Fetal Heterotaxy with Tricuspid Atresia, Pulmonary Atresia, and Isomerism of the Right Atrial Appendages at 22 Weeks

A 31-year-old gravida 1was seen for routine evaluation of fetal anatomy at 22 weeks. A complex cardiac abnormality was diagnosed. Findings at the time of the detailed exam included levocardia with a normal left-sided stomach bubble, but a midline-appearing liver. An absent right atrioventricular val-var connection typical of tricuspid atresiawas identifiedwith a patent and normal-sized left atrioventricular (mitral) valve (►Fig. 1A). A large unrestricted ventricular septal defect was noted, communicating to a relatively hypoplastic right ventri-cle. The atrial septum demonstrated normal bowing of the atrial septum from the right to the left atriumwith evidence of flow on color flow Doppler, with a moderate-sized ostium primum atrial septal defect. Atrial morphology was sugges-tive of bilateral right atrial appendages but inconclusive (►Fig. 1B). There was D-transposition with the aorta arisin