Complex aortopulmonary window in a single ventricle setting: Technical considerations for staged palliation

Abstract

We report a successful surgical management of a case presented with a combination of aortopulmonary window (APW) with large ventricular septal defect (VSD) amounting to a single ventricle, with a view to highlight technical considerations during staged single-ventricle palliation.

Uncommon Association of Aortopulmonary Window in a Patient with Complex Univentricular Heart and CHARGE Syndrome.

“…There were two earlier case reports with APW in the setting of complex univentricular heart, one with tricuspid atresia and the other had double inlet ventricle with large ventricular septal defect. 3,4 Univentricular hearts are uncommon and there is controversy about the definition, classification, and nomenclature. Van Praagh et al 5 has described them as hearts with one ventricular chamber that receives both atrioventricular valves or a common AV valve, excluding mitral and tricuspid atresia.…”

“…There were two earlier case reports with APW in the setting of complex univentricular heart, one with tricuspid atresia and the other had double inlet ventricle with large ventricular septal defect. 3 , 4 …”

“…CHARGE syndrome initially was defined as a non-random association of anomalies (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness). 3 , 4 The cardiac anomalies are present in up to 75% of these patients. Septal defects and tetralogy of Fallot are most common cardiac defects in CHARGE syndrome.…”

“…Only a handful of case reports of APW associated with complex single ventricle lesions have been described in the literature. 3 , 4 To our knowledge, there is no literature on association of CHARGE syndrome (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness) with complex univentricular heart and APW. We report a case of unique combination of large APW in a complex univentricular heart of undetermined morphology with malposed great arteries in a neonate with absent thymus and CHARGE syndrome.…”