Outcomes of total cavopulmonary connection in the treatment of functional single ventricle with heterotaxy syndrome: A propensity score matching study_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

HAN Linjiang ^1,2,3 , LIU Xiang ^2,3 , MA Jianrui ^2,3 , ZHOU Ziqin ^2,3 , TU Jiazichao ^1,2,3 , ZHANG Ruyue ^2,3 , TIAN Miao ^1,2,3 , LI Ying ^2,3 , YUAN Haiyun ^2,3 , WEN Shusheng ^2,3 ,  CHEN Jimei ^1,2,3

1. School of Medicine, South China University of Technology, Guangzhou, 510006, P. R. China;
2. Department of Cardiac Surgery, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangdong Cardiovascular Institute, Guangzhou, 510080, P. R. China;
3. Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangzhou, 510080, P. R. China;

Corresponding?author：

CHEN Jimei, Email: jimei_1965@outlook.com

Keywords：

Total cavopulmonary connection; functional single ventricle; heterotaxy syndrome; survival rate

DOI：

10.7507/1007-4848.202310032

Video：

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Objective To comprehensively analyze the clinical outcomes of total cavopulmonary connection (TCPC) in the treatment of functional single ventricle combined with heterotaxy syndrome (HS). Methods A retrospective analysis was conducted on the patients with functional single ventricle and HS who underwent TCPC (a HS group) in Guangdong Provincial People's Hospital between 2004 and 2021. The analysis focused on postoperative complications, long-term survival rates, and identifying factors associated with patient survival. Early and late postoperative outcomes were compared with matched non-HS patients (a non-HS group). Results Before propensity score matching, 55 patients were collected in the HS group, including 42 males and 13 females, with a median age of 6.0 (4.2, 11.8) years and a median weight of 17.0 (14.2, 28.8) kg. Among the patients, there were 53 patients of right atrial isomerism and 2 patients of left atrial isomerism. Eight patients underwent TCPC in one stage. TCPC procedures included extracardiac conduit (n=39), intracardiac-extracardiac conduit (n=14), and direct cavopulmonary connection (n=2). Postoperative complications included infections in 27 patients, liver function damage in 19 patients, and acute kidney injury in 11 patients. There were 5 early deaths. The median follow-up time was 94.7 (64.3, 129.8) months. The 1-year, 5-year, and 10-year survival rates were 87.2%, 85.3%, and 74.3%, respectively. After propensity score matching, there were 45 patients in the HS group and 81 patients in the non-HS group. Compared to the non-HS group, those with HS had longer surgical and mechanical ventilation time, higher infection rates (P<0.05), and a 12.9% lower 10-year survival rate. Multivariate Cox regression analysis identified asplenia was a risk factor for mortality (HR=8.98, 95%CI 1.86-43.34, P=0.006). Conclusion Compared to non-HS patients, patients with HS have lower survival rates after TCPC, and asplenia is an independent risk factor for the survival of these patients.

Citation： HAN Linjiang, LIU Xiang, MA Jianrui, ZHOU Ziqin, TU Jiazichao, ZHANG Ruyue, TIAN Miao, LI Ying, YUAN Haiyun, WEN Shusheng, CHEN Jimei. Outcomes of total cavopulmonary connection in the treatment of functional single ventricle with heterotaxy syndrome: A propensity score matching study. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2024, 31(4): 510-518. doi: 10.7507/1007-4848.202310032 Copy

