Superior Vena Cava and Pulmonary Connection Procedure for Patients with Single Ventricle with Total Anomalous Pulmonary Venous_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

HEYan ,  LIUYing-long , SUJun-wu , CHENYan , ZHANGJing , LIZhi-qiang

1. Department of Pediatirc Cardiology, Anzhen Hospital, Capital Medical University, Beijing 100029, P.R.China;
2. Department of Intensive Care Unit, Anzhen Hospital, Capital Medical University, Beijing 100029, P.R.China;

Corresponding?author：

LIUYing-long, Email: hybicq@qq.com

Keywords：

Congenital heart defects; Glenn procedure; Total anomalous pulmonary venous (TAPVC)

DOI：

10.7507/1007-4848.20160083

Video：

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Objective To summarize the experience of the superior vena cava and pulmonary connection surgery for functional single ventricle (SV) with total anomalous pulmonary venous (TAPVC). Methods We retrospectively analyzed the clinical data of 10 patients with SV and TAPVC in our hospital from January 2012 through June 2014. There were 7 males and 3 females at average age of 90.33±86.53 months. The 10 patients were with right atrial isomerism, 9 with heterotary and asplenia syndrome. Five patients were anatomic single ventricle and others were with functional uni-ventricle. Nine patients were with supracardiac pattern TAPVC and one was with intracardiac TAPVC. All patients were operated unilateral or bilateral bidirectional Glenn procedure with TAPVC correction. Results The arterial oxygen saturation (SaO₂) increased prominently after operation (86%±6% vs. 79%±6%, P<0.01). There were 3 patients with low cardiac output syndrome, one patient with severe arrhythmia, 4 patients with serious pleural effusion, 4 patients with hospital-acquired infection, and 3 patients with central nervous system complications (epilepsy or hemiplegia). One died because of hemorrhage and pulmonary thrombosis, and the other died of hypoxemia and mutiple organ dysfunction syndrome (MODS). Conclusion Glenn is one of palliated procedure choice for SV/TAPVC patients. The indication for surgery and perioperative management individually is crucial.

Citation： HEYan, LIUYing-long, SUJun-wu, CHENYan, ZHANGJing, LIZhi-qiang. Superior Vena Cava and Pulmonary Connection Procedure for Patients with Single Ventricle with Total Anomalous Pulmonary Venous. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2016, 23(4): 362-365. doi: 10.7507/1007-4848.20160083 Copy

