Tricuspid atresia associated with truncus arteriosus versus aortopulmonary window: Combining fetal and postnatal echocardiography to make the diagnosis

The coincident occurrence of tricuspid atresia and aortopulmonary window (APW) is exceedingly rare, with one previous case reported in the literature. We present a patient with tricuspid atresia, pulmonary atresia, and APW. Postnatal echocardiograms demonstrated no visible pulmonary valve, and additional defects including a bicuspid aortic valve, right aortic arch and anomalous coronary arteries raised suspicion for tricuspid atresia with persistent truncus arteriosus. However, fetal echocardiography and direct visualization of the anatomy confirmed the alternate diagnosis. The patient underwent successful palliation consisting of APW repair, atrial septectomy and a 3.5 mm modified Blalock-Taussig shunt, followed by a bidirectional cavopulmonary connection. Mini-Abstract The coincident occurrence of tricuspid atresia and aortopulmonary window (APW) is exceedingly rare, with one previous case reported in the literature. We present a patient with tricuspid atresia and pulmonary atresia with pulmonary blood flow supplied by an APW. Additional defects including a bicuspid aortic valve, right aortic arch, and anomalous coronary arteries raised suspicion for tricuspid atresia with persistent truncus arteriosus. However, combining fetal echocardiography and transthoracic echocardiography confirmed the diagnosis of tricuspid atresia with pulmonary atresia and APW.