Surgical treatment of mixed total anomalous pulmonary venous connection_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

WEN Chen ¹ , ZHU Fang ¹ , ZHANG Qian ¹ , HU Chen ² , CHEN Hao ¹ , QIU Lisheng ¹ , SHI Guocheng ¹ , ZHANG Hao ¹ , ZHU Zhongqun ¹ ,  CHEN Huiwen ¹

1. Department of Cardiothoracic Surgery, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, 200127, P.R.China;
2. Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, P.R.China;

Corresponding author：

CHEN Huiwen, Email: chenhuiwen@scmc.com.cn

Keywords：

Total anomalous pulmonary venous connection; congenital heart disease; surgery

DOI：

10.7507/1007-4848.201911042

Video：

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Objective To analyze the outcomes of surgical repair for mixed total anomalous pulmonary venous connection (TAPVC).Methods Between 2006 and 2018, a total of 51 patients with mixed TAPVC underwent surgery in our hospital. Patients with such associated anomalies as single ventricle and tetralogy of Fallot were excluded. There were 35 males and 16 females with a median age of 102.0 (59.0, 181.0) days and a median weight of 5.0 (4.1, 6.4) kg. Patients were divided into three categories based on the anatomy: "3+1" pattern (n=38, three pulmonary veins drained at one site, and the other drained at the opposite site); "2+2" pattern (n=9, the pulmonary veins from each lung joined to form a confluence and drained at separate sites); bizarre pattern (n=4, the anatomy could not be classified into the above two patterns).Results There was no in-hospital death. The median follow-up was 41.0 (18.0, 86.5) months. Postoperative pulmonary venous obstruction occurred in 10 patients. Kaplan-Meier survival curves showed no statistically significant difference in postoperative pulmonary venous obstruction among the three groups (P=0.239). Cox risk regression showed that preoperative pulmonary venous obstruction was significantly associated with postoperative pulmonary venous obstruction (P=0.024).Conclusion Mixed TAPVC has various anatomic morphologies and requires individualized surgery.

Citation： WEN Chen, ZHU Fang, ZHANG Qian, HU Chen, CHEN Hao, QIU Lisheng, SHI Guocheng, ZHANG Hao, ZHU Zhongqun, CHEN Huiwen. Surgical treatment of mixed total anomalous pulmonary venous connection. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2020, 27(4): 415-420. doi: 10.7507/1007-4848.201911042 Copy

1.	St Louis JD, Harvey BA, Menk JS, et al. Repair of "simple" total anomalous pulmonary venous connection: a review from the Pediatric Cardiac Care Consortium. Ann Thorac Surg, 2012, 94(1): 133-137.
2.	Shi G, Zhu Z, Chen J, et al. Total anomalous pulmonary venous connection: the current management strategies in a pediatric cohort of 768 patients. Circulation, 2017, 135(1): 48-58.
3.	Chowdhury UK, Airan B, Malhotra A, et al. Mixed total anomalous pulmonary venous connection: anatomic variations, surgical approach, techniques, and results. J Thorac Cardiovasc Surg, 2008, 135(1): 106-116.
4.	Chowdhury UK, Malhotra A, Kothari SS, et al. A suggested new surgical classification for mixed totally anomalous pulmonary venous connection. Cardiol Young, 2007, 17(4): 342-353.
5.	Ricci M, Elliott M, Cohen GA, et al. Management of pulmonary venous obstruction after correction of TAPVC: risk factors for adverse outcome. Eur J Cardiothorac Surg, 2003, 24(1): 28-36.
6.	St Louis JD, Turk EM, Jacobs JP, et al. Type Ⅳ total anomalous pulmonary venous connection. World J Pediatr Congenit Heart Surg, 2017, 8(2): 142-147.
7.	Zea-Vera R, Mery CM. Mixed total veins: the epitome of the few, the disparate, and the obscure. Semin Thorac Cardiovasc Surg, 2017, 29(3): 345-346.
8.	Furlanetto G, Furlanetto BH, Henriques SR, et al. Mixed type total anomalous pulmonary venous connection: early results and surgical techniques. World J Pediatr Congenit Heart Surg, 2015, 6(1): 26-32.
9.	Shen Q, Pa M, Hu X, et al. Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection. Pediatr Radiol, 2013, 43(7): 827-835.
10.	Shim H, Yang JH, Jun TG. Two-stage correction of type Ⅳ total anomalous pulmonary venous connection. J Cardiothorac Surg, 2017, 12(1): 54.
11.	Alsoufi B. Cracking the mystery of pulmonary vein stenosis. Semin Thorac Cardiovasc Surg, 2019, 31(2): 274-276.
12.	Yong MS, Yaftian N, Weintraub RG, et al. Outcomes of surgery for mixed total anomalous pulmonary venous drainage in children. Semin Thorac Cardiovasc Surg, 2017, 29(3): 338-344.
13.	Eyries M, Montani D, Girerd B, et al. EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat Genet, 2014, 46(1): 65-69.
14.	Honjo O, Atlin CR, Hamilton BC, et al. Primary sutureless repair for infants with mixed total anomalous pulmonary venous drainage. Ann Thorac Surg, 2010, 90(3): 862-868.
15.	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.

