Surgical management of total cavopulmonary connection procedure in functional single ventricle with separate hepatic venous drainage_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

LIU Xiaobing , CEN Jianzheng , CHEN Jimei , WEN Shusheng , XU Gang , QIU Hailong , YU Juemin ,  ZHUANG Jian

Department of Cardiovascular Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital, Guangdong Academy of Medical Sciences, Guangzhou, 510080, P. R. China;

Corresponding author：

ZHUANG Jian, Email: zhuangjian5413@163.com

Keywords：

Separate hepatic venous drainage; total cavopulmonary connection; functional single ventricle; heterotaxy syndrome

DOI：

10.7507/1007-4848.202103124

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Objective To investigate the management of hepatic vein (HV) in patients with functional single ventricle (FSV) and separate hepatic venous drainage (SHVD) undergoing total cavopulmonary connection (TCPC) and evaluate this kind of surgery.Methods The clinical data of 32 consecutive patients with SHVD who underwent modified TCPC operation from August 2005 to January 2017 in our center were retrospectively analyzed. There were 25 males and 7 females with an average age of 2-19 (8.0±5.0) years and body weight of 11-66 (25.4±15.8) kg.Results There were 20 patients with heterotaxy syndrome and 12 patients with other types of FSV. SHVD was diagnosed preoperatively in 27 patients, among whom 20 patients were connected by intra-extracardiac Gore-Tex conduit, and the other 7 patients were connected by extracardiac Gore-Tex conduit. Because of the missed diagnosis of SHVD, the other 5 patients showed severe decrease of blood oxygen saturation in the early postoperative period and underwent re-operation soon. The postoperative blood oxygen saturation was 92.0% (90.0%, 96.0%), central venous pressure was 10-23 (15.5±3.5) mm Hg, mechanical ventilation assisted time was 16.0 (7.5, 24.0) h, and ICU stay time was 3.0 (2.0, 5.5) d. There were 3 early and 1 late deaths.Conclusion Intra-extracardiac conduit is an effective and feasible modified TCPC operation for patients with FSV and SHVD, while the surgical details need to be formulated in combination with individual anatomical structure. Preoperative missed diagnosis of SHVD must be avoided. Otherwise, after TCPC, a large amount of stealing blood from HV with low circulation pressure into atrium would lead to unacceptable hypoxemia.

Citation： LIU Xiaobing, CEN Jianzheng, CHEN Jimei, WEN Shusheng, XU Gang, QIU Hailong, YU Juemin, ZHUANG Jian. Surgical management of total cavopulmonary connection procedure in functional single ventricle with separate hepatic venous drainage. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2022, 29(5): 597-601. doi: 10.7507/1007-4848.202103124 Copy

1.	Moodie DS, Ritter DG, Tajik AJ, et al. Long-term follow-up in the unoperated univentricular heart. Am J Cardiol, 1984, 53(8): 1124-1128.
2.	Constantine Mavroudis, Carl L. Backer, 主编. 刘锦纷, 孙彦隽, 主译. 小儿心脏外科学. 第四版. 上海: 世界图书出版公司, 2014: 617.
3.	Broda CR, Taylor DA, Adachi I. Progress in experimental and clinical subpulmonary assistance for Fontan circulation. J Thorac Cardiovasc Surg, 2018, 156(5): 1949-1956.
4.	Montesa C, Karamlou T, Ratnayaka K, et al. Hepatic vein incorporation into the azygos system in heterotaxy and interrupted inferior vena cava. World J Pediatr Congenit Heart Surg, 2019, 10(3): 330-337.
5.	McElhinney DB, Marx GR, Marshall AC, et al. Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation. J Thorac Cardiovasc Surg, 2011, 141(6): 1362-1370.
6.	Hsu KH, Chang CI. Total cavopulmonary connection with innovative combined lateral tunnel and extracardiac conduit in heterotaxy syndrome. Ann Thorac Surg, 2011, 92(5): 1907-1908.
7.	McElhinney DB, Marx GR, Newburger JW. Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: A case series and literature review. Congenit Heart Dis, 2011, 6(1): 28-40.
8.	Nakata T, Fujimoto Y, Hirose K, et al. Fontan completion in patients with atrial isomerism and separate hepatic venous drainage. Eur J Cardiothorac Surg, 2010, 37(6): 1264-1270.
9.	Sernich S, Ross-Ascuitto N, Dorotan J, et al. Surgical improvement of hepatic venous mixing to resolve systemic arterial hypoxemia associated with post-Fontan pulmonary arteriovenous fistulae. Tex Heart Inst J, 2009, 36(5): 480-482.
10.	Wu IH, Nguyen KH. Redirection of hepatic drainage for treatment of pulmonary arteriovenous malformations following the Fontan procedure. Pediatr Cardiol, 2006, 27(4): 519-522.
11.	岑坚正, 庄建, 陈寄梅, 等. 功能性单心室合并完全肺静脉异位引流的外科治疗. 中华胸心血管外科杂志, 2016, 32(3): 136-139.
12.	Yong MS, Zhu MZL, 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.
13.	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, 2021, 69(4): 646-653.
14.	王菊, 王强, 李巅远, 等. 肝静脉直接入心房的(功能)单心室患者的改良 Fontan 术式及早中期疗效. 中国循环杂志, 2017, 32(6): 599-602.
15.	Nakata T, Fujimoto Y, Hirose K, et al. Fontan completion in patients with atrial isomerism and separate hepatic venous drainage. Eur J Cardiothorac Surg, 2010, 37(6): 1264-1270.
16.	Rothman A, Acherman RJ, Luna CF, et al. Enlarged left vitelline vein remnant as a cause of cyanosis after the Fontan procedure: Resolution with an Amplatzer vascular plug. Pediatr Cardiol, 2006, 27(3): 381-384.

