Rare association of absent pulmonary valve syndrome, complete atrioventricular canal defect, double outlet right ventricle, right aortic arch, and aberrant right subclavian artery in a fetal case

Abstract

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly. It is often associated with TOF, but may also be associated with an intact ventricular septum or, more rarely, with tricuspid atresia. It's combination with a complete atrioventricular septal defects and double outlet right ventricle has been reported extremely rare, even in the postnatal period. Herein, we report a 20-week-old fetus with a right aortic arch and an aberrant left subclavian artery with this rare combination. We report a case of a 20-week fetus diagnosed having this rare combination of right aortic arch and aberrant left subclavian artery. The APVS with complete atrioventricular septal defects may represent another type of APVS.

Keywords: absent pulmonary valve syndrome; double outlet right ventricle; single ventricle.