1.	Ono M, Kasnar-Samprec J, Hager A, et al. Clinical outcome following total cavopulmonary connection: A 20-year single-centre experience. Eur J Cardiothorac Surg, 2016, 50(4): 632-641.
2.	Jacobs JP, Pasquali SK, Morales DL, et al. Heterotaxy: Lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons. World J Pediatr Congenit Heart Surg, 2011, 2(2): 278-286.
3.	Jonas RA. Surgical management of the neonate with heterotaxy and long-term outcomes of heterotaxy. World J Pediatr Congenit Heart Surg, 2011, 2(2): 264-274.
4.	Alsoufi B, McCracken C, Schlosser B, et al. Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome. J Thorac Cardiovasc Surg, 2016, 151(5): 1369-1377.
5.	Vodiskar J, Kido T, Strbad M, et al. Outcomes of single ventricle palliation in infants with heterotaxy syndrome. Eur J Cardiothorac Surg, 2021, 60(3): 554-561.
6.	Pundi KN, Johnson JN, Dearani JA, et al. 40-year follow-up after the Fontan operation: Long-term outcomes of 1, 052 patients. J Am Coll Cardiol, 2015, 66(15): 1700-1710.
7.	Schreiber C, Kostolny M, Weipert J, et al. What was the impact of the introduction of extracardiac completion for a single center performing total cavopulmonary connections? Cardiol Young, 2004, 14(2): 140-147.
8.	Yoshimura N, Yamaguchi M, Oshima Y, et al. Risk factors influencing early and late mortality after total cavopulmonary connection. Eur J Cardiothorac Surg, 2001, 20(3): 598-602.
9.	Zou M, Wang Y, Cui H, et al. Outcomes of total cavopulmonary connection for single ventricle palliation. J Thorac Dis, 2016, 8(1): 43-51.
10.	Marathe SP, Zannino D, Cao JY, et al. Heterotaxy is not a risk factor for adverse long-term outcomes after Fontan completion. Ann Thorac Surg, 2020, 110(2): 646-653.
11.	Pan JY, Lin CC, Wu CJ, et al. Comparable long-term results of the extracardiac conduit total cavopulmonary connection in heterotaxy to non-heterotaxy patients. J Card Surg, 2022, 37(4): 941-947.
12.	Marathe SP, Cao JY, Celermajer D, et al. Outcomes of the Fontan operation for patients with heterotaxy: A meta-analysis of 848 patients. Ann Thorac Surg, 2020, 110(1): 307-315.
13.	中華人民共和國衛生部. 醫院感染診斷標準(試行). 中華醫學雜志, 2001, 81(5): 314-320.
14.	Khwaja A. KDIGO clinical practice guidelines for acute kidney injury. Nephron Clin Pract, 2012, 120(4): c179-c184.
15.	Bartz PJ, Driscoll DJ, Dearani JA, et al. Early and late results of the modified Fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol, 2006, 48(11): 2301-2305.
16.	Stamm C, Friehs I, Duebener LF, et al. Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg, 2002, 74(6): 1967-1977.
17.	Duong SQ, Zaniletti I, Lopez L, et al. Post-operative morbidity and mortality after Fontan procedure in patients with heterotaxy and other situs anomalies. Pediatr Cardiol, 2022, 43(5): 952-959.
18.	劉巍, 劉錦紛, 張海波, 等. 內臟異位綜合征心血管畸形的解剖特征和Fontan手術的療效觀察. 中國胸心血管外科臨床雜志, 2015, 22(8): 729-733.
19.	Chowdhury UK, Anderson RH, Spicer DE, et al. Surgical management of hearts with isomeric atrial appendages. J Card Surg, 2022, 37(5): 1340-1352.
20.	Basgoze S, Temur B, Guvenc O, et al. Analysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery. Cardiol Young, 2022, 32(7): 1129-1135.
21.	曹躍豐, 范祥明, 張巍, 等. 48例心房異構合并心臟畸形外科治療后臨床轉歸. 醫學研究雜志, 2016, 45(7): 88-91.
22.	范沙麗, 劉晶哲, 劉東, 等. CT血管成像診斷先天性心臟病合并內臟異位. 中國介入影像與治療學, 2017, 14(3): 155-158.
23.	Stephens EH, Graham G, Dearani JA, et al. Fontan palliation in patients with heterotaxy syndrome: A five decade experience. World J Pediatr Congenit Heart Surg, 2022, 13(4): 436-442.
24.	Alsoufi B, Gillespie S, Kim D, et al. The impact of dominant ventricle morphology on palliation outcomes of single ventricle anomalies. Ann Thorac Surg, 2016, 102(2): 593-601.
25.	Yurlov IA, Podzolkov VP, Zelenikin MM, et al. Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy. Interact Cardiovasc Thorac Surg, 2011, 12(4): 563-568.
26.	Sano S, Fujii Y, Arai S, et al. Atrioventricular valve repair for patient with heterotaxy syndrome and a functional single ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2012, 15(1): 88-95.
27.	劉曉冰, 岑堅正, 陳寄梅, 等. 功能性單心室合并肝靜脈單獨回流入心房的全腔靜脈-肺動脈連接術. 中國胸心血管外科臨床雜志, 2022, 29(5): 597-601.
28.	Ohuchi H, Kagisaki K, Miyazaki A, et al. Impact of the evolution of the Fontan operation on early and late mortality: A single-center experience of 405 patients over 3 decades. Ann Thorac Surg, 2011, 92(4): 1457-1466.
29.	Desai MH, Ceneri NM, Dhari Z, et al. Cardiac surgical outcomes of patients with heterotaxy syndrome. JTCVS Open, 2022, 13: 292-306.
30.	Cao F, Zou MH, Ma L, et al. Improving outcomes of patients with heterotaxy and functional single ventricle: A 10-year follow-up of 70 cases in a single institution. Zhonghua Wai Ke Za Zhi, 2018, 56(5): 379-385.
31.	Ota N, Fujimoto Y, Murata M, et al. Improving outcomes of the surgical management of right atrial isomerism. Ann Thorac Surg, 2012, 93(3): 832-838.
32.	Bhaskar J, Galati JC, Brooks P, et al. Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery. J Thorac Cardiovasc Surg, 2015, 149(6): 1509-1513.
33.	Graham G, Dearani JA, Niaz T, et al. Outcomes of biventricular and single ventricle heterotaxy patients: A single center five-decade experience. Ann Thorac Surg, 2023, 115(5): 1206-1211.
34.	Orangzeb S, Watle SV, Caugant DA. Adherence to vaccination guidelines of patients with complete splenectomy in Norway, 2008-2020. Vaccine, 2023, 41(31): 4579-4585.

