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

Vistasp J Daruwalla,James Carr,Hassan Tahir,Jeremy D Collins,Keyur Parekh

doi:10.1155/2015/921247

Vistasp J Daruwalla, James Carr + Show 3 more

Open Access

https://doi.org/10.1155/2015/921247

Copy DOI

Abstract

Raghib Syndrome is a rare developmental complex, which consists of persistence of the left superior vena cava (PLSVC) along with coronary sinus ostial atresia and atrial septal defect. This Raghib complex anomaly has also been associated with other congenital malformations including ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis. Our case demonstrates an isolated PLSVC draining into the left atrium along with coronary sinus atresia in a young patient presenting with cryptogenic stroke without the atrial septal defect. Majority of the cases reported in the literature were found to have the lesion during the postmortem evaluation or were characterized at angiography and/or echocardiography. We stress the importance of modern day imaging like the computed tomography (CT) angiography and cardiac MRI in diagnosis and surgical management of such rare lesions leading to cryptogenic strokes.

Highlights

Persistence of the left superior vena cava (PLSVC) is seen in 0.3%–0.5% of normal subjects [1, 2] and in up to 2.1%– 4.3% of patients with congenital heart disease [3]
Coronary sinus ostial atresia with a persistence of the left superior vena cava (PLSVC) is usually associated with an anomalous connection between the coronary sinus ostium and the left atrium [4, 5]
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

Summary

Introduction

Persistence of the left superior vena cava (PLSVC) is seen in 0.3%–0.5% of normal subjects [1, 2] and in up to 2.1%– 4.3% of patients with congenital heart disease [3]. Coronary sinus ostial atresia with a PLSVC is usually associated with an anomalous connection between the coronary sinus ostium and the left atrium [4, 5]. 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. Cases of an intact atrial septum and with PLSVC into left atrium were noted [8]. Most of the cases reported in the literature were detected during postmortem evaluation or were characterized at angiography and/or echocardiography. We report a case of isolated PLSVC draining into the left atrium along with coronary sinus atresia in a young patient presenting with cryptogenic stroke evaluated with transthoracic echocardiography, computed tomography (CT), angiography and Cardiac MRI

Case Presentation

Discussion

Findings

Conclusion