1.	Jonas RA. Fontan or septation: When I abandon septation in complex lesion with two ventricles. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2009, 12(1): 94-98.
2.	劉迎龍, 于存濤, 魏波, 等. 非體外循環下行雙向格林手術(附58例報道). 中華胸心血管外科雜志, 2003, 19 (1): 4-6.
3.	周誠, 董念國, 杜心靈, 等. 姑息性手術在復雜先天性心臟病患者中的應用. 中國胸心血管外科臨床雜志, 2012, 19 (5): 494-497.
4.	張為民, 張晶, 吳祖凱, 等. 雙向Glenn手術治療紫紺型復雜先天性心臟病. 中國胸心血管外科臨床雜志, 2014, 21 (4): 482-485.
5.	Lee JR, Choi JS, Kang CH, et al. Surgical results of patients with a functional single ventricle. Eur J Cardiothorac Surg, 2003, 24 (5): 716-722.
6.	Hashmi A, Abu-Sulaiman R, McCrindle BW, et al. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol, 1998, 31 (5): 1120-1126.
7.	Yun TJ, Al-Radi OO, Adatia I, et al. Contemporary management of right atrial isomerism: effect of evolving therapeutic strategies. J Thorac Cardiovasc Surg, 2006, 131 (5): 1108-1113.
8.	Morales DL, Braud BE, Booth JH, et al. Heterotaxy patients with total anomalous pulmonary venous return: improving surgical result. Ann Thorac Surg, 2006, 82 (5): 1621-1628.
9.	Sachdev MS, Jena PK, Kurup RP, et al. Outcome of single ventricle and total anomalous pulmonary venous connection. Asian Cardio-vasc Thorac Ann, 2006, 14 (5): 367-370.
10.	Hancock-Friesen CL, Zurakowski D, Thiagarajan RR, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg, 2005, 79 (2): 596-606.
11.	Sinzobahamvya N, Arenz C, Reckers J, et al. Poor outcome for patients with total anomalous pulmonary venous connection and functionally single ventricle. Cardiol young, 2009, 19 (6): 594-600.
12.	Nakayama Y, Hiramatsu T, Iwata Y, et al. Surgical results for functional univentricular heart with TAPVC over a 25-year experience. Ann Thorac Surg, 2012, 93 (2): 606-613.
13.	Sinzobahamvya N, Arenz C, Brecher AM, et al. Atrial isomerism: a surgical experience. Cardiovasc Surg, 1999, 7 (4): 436-442.
14.	Lodge AJ, Rychik J, Nicolson SC, et al. Improving outcome in functional single ventricle and total anomalous pulmonary venous connection. Ann Thorac Surg, 2004, 78 (5): 1688-1695.
15.	Kelle AM, Backer CL, Gossett JG, et al. TAPVC: results of surgical repair of 100 patients at a single institution. J Thorac Cardiovasc Surg, 2010, 139 (6): 1387-1394.
16.	Gaynor JW, Collins MH, Rychik J, et al. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. Thorac Cardiovasc Surg, 1999, 117 (3): 506-514.
17.	Sadiq M, Stumper O, De Giovanni JV, et al. Management and outcome of infants and children with right atrial isomerism. Heart, 1996, 75 (3): 314-319.
18.	Husain SA, Maldonado E, Rasch D, et al. TAPVC: factors associated with mortality and recurrent pulmonary venous obstruction. Ann Thorac Surg, 2012, 94 (3): 825-832.
19.	Nakata T, Fujimoto Y, Hirose K, et al. Functional single ventricle with extracardiac total anomalous pulmonary venous connection. Europ J Cardiothorac Surg, 2009, 36 (1): 49-56.
20.	O’Donnell CP, Lock JE, Powell AJ, et al. Compression of pulmonary veins between the left atrium and the descending aorta. Am J Cardiol, 2003, 91 (1): 248-251.
21.	Van Son JA, Mohr FW, Hambsch J, et al. Conversion of atriopulmonary or lateral atrial tunnel cavopulnoary anastomosis to extracardiac conduit Fontan modification. Eur J Cardiothorac Surg, 1999, 15 (2): 150-158.
22.	Ungerleider RM, Johnston TA, O’Laughlin MP, et al. Intraoperative stents to rehabilitate severely stenotic pulmonary vessels. Ann Thorac Surg, 2001, 71 (2): 476-481.
23.	Tomita H, Watanabe K, Yazaki S, et al. Stent implantation and subsequent dilatation for pulmonary vein stenosis in pediatric patients: maximizing effectiveness. Circ J, 2003, 67 (3): 187-190.
24.	劉錦紛，祝忠群，蘇肇伉，等. 右房異構單心室伴完全性肺靜脈異位引流的外科治療. 中華胸心血管外科雜志, 2001, 17 (4): 207-209.
25.	賀彥, 劉迎龍, 陳焱, 等. 吸入伊洛前列素在Glenn術后的應用研究. 心肺血管病雜志, 2012, 31 (4): 389-391.