1. Ono M, Kasnar-Samprec J, Hager A, et al. Clinical outcome following total cavopulmonary connection: A 20-year single-centre experience. Eur J Cardiothorac Surg, 2016, 50(4): 632-641.
2. Jacobs JP, Pasquali SK, Morales DL, et al. Heterotaxy: Lessons learned about patterns of practice and outcomes from the congenital heart surgery database of the society of thoracic surgeons. World J Pediatr Congenit Heart Surg, 2011, 2(2): 278-286.
3. Jonas RA. Surgical management of the neonate with heterotaxy and long-term outcomes of heterotaxy. World J Pediatr Congenit Heart Surg, 2011, 2(2): 264-274.
4. Alsoufi B, McCracken C, Schlosser B, et al. Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome. J Thorac Cardiovasc Surg, 2016, 151(5): 1369-1377.
5. Vodiskar J, Kido T, Strbad M, et al. Outcomes of single ventricle palliation in infants with heterotaxy syndrome. Eur J Cardiothorac Surg, 2021, 60(3): 554-561.
6. Pundi KN, Johnson JN, Dearani JA, et al. 40-year follow-up after the Fontan operation: Long-term outcomes of 1, 052 patients. J Am Coll Cardiol, 2015, 66(15): 1700-1710.
7. Schreiber C, Kostolny M, Weipert J, et al. What was the impact of the introduction of extracardiac completion for a single center performing total cavopulmonary connections? Cardiol Young, 2004, 14(2): 140-147.
8. Yoshimura N, Yamaguchi M, Oshima Y, et al. Risk factors influencing early and late mortality after total cavopulmonary connection. Eur J Cardiothorac Surg, 2001, 20(3): 598-602.
9. Zou M, Wang Y, Cui H, et al. Outcomes of total cavopulmonary connection for single ventricle palliation. J Thorac Dis, 2016, 8(1): 43-51.
10. Marathe SP, Zannino D, Cao JY, et al. Heterotaxy is not a risk factor for adverse long-term outcomes after Fontan completion. Ann Thorac Surg, 2020, 110(2): 646-653.
11. Pan JY, Lin CC, Wu CJ, et al. Comparable long-term results of the extracardiac conduit total cavopulmonary connection in heterotaxy to non-heterotaxy patients. J Card Surg, 2022, 37(4): 941-947.
12. Marathe SP, Cao JY, Celermajer D, et al. Outcomes of the Fontan operation for patients with heterotaxy: A meta-analysis of 848 patients. Ann Thorac Surg, 2020, 110(1): 307-315.
13. 中華人民共和國衛生部. 醫院感染診斷標準(試行). 中華醫學雜志, 2001, 81(5): 314-320.
14. Khwaja A. KDIGO clinical practice guidelines for acute kidney injury. Nephron Clin Pract, 2012, 120(4): c179-c184.
15. Bartz PJ, Driscoll DJ, Dearani JA, et al. Early and late results of the modified Fontan operation for heterotaxy syndrome 30 years of experience in 142 patients. J Am Coll Cardiol, 2006, 48(11): 2301-2305.
16. Stamm C, Friehs I, Duebener LF, et al. Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Ann Thorac Surg, 2002, 74(6): 1967-1977.
17. Duong SQ, Zaniletti I, Lopez L, et al. Post-operative morbidity and mortality after Fontan procedure in patients with heterotaxy and other situs anomalies. Pediatr Cardiol, 2022, 43(5): 952-959.
