Clinical analysis of unroofed coronary sinus syndrome with endocardial cushion defect_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

SHI Hao , ZHANG Jing , WANG Qiang , LI Dianyuan , HUA Zhongdong ,  YAN Jun

Chinese Academy of Medical Sciences, Pekin Union Medical College, National Center for Cardiovascular Disease, Fu Wai Hospital, Beijing, 100037, P.R.China;

Corresponding author：

YAN Jun, Email: yanjun.1112@aliyun.com

Keywords：

Coronary vessel anomalies; cardiac surgical procedures; treatment outcome

DOI：

10.7507/1007-4848.201610030

Video：

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Objective To analyze pathologic features and surgical procedures for patients with unroofed coronary sinus syndrome (UCSS) associated with endocardial cushion defect (ECD). Methods The clinical data of 44 patients with UCSS and ECD from May 1998 to July 2016 were retrospectively reviewed. There were 18 males and 26 females with a mean age of 10.4±12.1 years (range: 5.0 months to 44.0 years) and mean weight of 25.2±20.9 kg (range 5.2-80.0 kg). According to the Kirklin and Barratt-Boyes classification, 28 patients were categorized into type Ⅰ, 5 typeⅡ , 4 type Ⅲ and 7 type Ⅳ. Among them 25 patients suffered partial ECD, 10 complete ECD, 9 transitional ECD, and 27 were associated with single atrium, 34 involved persistent left superior vena cava (PLSVC), and in 27 of the 34 patients PLSVC directly drained into the left atrium (LA). Among the 44 patients, 1 patient associated with complex anomalies underwent palliative operation, and other cardiac malformations were corrected simultaneously by surgical correction. PLSVC was ligated in 2 patients, and the intracardiac tunnels or baffles to drain PLSVC to right atrium (RA) were reconstructed in 25 patients. The associated cardiac lesions were corrected concomitantly. Results In-hospital death occurred in 2 patients, among whom 1 died of low cardiac output syndrome on postoperative day 8 and the other pulmonary infection on postoperative day 21. Thirty-one were followed up from 1 month to 10 years, and there was no death or severe complications. Conclusion When ECD is associated with PLSVC and a single atrium, UCSS may develop. Repair according to the type of UCSS is effective.

Citation： SHI Hao, ZHANG Jing, WANG Qiang, LI Dianyuan, HUA Zhongdong, YAN Jun. Clinical analysis of unroofed coronary sinus syndrome with endocardial cushion defect. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2017, 24(9): 672-676. doi: 10.7507/1007-4848.201610030 Copy

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2.	Zhang J, Huang ZX, Sun HS, et al. Clinical analysis of unroofed coronary sinus syndrome with atrioventricular canal defect. Zhonghua Wai Ke Za Zhi, 2009, 47(11): 845-848.
3.	张旌, 孙寒松, 罗新锦, 等. 无顶冠状静脉窦综合征的外科治疗. 中国胸心血管外科临床杂志, 2008, 15(6): 402-405.
4.	van Son JA, Falk V, Mohr FW. Pericardial patch augmentation of restrictive innominate vein and division of left superior vena cava in unroofed coronary sinus syndrome. J Thorac Cardiovasc Surg, 1997, 114(1): 132-134.
5.	Zimand S, Benjamin P, Frand M, et al. Left superior vena cava to the left atrium: Do we have to change the traditional approach? Ann Thorac Surg, 1999, 68(5): 1869-1872.
6.	Helseth HK, Peterson CR. Atrial septal defect with termination of left superior vena cava in the left atrium and absence of the coronary sinus: Recognition and correction. Ann Thorac Surg, 1974, 17(2): 186-192.
7.	李志强, 殷劲, 刘扬, 等. 不同类型无顶冠状静脉窦综合征手术治疗 13 例临床观察. 心肺血管病杂志, 2015, 34(1): 35-38.
8.	梁继河, 刘维永, 刘建萍, 等. 无顶冠状静脉窦综合征. 中华外科杂志, 1996, 34(9): 546-548.
9.	van Son JA, Black MD, Haas GS, et al. Extracadiac repair versus intracardiac baffle repair of complex unroofed coronary sinus. Thorac Cardiovasc Surg, 1998, 46(6): 371-374.
10.	van Son JA, Hambsch J, Mohr FW. Repair of complex unroofed coronary sinus by anastomosis of left to right superior vena cava. Ann Thorac Surg, 1998, 65(1): 280-281.
11.	de Leval MR, Ritter DG, McGoon DC, et al. Anomalous systemic venous connection. Surgical considerations. Mayo Clin Proc, 1975, 50(10): 599-610.
12.	Muthialu N, Fajardo D, Sullivan ID, et al. Repair of persistent left superior vena cava to unroofed coronary sinus defect by retro-aortic implantation (modified warden type procedure). J Card Surg, 2016, 31(2): 103-105.
13.	Takach TJ, Cortelli M, Lonquist JL, et al. Correction of anomalous systemic venous drainage: transposition of left SVC to left PA. Ann Thorac Surg, 1997, 63(1): 228-230.
14.	Chihara S, Yasunaga H, Todo K. anastomosis of left to right superior vena cava for repair of unroofed coronary sinus. Gen Thorac Cardiovasc Surg, 2012, 60(4): 244-246.