1. Moodie DS, Ritter DG, Tajik AJ, et al. Long-term follow-up in the unoperated univentricular heart. Am J Cardiol, 1984, 53(8): 1124-1128.
2. Constantine Mavroudis, Carl L. Backer, 主编. 刘锦纷, 孙彦隽, 主译. 小儿心脏外科学. 第四版. 上海: 世界图书出版公司, 2014: 617.
3. Broda CR, Taylor DA, Adachi I. Progress in experimental and clinical subpulmonary assistance for Fontan circulation. J Thorac Cardiovasc Surg, 2018, 156(5): 1949-1956.
4. Montesa C, Karamlou T, Ratnayaka K, et al. Hepatic vein incorporation into the azygos system in heterotaxy and interrupted inferior vena cava. World J Pediatr Congenit Heart Surg, 2019, 10(3): 330-337.
5. McElhinney DB, Marx GR, Marshall AC, et al. Cavopulmonary pathway modification in patients with heterotaxy and newly diagnosed or persistent pulmonary arteriovenous malformations after a modified Fontan operation. J Thorac Cardiovasc Surg, 2011, 141(6): 1362-1370.
6. Hsu KH, Chang CI. Total cavopulmonary connection with innovative combined lateral tunnel and extracardiac conduit in heterotaxy syndrome. Ann Thorac Surg, 2011, 92(5): 1907-1908.
7. McElhinney DB, Marx GR, Newburger JW. Congenital portosystemic venous connections and other abdominal venous abnormalities in patients with polysplenia and functionally univentricular heart disease: A case series and literature review. Congenit Heart Dis, 2011, 6(1): 28-40.
8. Nakata T, Fujimoto Y, Hirose K, et al. Fontan completion in patients with atrial isomerism and separate hepatic venous drainage. Eur J Cardiothorac Surg, 2010, 37(6): 1264-1270.
9. Sernich S, Ross-Ascuitto N, Dorotan J, et al. Surgical improvement of hepatic venous mixing to resolve systemic arterial hypoxemia associated with post-Fontan pulmonary arteriovenous fistulae. Tex Heart Inst J, 2009, 36(5): 480-482.
10. Wu IH, Nguyen KH. Redirection of hepatic drainage for treatment of pulmonary arteriovenous malformations following the Fontan procedure. Pediatr Cardiol, 2006, 27(4): 519-522.
11. 岑坚正, 庄建, 陈寄梅, 等. 功能性单心室合并完全肺静脉异位引流的外科治疗. 中华胸心血管外科杂志, 2016, 32(3): 136-139.
12. Yong MS, Zhu MZL, 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.
13. 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, 2021, 69(4): 646-653.
14. 王菊, 王强, 李巅远, 等. 肝静脉直接入心房的(功能)单心室患者的改良 Fontan 术式及早中期疗效. 中国循环杂志, 2017, 32(6): 599-602.
15. Nakata T, Fujimoto Y, Hirose K, et al. Fontan completion in patients with atrial isomerism and separate hepatic venous drainage. Eur J Cardiothorac Surg, 2010, 37(6): 1264-1270.
16. Rothman A, Acherman RJ, Luna CF, et al. Enlarged left vitelline vein remnant as a cause of cyanosis after the Fontan procedure: Resolution with an Amplatzer vascular plug. Pediatr Cardiol, 2006, 27(3): 381-384.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Surgical management of total cavopulmonary connection procedure in functional single ventricle with separate hepatic venous drainage

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