Prenatal Diagnosis of Isolated Atrioventricular Discordance and Ventriculoarterial Concordance and Double-Outlet Right Ventricle in Situs Inversus: Case Report and Review of the Literature

Pediatr Cardiol. 2020 Dec;41(8):1807-1810. doi: 10.1007/s00246-020-02467-z. Epub 2020 Sep 24.


Atrioventricular (AV) discordance and ventriculoarterial (VA) concordance in the setting of visceral situs inversus are one of the rarest forms of cardiac malformations. To our knowledge, this is the first reported case of prenatal diagnosis of such rare cardiac anatomy in association with double-outlet right ventricle on fetal echocardiography. The physiology of this cardiac anomaly is similar to that of transposition of the great arteries, and the best surgical option is the atrial switch operation.

Keywords: Atrial inversion; Atrioventricular discordance; Fetal echocardiography; Ventricular inversion.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Arterial Switch Operation / methods
  • Double Outlet Right Ventricle / diagnosis*
  • Double Outlet Right Ventricle / surgery
  • Echocardiography
  • Female
  • Fetal Heart / diagnostic imaging
  • Heart Atria / abnormalities
  • Heart Ventricles / abnormalities
  • Humans
  • Infant, Newborn
  • Male
  • Pregnancy
  • Prenatal Diagnosis
  • Situs Inversus / diagnosis*
  • Situs Inversus / surgery
  • Transposition of Great Vessels / diagnosis
  • Transposition of Great Vessels / surgery
  • Treatment Outcome