Surgical correction of the unroofed coronary sinus syndrome

Partially or totally unroofed coronary sinus is usually associated with other anomalies of the heart. In the period of 5 years between January 1987 to October 1992, 13 patients with this syndrome were encountered. All of them had a left superior vena cava draining into left atrium. Of these, 12 were totally unroofed and one had a partial unroofing of the coronary sinus. Associated anomalies encountered included common atria, cortriatriatum, complete A-V canal, tetralogy of Fallot, total and hemianomalous pulmonary venous connection, double outlet right ventricle and atrial isomerism with dextrocardia. Four patients died in the post operative period due to low cardiac output. The nine survivors are all in NYHA class I. At a mean follow up of 4 years, all except one were in sinus rhythm. The other had a nodal rhythm persisting for 4 years. Pericardial baffle redirection of LSVC into the systemic venous atrium which is advocated, was performed in all but one of our earliest patients. Unroofed coronary sinus syndrome is a correctable lesion, the results of which are influenced more by the complexity of the associated anomalies rather than the syndrome per se.