Raghib Syndrome, an Unsuspecting Culprit of Recurrent Strokes
Document Type
Article
Abstract
Background: Raghib syndrome is a rare congenital defect composed of a persistent left superior vena cava (PLSVC) with an unroofed coronary sinus (CS), allowing for systemic venous drainage directly into the left atrium (LA). Repair can be achieved with both percutaneous and surgical methods.
Case: A 66-year-old male with bicuspid aortic valve status post aortic valve replacement, hypertrophic cardiomyopathy with prior ventricular tachycardia status post ICD, atrial fibrillation on anticoagulation, and recurrent strokes on multiple anticoagulation changes [NOACs, Warfarin] presented with confusion and expressive aphasia. Work up for CVA revealed a left parieto-occipital ischemic stroke despite adequate anticoagulation and a positive bubble echocardiogram study (via the left arm) demonstrating filling of the LA prior to the right atrium. Subsequent cardiac CT revealed PLSVC draining into the LA with a long 6.8 cm unroofed CS.
Decision-making: Recurrent strokes had occurred despite anticoagulation. Continued patency of the PLSVC posed a risk for future stroke. To ameliorate stroke risk, the PLSVC draining into the LA was occluded with a 14 mm × 10 mm Amplatzer vascular plug II.
Conclusion: Abnormal communication between systemic venous and arterial circulation, as seen in Raghib syndrome, can increase the risk of thromboembolism including stroke. Percutaneous closure with a vascular plug is an effective treatment option.
First Page
3769
DOI
10.1016/S0735-1097(23)04213-4
Publication Date
3-7-2023
Recommended Citation
Kompella R, Naik R, Fairchild W, Heyden C, Mesia CI, Tiu JG, Upadhyay S, Dodeja A. RAGHIB SYNDROME, AN UNSUSPECTING CULPRIT OF RECURRENT STROKES. Journal of the American College of Cardiology. 2023 Mar 7;81(8_Supplement):3769-.