Scimitar Syndrome: How to suspect and diagnose on fetal echo

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On fetal echocardiography, scimitar syndrome is suspected when there is dextroposition of an otherwise structurally normal heart due to right lung hypoplasia, with a small right pulmonary artery and reduced pulmonary venous return from the right lung. The hallmark lesion is partial anomalous pulmonary venous connection, where one or more right pulmonary veins drain inferiorly toward the inferior vena cava rather than into the left atrium; in the fetus this is often inferred rather than fully demonstrated, using color Doppler to follow venous flow coursing caudally along the right atrial border. Associated findings can include an atrial septal defect, mild right heart volume loading, and occasionally an abnormal systemic arterial supply to the right lung from the aorta, suggesting a component of pulmonary sequestration. Prenatal diagnosis can be challenging because of small vessel size and suboptimal fetal position, so the echo report should clearly describe the suspicion, document right lung hypoplasia and cardiac position, and recommend detailed postnatal echocardiography
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Cardiology
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