Raghib’s syndrome with the absence of the right superior vena cava

Abstract

A 40-year-old female diagnosed with a 42 mm atrial septal defect (ASD) (Figure 1a), drainage of the persistent left superior vena cava (PLSVC) into the left atrium (Figure 1b), and an absence of both the coronary sinus (Figure 2a) and right SVC (Figure 2b) was referred to our department. Cardiac catheterization showed the persistent drainage of the LSVC into the left atrium. During the catheterization, the mean pulmonary artery pressure (PAP) was 22 mmHg, and the volume of pulmonary flow (Qp) / the volume of systemic flow (Qs) was 1.5. The patient then underwent cardiac surgery. Arterial cannulation was performed from the ascending aorta and venous cannulation was carried out from the PLSVC and inferior vena cava (IVA). Under cardiopulmonary bypass (CPB), the ASD was closed with a pericardial patch, and the PLSVC was separated from the left atrium and anastomosed to the left pulmonary artery (LPA). The patient’s postoperative course was uneventful. Five years have passed since the surgery, and she has no upper extremity or facial edema. Furthermore, the most recent control computed tomography (CT) angiography showed patent anastomosis (Figure 2b). In addition, control echocardiography detected a systolic PAP of 22 mmHg and clearly indicated an intact interatrial septum with drainage from the PLSVC into the LPA.