The scimitar is a rare congenital syndrome with female predominance (2:1) and unknown etiology (3). The manifestations vary from being asymptomatic at birth as seen in our patient to symptomatic patients presenting with signs of respiratory distress, pulmonary hypertension, and/or congestive heart failure due to shunting of the blood(2, 4). Based on the presentation it can be the infantile type or childhood/adult type (2). The presentations also vary with the type of associated cardiac anomalies such as VSD as seen in our case (5). Usually, patients present at an early age and with severe symptoms. Adult presentation is rare and usually occurs as an incidental finding due to secondary symptoms (6). The most common associated defect is ASD followed by PDA and VSD. The other associations are pulmonary vein stenosis, CoA, HLHS, arch abnormalities, and TOF (2). The case under discussion had ASD and VSD at presentation but again it was an incidental finding. There is evidence of other rare associations like congenital hydrocephalus and heart blockage(3). In this case, we discover a unique occurrence of left pulmonary vein dilation and tortuosity, which has never been seen before in any mentioned case reports. Non-cardiac associations could be a diaphragmatic hernia, vertebral anomalies, pulmonary sequestration, and genitourinary tract abnormalities (7).
Stabilizing the patient comes first, following surgical treatment of the aberrant circulation and associated multi-system abnormalities, which varies from case to case. Scimitar syndrome is usually detected via imaging. In 1/3rd of the cases, Scimitar/Turkish sword shape appearance due to the anomalous draining vein can be seen on chest radiograph (7). CT and cardiac MRI aid in delineating the vascular anomalies of the lungs. The diagnostic modality used in our cases was a CTA. Due to the high association of cardiac defects, an ECHO could be useful, especially during the follow-up visits. Some cases like ours but not all may require more invasive procedures like cardiac catheterization to assess the vasculature. Cardiac catheterization allows for coil occlusion of the anomalous systemic supply to the lung. Surgical treatment includes rerouting the anomalous veins to the left atrium and outcomes are with early diagnosis and a well-defined surgical plan, the outcomes are generally favorable, with a low risk of morbidity and mortality following corrective surgery, especially for the associated anomalies (2). To summarize, Scimitar is an uncommon congenital cardiac disease that has been well documented in the literature. However, because of the broad array of presentations and the association with various other congenital defects, a high index of suspicion is essential to make the diagnosis, which makes individual cases distinctive.