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

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.