Raghib syndrome: Echocardiographic features

A 25-year-old female was referred to our hospital with a 3 year history of palpitation and cyanosis. the chest X-ray was suggestive of pulmonary artery and right heart dilatation, and the eCg showed right ventricular hypertrophy. A transthoracic echocardiogram showed absent coronary sinus (Cs) on the parasternal long axis view (Fig1A). the roof of coronary sinus was absent and a secundum type atrial septal defect (AsD) was also noticed on the apical four chamber view with left-to-right shunt from left atrium (LA) to right atrium (RA) through the absent coronary sinus, on color Doppler (Figs1B1C). the persistent left superior vena cava (PLsVC) was demonstrated on the suprastenal view. A right heart contrast echocardiography showed the micro-bubbles first appearing in the LA appendage, confirming the PLsVC draining into LA (Fig1D). thus, the diagnosis was Raghib syndrome with secundum AsD, and the patient was referred for surgical repair.