Coronary sinus atresia in a pediatric case: Review of literature

Coronary sinus (CS) is the venous drainage system of the heart. Absence of the CS or CS ostium atresia is rarely seen cardiac malformations. Congenital absence of CS usually is found together with other cardiac malformations.CaseA one day old newborn was referred to our hospital for cyanosis. His saturation was 84% patient was referred to cardiology unit. In echocardiographic examination hypoplastic left heart syndrome was revealed. Prostoglandin infusion was started, catheterization was planned for ductal stent implantation. Catheterization revealed the presence of persistent left superior vena cava (LSVC). When radiocontrast was given to LSVC, it drained to the CS. However CS did not drain to right atrium at normal anatomy. Coronary sinus drained to the base of right atrium, where right superior vena cava opened, via a tunnel shaped vein (shown by arrow and schematically in Fig. 1).DiscussionAbsence of coronary sinus is an extremely rare condition, and in patients with other congenital cardiac malformations. Such malformations can be managed surgically or percutaneously. But either method may disrupt coronary venous drainage therefore it should be paid great attention to the anatomy before doing these procedures. Also it is important to inform the cardiac surgeons before the operation of associated cardiac lesions.ConclusionCoronary sinus atresia is a rare condition that should be kept in mind especially in complex heart defects; diagnosis is critical before starting surgical procedure