We present a patient with complex single ventricle physiology who was subsequently diagnosed with atresia of the coronary sinus ostium in the setting of myocardial dysfunction following operative palliation. Although a rare cardiac defect, awareness is important as the coronary venous system will often drain to a left superior vena cava (LSVC). If the LSVC is ligated without knowing of this defect, cardiac dysfunction and death can occur.
SalminenJTHakalaTPihkalaJMattilaIPuntilaJSairanenH. Coronary sinus orifice atresia with left superior vena cava in patients with univentricular heart. Ann Thorac Surg. 2006;81(5):e16–e17.
2.
FilippiniLHOvaertCNykanenDGFreedomRM. Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections. Heart. 1998;79(5):509–512.
3.
SantoscoyRWaltersHL IIIRossRDLyonsJMHakimiM. Coronary sinus ostial atresia with persistent left superior vena cava. Ann Thorac Surg. 1996;61(3):879–882.
4.
FultonJOMasCBrizardCPKarlTR. The surgical importance of coronary sinus orifice atresia. Ann Thorac Surg. 1998;66(6):2112–2114.
5.
TakabayashiSShimpoHYokoyamaK. Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia. Gen Thorac Cardiovasc Surg. 2007;55(5):197–199.
