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

Abstract

Objective: 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.

Methods: 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.

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.

Conclusions: 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.

Keywords: Atrial septal defect; Contrast echocardiography; Coronary sinus septal defect; Echocardiography; Raghib syndrome; Unroofed coronary sinus.