1. St Louis JD, Harvey BA, Menk JS, et al. Repair of "simple" total anomalous pulmonary venous connection: a review from the Pediatric Cardiac Care Consortium. Ann Thorac Surg, 2012, 94(1): 133-137.
2. Shi G, Zhu Z, Chen J, et al. Total anomalous pulmonary venous connection: the current management strategies in a pediatric cohort of 768 patients. Circulation, 2017, 135(1): 48-58.
3. Chowdhury UK, Airan B, Malhotra A, et al. Mixed total anomalous pulmonary venous connection: anatomic variations, surgical approach, techniques, and results. J Thorac Cardiovasc Surg, 2008, 135(1): 106-116.
4. Chowdhury UK, Malhotra A, Kothari SS, et al. A suggested new surgical classification for mixed totally anomalous pulmonary venous connection. Cardiol Young, 2007, 17(4): 342-353.
5. Ricci M, Elliott M, Cohen GA, et al. Management of pulmonary venous obstruction after correction of TAPVC: risk factors for adverse outcome. Eur J Cardiothorac Surg, 2003, 24(1): 28-36.
6. St Louis JD, Turk EM, Jacobs JP, et al. Type Ⅳ total anomalous pulmonary venous connection. World J Pediatr Congenit Heart Surg, 2017, 8(2): 142-147.
7. Zea-Vera R, Mery CM. Mixed total veins: the epitome of the few, the disparate, and the obscure. Semin Thorac Cardiovasc Surg, 2017, 29(3): 345-346.
8. Furlanetto G, Furlanetto BH, Henriques SR, et al. Mixed type total anomalous pulmonary venous connection: early results and surgical techniques. World J Pediatr Congenit Heart Surg, 2015, 6(1): 26-32.
9. Shen Q, Pa M, Hu X, et al. Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection. Pediatr Radiol, 2013, 43(7): 827-835.
10. Shim H, Yang JH, Jun TG. Two-stage correction of type Ⅳ total anomalous pulmonary venous connection. J Cardiothorac Surg, 2017, 12(1): 54.
11. Alsoufi B. Cracking the mystery of pulmonary vein stenosis. Semin Thorac Cardiovasc Surg, 2019, 31(2): 274-276.
12. Yong MS, Yaftian N, Weintraub RG, et al. Outcomes of surgery for mixed total anomalous pulmonary venous drainage in children. Semin Thorac Cardiovasc Surg, 2017, 29(3): 338-344.
13. Eyries M, Montani D, Girerd B, et al. EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension. Nat Genet, 2014, 46(1): 65-69.
14. Honjo O, Atlin CR, Hamilton BC, et al. Primary sutureless repair for infants with mixed total anomalous pulmonary venous drainage. Ann Thorac Surg, 2010, 90(3): 862-868.
15. 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.

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Surgical treatment of mixed total anomalous pulmonary venous connection

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