Mitral Valve Replacement in Patient with Absent Right Superior Vena Cava, Pectus Excavatum, Marfan Syndrome and Severe Mitral Regurgitation

Combination of persistent left superior vena cava (PLSVC) draining into right atrium via the coronary sinus with a normal right superior vena cava (RSVC) is the most common systemic venous anomaly in visceroatrial situs solitus. On the other hand, a PLSVC in absence of RSVC is an extremely rare anomaly. Cardiovascular abnormalities are frequently encountered in patients with Marfan's syndrome but absence of RSVC with PLSVC is not reported in these patients. We report on a 52-year-old man with Marfan syndrome, an absence of right superior vena cava, severe pectus excavatum and severe mitral regurgitation who underwent successful mitral valve replacement. CT scan chest revealed marked cardiac deviation to the left associated with severe pectus excavatum, absent right superior vena cava and persistent left superior vena cava. During surgery replacement of mitral valve was performed through midsternotomy successfully.