Beyond Scimitar Syndrome: A Unique Combination of Complex Congenital Heart Disease (original) (raw)

2019, Journal of the American College of Cardiology

Background: Scimitar Syndrome is a rare entity within congenital heart disease. The clinical presentation may be insidious and associated with a number of cardiopulmonary anomalies. Case: A 47 year old male presented with progressive exertional dyspnea, palpitations, and a new systolic murmur in the setting of known dextrocardia. He underwent TTE which showed mildly reduced biventricular function, dilated RV, and moderate TR. Decision-making: Given the suspicion for underlying congenital disease, he underwent cardiac MRI, X-ray, and CTA (Figure 1) which confirmed dextrocardia with evidence of Scimitar syndrome with all right sided pulmonary veins draining into the IVC, a Qp/Qs of 1.6:1, and concomitant dilation of the RA, RV, and PA. Additional findings included an anomalous single coronary artery originating from the right coronary cusp, aortic root dilation (5 cm) and moderate TR. Surgical repair was performed with in-situ pericardial repair of scimitar vein routing to the LA, repair of the TV, and repair of the noncoronary aortic sinus with CardioCel. On follow up, the patient reported resolution of dyspnea on exertion and improved functional capacity. Conclusion: This unique case highlights dextrocardia with Scimitar Syndrome, aortopathy, and anomalous single coronary artery. This congenital complex went undiagnosed for nearly five decades with only mild symptom burden. Multimodality cardiac imaging played a crucial role in the diagnosis and surgical planning of this case.