Raghib Syndrome Physiology Revealed by Multimodality Cardiac Imaging

Raghib syndrome is a congenital anomaly that is caused by a persistent left superior vena cava draining into the left atrium, unroofed coronary sinus, and atrial septal defect. It can lead to cyanosis, cryptogenic strokes, pulmonary hypertension, and arrythmias.

A 60-year-old woman presented with palpitations due to atrial flutter. Cardiac imaging, including computed tomography, transesophageal echocardiography, and magnetic resonance imaging, confirmed bilateral superior vena cavae, with the left superior vena cava draining into the left atrium and minimal right-to-left shunting (3%). She was managed with anticoagulation and underwent regular follow-up without complications.

Diagnosis of Raghib syndrome relies on multimodality imaging. In cases with minimal symptoms and left-to-right shunting, conservative management with biannual follow-up may be sufficient.

Multimodality imaging is crucial in confirming anatomy and characterizing flow dynamics in cyanotic congenital heart disease. In patients with Raghib syndrome without cyanosis, conservative management with anticoagulation may be a safe alternative to surgical intervention.