Prenatal Diagnosis of Isolated Atrioventricular Discordance and Ventriculoarterial Concordance and Double-Outlet Right V
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CASE REPORT
Prenatal Diagnosis of Isolated Atrioventricular Discordance and Ventriculoarterial Concordance and Double‑Outlet Right Ventricle in Situs Inversus: Case Report and Review of the Literature Saif Aljemmali1 · John Bokowski1 · Karim Diab1 Received: 12 July 2020 / Accepted: 16 September 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
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. Keywords Atrioventricular discordance · Atrial inversion · Ventricular inversion · Fetal echocardiography
Case Report A 27-year-old Hispanic multipara female was referred to us at 35 weeks of gestation for fetal echocardiography in view of suspected congenital heart disease due to the presence of visceral situs inversus with levocardia (isolated levocardia) on routine sonography. Fetal echocardiography demonstrated visceral situs inversus and isolated levocardia. The left-sided atrium had features of a morphological right atrium as it received the inferior vena cava, right superior vena cava, and a dilated coronary sinus with a persistent left superior vena cava. All pulmonary veins entered the right-sided atrium, which was the left morphological atrium as it had a thin valve of the fossa ovalis. There was an atrial communication with right to left shunt. The left-sided morphological right atrium was connected to a left-sided morphological left ventricle, which had a smooth wall. The right-sided morphological left atrium was connected to the right-sided morphological right ventricle that had trabeculations, moderator band, and associated with apical displacement of the Electronic supplementary material The online version of this article (https://doi.org/10.1007/s00246-020-02467-z) contains supplementary material, which is available to authorized users. * Saif Aljemmali [email protected] 1
Department of Pediatrics, Section of Pediatric Cardiology, Rush University Medical Center, Chicago, IL, USA
right-sided atrioventricular valve, i.e., tricuspid valve (Fig. 1, 2; Vid.1). There was ventriculoarterial concordance with the aorta arising from the left ventricle and the pulmonary artery arising from the right ventricle. In addition, the diagnosis of double-outlet right ventricle (DORV) was made due to the presence of fibrous discontinuity of mitral and aortic valve, over-riding aorta, and ventricular septal defect (VSD) with normally related great arteries (Vid. 2). The patient was born at 39 weeks of gestation via induced vaginal delivery. He was hemodynamically stable with expected hypoxemia (oxygen saturation ~ 75%). Physical e
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