Unroofed coronary sinus syndrome: A case report / 中南大学学报(医学版)

Unroofed coronary sinus syndrome (UCSS), also named coronary sinus septal defect, is a rare type of atrial septal defect with the incidence less than 1% of the total number of atrial septal defects. It is caused by incomplete formation of left atrial venous folds during embryonic development. Here we reported a patient with UCSS, who was treated in the Second Xiangya Hospital of Central South University. The patient was 50 years old and the main clinical manifestations were fatigue and shortness of breath after repeated exercise. Color Doppler echocardiography showed coronary sinus dilatation (17 mm×14 mm), indicating the possibility of permanent left superior vena cava. Pulmonary angiography showed that the left ventricle and coronary sinus were developed at the same time while the atrial septum was intact after the development of the left atrium, followed by the right atrium and right ventricle, indicating a partial anomalous pulmonary venous drainage (intracardiac type). Finally, the cardiac computed tomograhic angiography showed that 4 pulmonary veins and permanent left superior vena cava (PLSVC) went into the left atrium and the coronary sinus, respectively, while the coronary sinus septum was absent and the PLSVC was connected with the left atrium. The patient was later treated with the correction of non-parietal sinus syndrome in the Cardiovascular Surgery Department of our hospital.

Surgical treatment of pediatric unroofed coronary sinus syndrome / 中华实用儿科临床杂志

Objective@#To analyze the clinical features and surgical treatment of unroofed coronary sinus syndrome(UCSS) in children, and to provide evidence for the diagnosis and treatment of such diseases during the perio-perative period.@*Methods@#A retrospective analysis was conducted based on the clinical data of 13 patients with UCSS, who underwent surgical treatment at Beijing Children′s Hospital Affiliated to Capital Medical University from January 2011 to December 2017.All 13 patients were complicated with persistent left superior vena cava(PLSVC) and other cardiac malformations; 7 patients were diagnosed preoperatively and 6 patients were diagnosed intraoperatively.Eleven cases were diagnosed type Ⅰ and 2 cases were type Ⅳ according to Kirklin′s classification.In all of 13 cases, atrial septal reconstruction was performed to drain the left superior vena cava(LSVC) to the right atrium, and other cardiac malformations were corrected at the same time.@*Results@#One patient died in this group, and the other 12 patients had early recovery after operation.Postoperative-ventilator-assisted time was 5-246 h(median 29 h) and hospital stay time was 8-40 d(median 16 d). The remaining 12 cases were followed up from 6 months to 7 years and 2 months.No death or complications occurred.No residual shunt or residual obstruction was found.@*Conclusions@#In the case of congenital heart disease with PLSVC, UCSS should be alerted.Atrial septal reconstruction and drainage of LSVC into right atrium in children with UCSS can achieve satisfactory surgical results.

Surgical treatment of pediatric unroofed coronary sinus syndrome / 中华实用儿科临床杂志

Objective To analyze the clinical features and surgical treatment of unroofed coronary sinus syn-drome(UCSS)in children,and to provide evidence for the diagnosis and treatment of such diseases during the perio-perative period. Methods A retrospective analysis was conducted based on the clinical data of 13 patients with UCSS, who underwent surgical treatment at Beijing Children＇s Hospital Affiliated to Capital Medical University from January 2011 to December 2017. All 13 patients were complicated with persistent left superior vena cava( PLSVC)and other cardiac malformations;7 patients were diagnosed preoperatively and 6 patients were diagnosed intraoperatively. Eleven cases were diagnosed typeⅠand 2 cases were typeⅣaccording to Kirklin＇s classification. In all of 13 cases,atrial sep-tal reconstruction was performed to drain the left superior vena cava(LSVC)to the right atrium,and other cardiac mal-formations were corrected at the same time. Results One patient died in this group,and the other 12 patients had early recovery after operation. Postoperative-ventilator-assisted time was 5-246 h( median 29 h)and hospital stay time was 8-40 d(median 16 d). The remaining 12 cases were followed up from 6 months to 7 years and 2 months. No death or complications occurred. No residual shunt or residual obstruction was found. Conclusions In the case of congenital heart disease with PLSVC,UCSS should be alerted. Atrial septal reconstruction and drainage of LSVC into right atrium in children with UCSS can achieve satisfactory surgical results.

Clinical characters and surgical treatment of unroofed coronary sinus syndrome / 中华胸心血管外科杂志

Objective To analyze clinical characters,associated anomalies,diagnostic approach,and surgical procedures in patients with unroofed coronary sinus syndrome.Methods 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 Ⅰ51cases,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.Results 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.Conclusion 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.

Diagnosis and surgical treatment of unroofed coronary sinus syndrome / 第二军医大学学报

Objective To summarize the clinical manifestations and surgical treatment of unroofed coronary sinus syndrome(UCSS). Methods Nine patients underwent surgical repair for UCSS from Jan. 1999 to Jun. 2009 in our hospital, and the patients included 3 with type I a, 1 with type I b, 1 with type II a, 3 with type III a, and 1 with type III b UCSS. The diagnosis of UCSS was made by echocardiography in 6 patients and by the surgical exploration in 3 patients. Persistent left superior vena cava (PLSVC) was found in 7 cases; 3 cases with PLSVC directly draining into the left atrium ( I a) were reconstructed by the intra-atrial routes to guide PLSVC to the right atrium, and 4 cases with PLSVC draining into the coronary sinus and coronary sinus orifice opening to the left atrium and right atrium (1 case, II a) and left atrium (3 cases, III a) were dealt with the abnormal coronary sinus orifice. Other associated cardiac lesions were corrected concomitantly. Results Two cases had early complications and the other 7 cases had smooth recovery after operation; all the patients were cured and discharged. Eight cases were followed up for 2 months to 5 years and there was no death or late complications. Conclusion UCSS is a rare congenital cardiac anomaly and is difficult to diagnose preoperatively; so careful exploration should be done during operation. Different surgical approaches should be chosen according to the types of communication between PLSVC and cardiac atrium, the positions of abnormal sinus ostium, and other cardiac lesions. Satisfactory prognosis can be obtained if physiological correction is achieved.

Reestablishment the roof of coronary sinus in treating unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava / 中华胸心血管外科杂志

Objective To summarize the surgical procedures of unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava.Methods From 2000 to 2008,753 cases of endocardial cushion defect underwent surgical repair.Among them 15 cages(2.0%)were associated with persistent left superior vena cava and unroofed coronary sinus syndrome.The drainage of the persistent left superior vena cava and coronary vein were corrected simultaneously during the operation by reestablishing the roof of the coronary sinus.The persistent left superior vena cava were drained to right atrium by intra-atrial tunnel with pericardial patch or left atrium folding in 14 cases and it was ligated in the remain one.Most of the coronary veins were drained into right atrium after the correction of cardiac defect.Results One patient died of severe pulmonary infection early after operation.Echocardiogram indicated that the velocity of left superior vena cava increased in the site of the roof of left atrium in one patient after operation.No obstruction of vena cava and pulmonary vein and remnant shunt between the atriums were found in the other 14 cases.There were no death and other complications during3 months to 6 years of follow-up.Conclusion The surgical procedures of unroofed coronary sinus syndrome with endocardial cushion defect and persistent left superior vena cava is difficult and good result can be expected by reestablishing the roof of the coronary sinus.