1. Jonas RA. Fontan or septation: When I abandon septation in complex lesion with two ventricles. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2009, 12(1): 94-98.
2. 劉迎龍, 于存濤, 魏波, 等. 非體外循環下行雙向格林手術(附58例報道). 中華胸心血管外科雜志, 2003, 19 (1): 4-6.
3. 周誠, 董念國, 杜心靈, 等. 姑息性手術在復雜先天性心臟病患者中的應用. 中國胸心血管外科臨床雜志, 2012, 19 (5): 494-497.
4. 張為民, 張晶, 吳祖凱, 等. 雙向Glenn手術治療紫紺型復雜先天性心臟病. 中國胸心血管外科臨床雜志, 2014, 21 (4): 482-485.
5. Lee JR, Choi JS, Kang CH, et al. Surgical results of patients with a functional single ventricle. Eur J Cardiothorac Surg, 2003, 24 (5): 716-722.
6. Hashmi A, Abu-Sulaiman R, McCrindle BW, et al. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol, 1998, 31 (5): 1120-1126.
7. Yun TJ, Al-Radi OO, Adatia I, et al. Contemporary management of right atrial isomerism: effect of evolving therapeutic strategies. J Thorac Cardiovasc Surg, 2006, 131 (5): 1108-1113.
8. Morales DL, Braud BE, Booth JH, et al. Heterotaxy patients with total anomalous pulmonary venous return: improving surgical result. Ann Thorac Surg, 2006, 82 (5): 1621-1628.
9. Sachdev MS, Jena PK, Kurup RP, et al. Outcome of single ventricle and total anomalous pulmonary venous connection. Asian Cardio-vasc Thorac Ann, 2006, 14 (5): 367-370.
10. Hancock-Friesen CL, Zurakowski D, Thiagarajan RR, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg, 2005, 79 (2): 596-606.
11. Sinzobahamvya N, Arenz C, Reckers J, et al. Poor outcome for patients with total anomalous pulmonary venous connection and functionally single ventricle. Cardiol young, 2009, 19 (6): 594-600.
12. Nakayama Y, Hiramatsu T, Iwata Y, et al. Surgical results for functional univentricular heart with TAPVC over a 25-year experience. Ann Thorac Surg, 2012, 93 (2): 606-613.
13. Sinzobahamvya N, Arenz C, Brecher AM, et al. Atrial isomerism: a surgical experience. Cardiovasc Surg, 1999, 7 (4): 436-442.
14. Lodge AJ, Rychik J, Nicolson SC, et al. Improving outcome in functional single ventricle and total anomalous pulmonary venous connection. Ann Thorac Surg, 2004, 78 (5): 1688-1695.
15. Kelle AM, Backer CL, Gossett JG, et al. TAPVC: results of surgical repair of 100 patients at a single institution. J Thorac Cardiovasc Surg, 2010, 139 (6): 1387-1394.
16. Gaynor JW, Collins MH, Rychik J, et al. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. Thorac Cardiovasc Surg, 1999, 117 (3): 506-514.
17. Sadiq M, Stumper O, De Giovanni JV, et al. Management and outcome of infants and children with right atrial isomerism. Heart, 1996, 75 (3): 314-319.
18. Husain SA, Maldonado E, Rasch D, et al. TAPVC: factors associated with mortality and recurrent pulmonary venous obstruction. Ann Thorac Surg, 2012, 94 (3): 825-832.
19. Nakata T, Fujimoto Y, Hirose K, et al. Functional single ventricle with extracardiac total anomalous pulmonary venous connection. Europ J Cardiothorac Surg, 2009, 36 (1): 49-56.
20. O’Donnell CP, Lock JE, Powell AJ, et al. Compression of pulmonary veins between the left atrium and the descending aorta. Am J Cardiol, 2003, 91 (1): 248-251.
21. Van Son JA, Mohr FW, Hambsch J, et al. Conversion of atriopulmonary or lateral atrial tunnel cavopulnoary anastomosis to extracardiac conduit Fontan modification. Eur J Cardiothorac Surg, 1999, 15 (2): 150-158.
22. Ungerleider RM, Johnston TA, O’Laughlin MP, et al. Intraoperative stents to rehabilitate severely stenotic pulmonary vessels. Ann Thorac Surg, 2001, 71 (2): 476-481.
23. Tomita H, Watanabe K, Yazaki S, et al. Stent implantation and subsequent dilatation for pulmonary vein stenosis in pediatric patients: maximizing effectiveness. Circ J, 2003, 67 (3): 187-190.
24. 劉錦紛，祝忠群，蘇肇伉，等. 右房異構單心室伴完全性肺靜脈異位引流的外科治療. 中華胸心血管外科雜志, 2001, 17 (4): 207-209.
25. 賀彥, 劉迎龍, 陳焱, 等. 吸入伊洛前列素在Glenn術后的應用研究. 心肺血管病雜志, 2012, 31 (4): 389-391.

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Superior Vena Cava and Pulmonary Connection Procedure for Patients with Single Ventricle with Total Anomalous Pulmonary Venous

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