Surgical treatment and mid-term results of functional single ventricle associated with total anomalous pulmonary venous connection_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

LI Bin , LIU Aijun , SI Rui , YANG Ming , FAN Xiangming ,  SU Junwu

Department of Pediatric Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing, 100029, P.R.China;

Corresponding author：

SU Junwu, Email: azsujunwu@126.com

Keywords：

Single ventricle; total anomalous pulmonary venous connection; congenital heart disease; surgery

DOI：

10.7507/1007-4848.202009050

Video：

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Objective To evaluate the mid-term results of surgical treatment for functional single ventricle associated with total anomalous pulmonary venous connection.Methods We reviewed the clinical data and follow-up results of 12 patients in our hospital who underwent both single ventricle series palliation and total anomalous pulmonary venous connection correction from 2008 to 2018. There were 6 males and 6 females at age of 2.3 (1-21) years. Univariable and multivariable Cox proportional hazard regression methods were performed. The Kaplan-Meier method was used to estimate the survival rate.Results All patients were successfully removed from extracorporeal circulation. The cardiopulmonary bypass time was 113.8±42.5 min. The myocardial block time was 57.7±31.7 min. There were 3 in-hospital deaths and 4 late deaths. The causes of death in hospital were pulmonary hypertension, pulmonary hemorrhage, and hypoxemia. During follow-up, the cause of death was heart failure. The survival rate at 1 year and 3 years was 58.3% (95%CI 27% to 80%) and 40% (95%CI 13% to 65%), respectively. Median follow-up was 48.3 months (range: 1 to 118 months).Conclusion Functional single ventricle combined with complete pulmonary venous drainage is a serious clinical disease, with poor therapeutic effect and high overall mortality.

Citation： LI Bin, LIU Aijun, SI Rui, YANG Ming, FAN Xiangming, SU Junwu. Surgical treatment and mid-term results of functional single ventricle associated with total anomalous pulmonary venous connection. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2021, 28(3): 344-348. doi: 10.7507/1007-4848.202009050 Copy

1.	杜欣为, 孙彦隽, 祝忠群, 等. 功能性单心室合并完全性肺静脉异位引流的外科策略与结果. 中国胸心血管外科临床杂志, 2018, 25(2): 118-122.
2.	Hoashi T, Kagisaki K, Oda T, et al. Long-term results of treatments for functional single ventricle associated with extracardiac type total anomalous pulmonary venous connection. Eur J Cardiothorac Surg, 2013, 43(5): 965-970.
3.	Sugano M, Murata M, Ide Y, et al. Midterm results and risk factors of functional single ventricles with extracardiac total anomalous pulmonary venous connection. Gen Thorac Cardiovasc Surg, 2019, 67(11): 941-948.
4.	Yong MS, Zhu M, du Plessis K, et al. Long-term outcomes of the fontan operation in patients with total anomalous pulmonary venous drainage. Ann Thorac Surg, 2019, 108(4): 1234-1241.
5.	Okamoto T, Nakano T, Goda M, et al. Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection. Gen Thorac Cardiovasc Surg, 2020. [Epub ahead of print].
6.	Nakayama Y, Hiramatsu T, Iwata Y, et al. Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over a 25-year experience. Ann Thorac Surg, 2012, 93(2): 606-613.
7.	Lodge AJ, Rychik J, Nicolson SC, et al. Improving outcomes in functional single ventricle and total anomalous pulmonary venous connection. Ann Thorac Surg, 2004, 78(5): 1688-1695.
8.	Gaynor JW, Collins MH, Rychik J, et al. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg, 1999, 117(3): 506-514.
9.	Khan MS, Bryant R 3rd, Kim SH, et al. Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg, 2015, 99(6): 2134-2140.
10.	曹凡, 邹明晖, 马力等. 内脏异位综合征合并单心室外科治疗 70 例 10 年随访分析. 中华外科杂志, 2018, 56(5): 379-385.

1. 杜欣为, 孙彦隽, 祝忠群, 等. 功能性单心室合并完全性肺静脉异位引流的外科策略与结果. 中国胸心血管外科临床杂志, 2018, 25(2): 118-122.
2. Hoashi T, Kagisaki K, Oda T, et al. Long-term results of treatments for functional single ventricle associated with extracardiac type total anomalous pulmonary venous connection. Eur J Cardiothorac Surg, 2013, 43(5): 965-970.
3. Sugano M, Murata M, Ide Y, et al. Midterm results and risk factors of functional single ventricles with extracardiac total anomalous pulmonary venous connection. Gen Thorac Cardiovasc Surg, 2019, 67(11): 941-948.
4. Yong MS, Zhu M, du Plessis K, et al. Long-term outcomes of the fontan operation in patients with total anomalous pulmonary venous drainage. Ann Thorac Surg, 2019, 108(4): 1234-1241.
5. Okamoto T, Nakano T, Goda M, et al. Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection. Gen Thorac Cardiovasc Surg, 2020. [Epub ahead of print].
6. Nakayama Y, Hiramatsu T, Iwata Y, et al. Surgical results for functional univentricular heart with total anomalous pulmonary venous connection over a 25-year experience. Ann Thorac Surg, 2012, 93(2): 606-613.
7. Lodge AJ, Rychik J, Nicolson SC, et al. Improving outcomes in functional single ventricle and total anomalous pulmonary venous connection. Ann Thorac Surg, 2004, 78(5): 1688-1695.
8. Gaynor JW, Collins MH, Rychik J, et al. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg, 1999, 117(3): 506-514.
9. Khan MS, Bryant R 3rd, Kim SH, et al. Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg, 2015, 99(6): 2134-2140.
10. 曹凡, 邹明晖, 马力等. 内脏异位综合征合并单心室外科治疗 70 例 10 年随访分析. 中华外科杂志, 2018, 56(5): 379-385.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Surgical treatment and mid-term results of functional single ventricle associated with total anomalous pulmonary venous connection

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