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

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Abstract

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.

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The authors declare that they have no funding support for this case report.

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Authors and Affiliations

  1. Department of Pediatrics, Section of Pediatric Cardiology, Rush University Medical Center, Chicago, IL, USA

    Saif Aljemmali, John Bokowski & Karim Diab

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  1. Saif Aljemmali
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  2. John Bokowski
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  3. Karim Diab
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Correspondence to Saif Aljemmali.

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Supplementary file1 (AVI 47703 kb)

Vid. 1 Fetal Echocardiography showing the four-chamber view of the heart in which the right side of the fetus is located on the right lower portion of the screen. Notice the characteristic features of the right-sided morphological left atrium, such as the flap of PFO, cross-section view of the dilated coronary sinus, and the two pulmonary veins draining into this atrium. This atrium is connected into right-sided morphological right ventricle with coarse trabeculation and an apically displaced atrioventricular valve (the tricuspid valve). Notice the smooth trabeculation of the left-sided morphological left ventricle.

Supplementary file2 (AVI 41403 kb)

Vid 2 Fetal Echocardiography showing the five-chamber view of the heart in which the left side of the fetus is located on the left side of the screen. Notice the characteristic features of the right-sided morphological left atrium, such as the flap of PFO, cross-section view of the dilated coronary sinus and one pulmonary vein draining into this atrium. Notice the fibrous discontinuity between mitral and aortic valves in addition to the VSD.

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Aljemmali, S., Bokowski, J. & Diab, K. 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 41, 1807–1810 (2020). https://doi.org/10.1007/s00246-020-02467-z

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  • DOI: https://doi.org/10.1007/s00246-020-02467-z

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