A 4-month-old male with Raghib syndrome: a rare case report from Syria

• Published in: Annals of medicine and surgery Vol. 86; no. 3; pp. 1687 - 1690
• Main Authors: Sleiay, Mouhammed, Sleiay, Bilal, Batrash, Obeda, Alabdullah, Hadi, Alsmoudi, Hasan, AlBaroudi, Douha, Almohamed, Ahmad, Ali Abshi, Mohammad, Takkem, Saleh
• Format: Journal Article
• Language: English
• Published: England 01-03-2024
• Subjects: persistence of the left superior vena cava; unroofed coronary sinus syndrome; Raghib Syndrome; tetralogy of Fallot
• ISSN: 2049-0801; 2049-0801
• DOI: 10.1097/MS9.0000000000001693

Raghib syndrome is a rare congenital complication consisting of the termination of the left superior vena cava (LSVC) in the left atrium, an unroofed coronary sinus, and an atrial septal defect most often found in the posterior-inferior angle of the atrial septum. Both a right-to-left and a left-to-right intracardiac shunt exist. In most circumstances, they do not show any symptoms.
The patient presented with a persistent left superior vena cava draining into the left atrium, an unroofed coronary sinus, and a secondary atrial septal defect (ASD). Transthoracic echocardiography was used to diagnose the condition, and surgery was applied as the primary treatment.
It was formerly believed that this complex was exclusive to Raghib syndrome; however, cases have been found in which the interatrial connection is the aperture of the unroofed coronary sinus in patients with a normal atrial septum.
Extracardiac treatment for this illness reduces the load on the left atrial suture and may stop further arrhythmias. There is no possibility of pulmonary vein flow restriction when there are no atrial tunnels.