Perioperative Management of Scimitar Syndrome Mimicking Total Anomalous Pulmonary Venous Return Posing a Diagnostic Dilemma: A Compilation of Two Cases

The low prevalence of scimitar syndrome along with its varied clinical presentation poses a diagnostic dilemma to the treating clinicians. It usually falls under a large spectrum of conditions called venolobar syndrome. Scimitar involves the partial venous drainage of the right lung to the inferior vena cava (IVC). We share our experience of two cases that were diagnosed as partial anomalous pulmonary venous connection/drainage (PAPVC/PAPVD) on echocardiography but CT scan revealed the underlying scimitar syndrome. Perioperative pulmonary arterial hypertension, intraoperative ventilation strategies for managing associated lung hypoplasia, and postoperative right ventricular dysfunction are a few challenges faced in the perioperative period.