Coronary sinus septal defect (unroofed coronary sinus): echocardiographic diagnosis and surgical treatment

To explore the value of transthoracic and right heart contrast echocardiography in the diagnosis of coronary sinus septal defect (CSSD), also known as unroofed coronary sinus.

The echocardiographic characteristics of 20 patients with CSSD who underwent surgery in our hospital between October 1999 and June 2012 were reviewed retrospectively, including results of 9 cases studied by contrast echocardiography, and compared with surgical results.

Of the coronary sinuses in these 20 patients, 40% were totally unroofed, 35% partially unroofed in mid-portion, 20% partially unroofed in terminal portion, and 5% mixed type (mid- and terminal portions). Of these 20 patients, 65% were associated with other types of atrial septal defects, and 65% had a persistent left superior vena cava. The diagnostic accuracy of echocardiography for the CSSD was 65%. In patients whose diagnoses were confirmed by echocardiography, the accuracy for the types of CSSD was 84.6%. All of the first 6 cases studied before 2002 were either undiagnosed or misdiagnosed, and 78.6% of the last 14 cases studied were diagnosed accurately. Of 9 patients who underwent contrast echocardiography, 8 were correctly diagnosed, including 5 type II and 3 Raghib syndromes. 1 case of type IIIa was misdiagnosed as Raghib syndrome.

The combination of transthoracic and contrast echocardiography can diagnose and classify CSSD accurately in most cases and should be the first choice for diagnosing CSSD.