18. 劉巍, 劉錦紛, 張海波, 等. 內臟異位綜合征心血管畸形的解剖特征和Fontan手術的療效觀察. 中國胸心血管外科臨床雜志, 2015, 22(8): 729-733.
19. Chowdhury UK, Anderson RH, Spicer DE, et al. Surgical management of hearts with isomeric atrial appendages. J Card Surg, 2022, 37(5): 1340-1352.
20. Basgoze S, Temur B, Guvenc O, et al. Analysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery. Cardiol Young, 2022, 32(7): 1129-1135.
21. 曹躍豐, 范祥明, 張巍, 等. 48例心房異構合并心臟畸形外科治療后臨床轉歸. 醫學研究雜志, 2016, 45(7): 88-91.
22. 范沙麗, 劉晶哲, 劉東, 等. CT血管成像診斷先天性心臟病合并內臟異位. 中國介入影像與治療學, 2017, 14(3): 155-158.
23. Stephens EH, Graham G, Dearani JA, et al. Fontan palliation in patients with heterotaxy syndrome: A five decade experience. World J Pediatr Congenit Heart Surg, 2022, 13(4): 436-442.
24. Alsoufi B, Gillespie S, Kim D, et al. The impact of dominant ventricle morphology on palliation outcomes of single ventricle anomalies. Ann Thorac Surg, 2016, 102(2): 593-601.
25. Yurlov IA, Podzolkov VP, Zelenikin MM, et al. Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy. Interact Cardiovasc Thorac Surg, 2011, 12(4): 563-568.
26. Sano S, Fujii Y, Arai S, et al. Atrioventricular valve repair for patient with heterotaxy syndrome and a functional single ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2012, 15(1): 88-95.
27. 劉曉冰, 岑堅正, 陳寄梅, 等. 功能性單心室合并肝靜脈單獨回流入心房的全腔靜脈-肺動脈連接術. 中國胸心血管外科臨床雜志, 2022, 29(5): 597-601.
28. Ohuchi H, Kagisaki K, Miyazaki A, et al. Impact of the evolution of the Fontan operation on early and late mortality: A single-center experience of 405 patients over 3 decades. Ann Thorac Surg, 2011, 92(4): 1457-1466.
29. Desai MH, Ceneri NM, Dhari Z, et al. Cardiac surgical outcomes of patients with heterotaxy syndrome. JTCVS Open, 2022, 13: 292-306.
30. Cao F, Zou MH, Ma L, et al. Improving outcomes of patients with heterotaxy and functional single ventricle: A 10-year follow-up of 70 cases in a single institution. Zhonghua Wai Ke Za Zhi, 2018, 56(5): 379-385.
31. Ota N, Fujimoto Y, Murata M, et al. Improving outcomes of the surgical management of right atrial isomerism. Ann Thorac Surg, 2012, 93(3): 832-838.
32. Bhaskar J, Galati JC, Brooks P, et al. Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery. J Thorac Cardiovasc Surg, 2015, 149(6): 1509-1513.
33. Graham G, Dearani JA, Niaz T, et al. Outcomes of biventricular and single ventricle heterotaxy patients: A single center five-decade experience. Ann Thorac Surg, 2023, 115(5): 1206-1211.
34. Orangzeb S, Watle SV, Caugant DA. Adherence to vaccination guidelines of patients with complete splenectomy in Norway, 2008-2020. Vaccine, 2023, 41(31): 4579-4585.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Outcomes of total cavopulmonary connection in the treatment of functional single ventricle with heterotaxy syndrome: A propensity score matching study

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