Raghib’s syndrome: A report of two cases

Abstract

We treated two patients with a rare developmental complex. The persistent left superior vena cava draining into the left atrium (PLSVC into LA) was associated with an absent coronary sinus and an atrial septal defect. Ligation of PLSVC and patch-repair of the atrial septal defect were successfully performed in one stage. The atrial septal defect was located in the upper and posterior aspect of the interatrial septum and appeared to be an unique type of atrial septal defect. In the other patient, additional multiple cardiac defects were associated with this syndrome, including ventricular septal defect, pulmonary stenosis, tricuspid insufficiency, and complete transposition of the great arteries. Palliative Blalock procedure was used for this patient. The PLSVC into LA was discovered accidentally in both cases during heart catheterization and it was clearly demonstrated by venography. For a preoperative recognition of PLSVC, computed tomograms of the heart are of great assistance. Surgical correction of the persistent superior vena cava was emphasized for treatment of this syndrome.

Raghib Syndrome Presenting as a Cryptogenic Stroke: Role of Cardiac MRI in Accurate Diagnosis

“…Drainage of the PLSVC into the left atrium due to congenital defects which prevent the rotation of the sinoatrial region; this in turn causes the left and the right cardinal veins to lie at the same level rather than the usual inferior location of the right cardinal vein, thus blocking the development of the coronary sinus. As a result a PLSVC draining into left atrium is commonly associated with coronary sinus ostial atresia [ 7 ]. LSVC may serve as a collateral channel secondary to coronary sinus atresia.…”

“…Raghib syndrome is a rare developmental complex, which consists of PLSVC draining in the left atrium, coronary sinus ostial atresia, and atrial septal defect in the posteroinferior angle of the atrial septum. Okumori et al stated that the atrial septal defect was true and this specific type of atrial septal defect associated with an absent coronary sinus [ 7 ]. Cases of an intact atrial septum and with PLSVC into left atrium were noted [ 8 ].…”

Raghib Syndrome and Pulmonary Arterial Hypertension in a Pediatric Patient: Case Report and Literature Review

“…Therefore, pre-operatory recognition of the LSVC and its aberrant drainage ensure appropriate surgical management of Raghib syndrome cases, which is mandatory for therapeutic success. Okumori et al further described two cases of middle-aged women presenting with heart failure symptoms, in whom cardiac catheterization revealed the persistence of the LSVC, its abnormal drainage and the ASD [ 11 ]. In one of the cases, a complete surgical correction was accomplished, whereas the other patient had other associated cardiac anomalies and underwent a palliative Blalock procedure [ 11 ].…”

“…Okumori et al further described two cases of middle-aged women presenting with heart failure symptoms, in whom cardiac catheterization revealed the persistence of the LSVC, its abnormal drainage and the ASD [ 11 ]. In one of the cases, a complete surgical correction was accomplished, whereas the other patient had other associated cardiac anomalies and underwent a palliative Blalock procedure [ 11 ]. Although surgery remains the definitive treatment for Raghib syndrome, a unique case of a 58-year-old female who underwent complete correction through percutaneous repair has also been described in the literature [ 12 ].…”

Coronary venous anatomy and anomalies

“…Veins that would normally empty into the CS may drain into the superior vena cava or left atrium. 27,28…”