To analyze clinical characters, associated anomalies, diagnostic approach, and surgical procedures in patients with unroofed coronary sinus syndrome.

The clinical, echocardiographic, operative and follow-up data of 109 patients with unroofed coronary sinus syndrome from May 1999 to July 2016 were reviewed retrospectively(type I 51 cases, type Ⅱ 17 cases, type Ⅲ 17 case and type Ⅳ 24 cases). The initial diagnosis of unroofed coronary sinus syndrome was made by echocardiography in 56 cases, while the remains were confirmed by the surgeon at repair of other congenital cardiac anomalies. 69 of the 109 cases associated with left superior vena cava(LSVC), and 53 of the 69 directed drained into the left atrium(LA). Among the 109 cases, 2 cases associated with complex anomalies underwent palliative operations, and other cardiac malformations were corrected simultaneously by surgical correction in the other cases. LSVC was ligated in 8 cases, and the intracardiac tunnel to drain LSVC to right atrium(RA) was reconstructed in 38 cases, intracardiac baffle in 9 cases, extracardiac repair in 2 cases. The associated cardiac lesions were corrected concomitantly.

There were 5 hospital deaths. 3 patients died of low cardiac output syndrome and 2 patients died of pulmonary infection. We followed up 87 early survivors, and there was no death and severe complications.

Unroofed coronary sinus syndrome is often missed in the preoperative evaluation of congenitally malformed hearts. Preoperative TTE is still the most important method in the diagnosis of UCSS. Strengthening the understanding of the pathological anatomy and physiological characteristics of the heart malformation may help us to improve the preoperative diagnosis of UCSS. When associated with LSVC, UCSS should be considered as a possible additional finding. We performed different surgical approaches to deal with the different kinds of LSVC with a good result.

Unroofed coronary sinus syndrome; Surgical procedures; Left superior vena cava; Ultrasound cardiogram