Purpose or Case Report: Scimitar syndrome, also called congenital venolobar syndrome, is a rare phenomenon characterized by partial anomalous pulmonary venous return (PAPVR) with variable degrees of right lung and pulmonary artery hypoplasia. In this report, we present an extremely rare case demonstrating co-occurrence of Tetralogy of Fallot (TOF) and scimitar syndrome.

A two-day-old female was presented with dyspnea and acidosis. An echocardiography and subsequent ECG-gated CT angiogram of the chest revealed findings of TOF and complete pulmonary atresia, accompanied by arterial and ventricular septal defects (ASD, VSD) and a large patent ductus arteriosus (PDA) connecting to hypoplastic left pulmonary artery. Additionally, PAPVR was observed with drainage from the hypoplastic right lung to the IVC in the form of scimitar syndrome. Correctional surgery was undertaken, with creation of a right ventricular to pulmonary artery conduit, pulmonary arterio-plasty, ASD closure and creation of a fenestrated VSD patch. Follow-up imaging in two years showed development of multiple aortopulmonary collateral arteries (MAPCAs), persistent PAPVR and development of chronic right lung interstitial edema.

When there is suspicion for the association of TOF and scimitar syndrome, CT angiography, gated cardiac MRI, and 3-D contrast enhanced MR angiography are all preferred diagnostic modalities, providing excellent delineation of the abnormal pulmonary vein, its course and drainage, and quantification of the pulmonary venous flow. The anomalous vein most commonly drains into the IVC and less likely to the hepatic veins, right atrium, or portal vein.

While scimitar syndorme in conjunction with Tetralogy of Fallot is an extremely rare association, meticulous evaluation of the pulmonary venous connections is important in all patients with TOF, as the presence of PAPVR can result in right ventricular dilation and dysfunction.
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