An extreme case of Raghib syndrome: combination of mirror-image dextrocardia, single atrium, double-outlet right ventricle, and patent ductus arteriosus

Extract

A 22-year-old female with untreated cyanotic congenital heart disease was admitted following syncope. Chest X-ray revealed mirror-image dextrocardia, with cardiac apex and gastric bubble on the right, along with increased pulmonary blood flow (Panel A). Enhanced computed tomography confirmed mirror-image dextrocardia with cardiomegaly, single atrium (SA), and right ventricular hypertrophy (Panel A). Additionally, the coronary sinus was absent, with bilateral superior vena cava (SVC) draining into SA (Panel B). Hemiazygos vein joined the left-SVC (L-SVC) (Panel B). The inferior vena cava was absent, and two hepatic veins directly drained into the SA. Additionally, a 1.8 cm ventricular septal defect (VSD), a 6 mm patent ductus arteriosus (PDA), double-outlet right ventricle (DORV), and right ventricular outflow tract stenosis (black asterisk) were identified (Panel C).

Raghib syndrome, or completely unroofed coronary sinus syndrome, manifests as the termination of the persistent L-SVC (right side in mirror-image dextrocardia) into left atrium and atrial septal defect (ASD). SA represents an extreme condition of ASD. Although Raghib syndrome is well documented with VSD, PDA, and DORV, the combination of mirror-image dextrocardia and SA remains unreported. In this case, Raghib syndrome is accompanied by multiple cardiovascular malformations, leading to increased pulmonary blood flow, mixing of oxygenated and deoxygenated blood, and hypoxia symptoms. Surgical intervention is necessary for survival, but dextrocardia complicates the procedure. Computed tomography imaging identified cardiovascular anomalies for surgical planning, enabling the successful operation.