Aortic Atresia or Complex Left Outflow Tract Obstruction in the Presence of a Ventricular Septal Defect

World J Pediatr Congenit Heart Surg. 2022 Sep;13(5):624-630. doi: 10.1177/21501351221114881.

Abstract

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.

Keywords: Ross-Konno; aortic atresia; biventricular repair; interrupted aortic arch; left ventricular outflow tract obstruction.

Publication types

  • Review

MeSH terms

  • Aortic Diseases* / complications
  • Heart Septal Defects, Ventricular* / complications
  • Heart Septal Defects, Ventricular* / surgery
  • Heart Valve Diseases* / surgery
  • Humans
  • Infant
  • Infant, Newborn
  • Retrospective Studies
  • Treatment Outcome
  • Ventricular Outflow Obstruction* / complications
  • Ventricular Outflow Obstruction* / surgery