Visceroatrial situs solitus with atrioventricular alignment discordance double outlet right ventricle and superoinferior ventricles: fetal and neonatal echocardiographic findings.

Visceroatrial situs, ventricular morphology, and atrioventricular and ventriculoarterial alignments can reliably be assessed with fetal echocardiography. We herein describe, to our knowledge, the first reported fetal echocardiographic diagnosis of a fetus with visceroatrial situs solitus, atrioventricular alignment discordance, double outlet right ventricle, and superoinferior ventricles of a 22-year-old gravida III para II woman with a previous history of intrauterine fetal death. She had been evaluated at another center and was referred to our unit because of the suspicion about her diagnosis and underwent fetal ultrasonographic evaluation at 39 weeks gestation. Neonatal echocardiographic evaluation and autopsy findings confirmed the diagnosis. Accordingly there was (solitus, L-loop ventricle, and anterior aorta) double outlet right ventricle, superoinferior ventricles with diminutive right ventricle, noncommitted trabecular ventricular septal defect, and pulmonary hypertension.