1. Kirklin JW, Barratt-Boyes BG. Unroofed coronary sinus syndrome. In: Kirklin JW, Barratt-Boyes BG. Cardiac surgery. 2^nd ed. New York: Churchill-Livingstone, 1993. 683-692.
2. Zhang J, Huang ZX, Sun HS, et al. Clinical analysis of unroofed coronary sinus syndrome with atrioventricular canal defect. Zhonghua Wai Ke Za Zhi, 2009, 47(11): 845-848.
3. 张旌, 孙寒松, 罗新锦, 等. 无顶冠状静脉窦综合征的外科治疗. 中国胸心血管外科临床杂志, 2008, 15(6): 402-405.
4. van Son JA, Falk V, Mohr FW. Pericardial patch augmentation of restrictive innominate vein and division of left superior vena cava in unroofed coronary sinus syndrome. J Thorac Cardiovasc Surg, 1997, 114(1): 132-134.
5. Zimand S, Benjamin P, Frand M, et al. Left superior vena cava to the left atrium: Do we have to change the traditional approach? Ann Thorac Surg, 1999, 68(5): 1869-1872.
6. Helseth HK, Peterson CR. Atrial septal defect with termination of left superior vena cava in the left atrium and absence of the coronary sinus: Recognition and correction. Ann Thorac Surg, 1974, 17(2): 186-192.
7. 李志强, 殷劲, 刘扬, 等. 不同类型无顶冠状静脉窦综合征手术治疗 13 例临床观察. 心肺血管病杂志, 2015, 34(1): 35-38.
8. 梁继河, 刘维永, 刘建萍, 等. 无顶冠状静脉窦综合征. 中华外科杂志, 1996, 34(9): 546-548.
9. van Son JA, Black MD, Haas GS, et al. Extracadiac repair versus intracardiac baffle repair of complex unroofed coronary sinus. Thorac Cardiovasc Surg, 1998, 46(6): 371-374.
10. van Son JA, Hambsch J, Mohr FW. Repair of complex unroofed coronary sinus by anastomosis of left to right superior vena cava. Ann Thorac Surg, 1998, 65(1): 280-281.
11. de Leval MR, Ritter DG, McGoon DC, et al. Anomalous systemic venous connection. Surgical considerations. Mayo Clin Proc, 1975, 50(10): 599-610.
12. Muthialu N, Fajardo D, Sullivan ID, et al. Repair of persistent left superior vena cava to unroofed coronary sinus defect by retro-aortic implantation (modified warden type procedure). J Card Surg, 2016, 31(2): 103-105.
13. Takach TJ, Cortelli M, Lonquist JL, et al. Correction of anomalous systemic venous drainage: transposition of left SVC to left PA. Ann Thorac Surg, 1997, 63(1): 228-230.
14. Chihara S, Yasunaga H, Todo K. anastomosis of left to right superior vena cava for repair of unroofed coronary sinus. Gen Thorac Cardiovasc Surg, 2012, 60(4): 244-246.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Clinical analysis of unroofed coronary sinus syndrome with endocardial cushion defect

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