LAUSR

Right aortic arch (RAA) with mirror image branching (RAMI) accompanied by absent pulmonary valve syndrome (APVS), tricuspid stenosis, and hypoplastic right ventricle is an extremely rare combination of congenital heart anomalies. This combination might result in severe cardiac failure and respiratory compromise, indicating a poor prognosis. Here, we describe a detailed prenatal echocardiographic diagnosis of RAMI accompanied by APVS and tricuspid stenosis. These anomalies were further confirmed by autopsy. This case could be helpful in improving our understanding of this abnormal combination and the development of an early therapeutic strategy.