with interrupted aortic arch and left ventricular outflow tract obstruction (IAA/LVOTO) or aortic atresia-severe stenosis with ventricular septal defect (AA/VSD) and 2 adequate-sized ventricles. This combines a Norwood arch reconstruction with a Rastelli operation establishing a biventricular repair.

Methods: From 2002 to 2011, 21 neonates aged 3 to 55 days (mean 12.2 days, median 7 days) had IAA/LVOTO (n=13), AA/VSD (n=7), or AA/IAA with aortopulmonary window (n=1); ten (48%) had genetic abnormalities (8 with DiGeorge syndrome). Based on clinical characteristics and surgeon preference, 6 had a primary Yasui repair (4 AA/VSD, 2 IAA/LVOTO); 15 were staged with an initial Norwood repair (3 AA/VSD, 12 IAA) followed by Yasui completion in 13 (2 await completion) 4.3 to 26.6 months later (median 6.9 months).

Results: Early mortality was zero with no interstage deaths in the staged patients. One patient died 2 months after staged repair. Since biventricular repair, 8 survivors (44%) had reoperation for conduit replacement (n=6), recurrent LVOTO (n=1), or a residual VSD (n=1). No patient requires a pacemaker. There were 3 late deaths after biventricular repair, all in patients with genetic syndromes and IAA/LVOTO. Actuarial survival after initial operation was 100% at 1 year and 75% at 5 years. Actuarial freedom from reoperation or death after biventricular repair was 14% at 5 years.

Conclusions: The Yasui operation is effective for patients with IAA/LVOTO and AA/VSD. Primary and staged repair have comparable results. Reoperation after biventricular repair seems inevitable, mostly for conduit replacement. Genetic factors may affect long-term survival.