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Uhl anomaly is an extremely rare condition, and herein, we present a case of prenatally diagnosed Uhl anomaly to illustrate the associated echocardiographic features. Fetal echocardiography at 26 weeks indicated an enlarged right ventricle, severe tricuspid and pulmonary regurgitation, and ruptured ventricular septal aneurysm, resulting in a circular shunt. At 36 weeks, based on the presence of a thin ventricular wall and absence of apical trabeculation, a diagnosis of Uhl anomaly was made. The neonate, delivered by cesarean section, underwent main pulmonary artery ligation to eliminate the circular shunt immediately after birth. Moreover, he underwent ductus arteriosus ligation and received a pulmonary artery to aorta shunt for progressive lung congestion. He is currently well and awaiting a staged Fontan operation involving a bidirectional Glenn procedure. Understanding and accurate prenatal diagnoses of Uhl anomaly may help in parental counseling, planning appropriate perinatal care, and increasing the chances of survival.

Prenatal ultrasonographic diagnosis of Uhl anomaly