Severe left outflow tract obstruction (LVOTO) is not always associated with hypoplastic left heart syndrome (HLHS). Aortic valvar atresia or complex LVOTO in the presence of a large ventricular septal defect (VSD) are a rare group of lesions that offer the possibility of biventricular repair. The Yasui procedure is the commonest surgical approach which can be performed as a primary neonatal correction or as a staged procedure with a Norwood followed by a subsequent Rastelli. This article reviews the surgical outcomes and decision-making process. Both strategies are reasonable with the trend toward staged procedure in the setting of the additional interrupted arch, with neonatal survival of > 90% in the modern era and excellent long-term survival. Re-intervention is mostly related to conduit revision and the need for re-operation for LVOTO is rare. Deciding between conventional repair and the Yasui in cases of LVOTO/VSD can be difficult and there are no uniform accepted criteria. In a typical neonate, an aortic annulus < 4.5 mm is generally the limit of acceptability for a conventional repair. In selected cases of LVOTO/VSD, an alternative to the Yasui is the Ross-Konno. Retrospective comparisons between the 2 techniques are difficult due to differing patient characteristics (especially associated with mitral valve disease) but the neonatal Ross has been associated with higher early mortality.

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Introduction: The hybrid procedure (bilateral PA bands [PAB] ± ductal stent) is a contemporary management option for infants with critical left heart obstruction. We sought to determine patient/procedural characteristics, subsequent outcomes and associated factors after PAB in a multi-institutional study. Methods: From 2005-2019, 214 of 1236 infants from 24 Congenital Heart Surgeons’ Society participating institutions underwent PAB. Median follow-up was 7 years. Parametric hazard modeling with competing risk methodology was performed to determine risk and associated factors for end-states of biventricular repair (BVR), Fontan, transplantation (Tx) or death without an end-state. Results: PAB was performed at a median age of 7 days & median weight of 3.1 kg. Intention of PAB varied - institutional preference (21%), non-cardiac comorbidities (20%), BVR consideration (12%), etc. Ductal stent was placed in 69% of patients (74% same day as PAB) and atrial septal interventions occurred in 61% (24% same day as PAB). Preoperative comorbidities (e.g. prematurity, genetic syndromes, neurological diagnoses, etc.) were present in 70%. At 5 years, 9% had reached BVR, 36% Fontan and 12% Tx (21/26 Tx immediately after PAB), with 35% having died and 8% alive without an end-state (Figure). Significant factors associated with BVR were presence of VSD and ductal stent; with Tx: earlier era, underlying aortic atresia, older age and greater weight at PAB, and absence of ductal stent; with death: low birth weight, ≥ moderate tricuspid valve regurgitation before PAB, & older age at PAB. Conclusions: Heterogeneity is prevalent in patient/procedural characteristics and subsequent procedures and outcomes after PAB, possibly influenced by institutional preference and different intended management pathways. There is important mortality and <50% of patients achieve a Fontan or BVR. Preoperative factors influence the use of this approach and thus are an important influence on outcomes.

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