Surgical treatment strategy and outcome of functional univentricular heart with total anomalous pulmonary vein drainage_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

DU Xinwei , SUN Yanjun , ZHU Zhongqun , ZHU Hongbin , ZHENG Jinghao , ZHANG Haibo , XU Zhiwei ,  WANG Shunming

Department of Cardiac and Thoracic Surgery, Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai, 200127, P.R.China;

Corresponding author：

WANG Shunming, Email: wangshunmin@scmc.com.cn

Keywords：

Functional single ventricle; anomalous pulmonary vein drainage; pulmonary vein obstruction

DOI：

10.7507/1007-4848.201710067

Video：

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Objective To explore the surgical management method and outcome of functional univentricular heart with total anomalous pulmonary vein drainage (UVH-TAPVD). Methods We reviewed the surgical procedures and results for 44 UVH-TAPVD patients in our hospital between the year 2010 and 2016. There were 34 males and 10 females. The age of the patients was 312 (77-4 220) d when they accepted the first surgical treatment. Results There were 8 deaths in stage Ⅰ palliation, 1 death in stage Ⅱ palliation and 5 deaths during the follow-up. The overall survival rate was 68.2% (30/44). Glenn operation was undertaken in 34 patients with 5 deaths. Fontan operation was undertaken in 9 patients with 2 deaths. Conclusion Surgical strategies for UVH-TAPVD should be planned according to different anatomical and pathophysiological conditions in different patients. Staged palliations can reduce mortality and morbidity. But pulmonary venous obstruction and heterotaxy syndrome are still risk factors for these patients.

Citation： DU Xinwei, SUN Yanjun, ZHU Zhongqun, ZHU Hongbin, ZHENG Jinghao, ZHANG Haibo, XU Zhiwei, WANG Shunming. Surgical treatment strategy and outcome of functional univentricular heart with total anomalous pulmonary vein drainage. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2018, 25(2): 118-122. doi: 10.7507/1007-4848.201710067 Copy

1.	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.
2.	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.
3.	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.
4.	Seale AN, Uemura H, Webber SA, et al. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation, 2010, 122(25): 2718-2726.
5.	Morales DL, Braud BE, Booth JH, et al. Heterotaxy patients with total anomalous pulmonary venous return: improving surgical results. Ann Thorac Surg, 2006, 82(5): 1621-1627.
6.	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.
7.	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): 2134-2139.
8.	Foerster SR, Gauvreau K, McElhinney DB, et al. Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome. Pediatr Cardiol, 2008, 29(3): 536-544.
9.	Song J, Kang IS, Huh J, et al. Interstage mortality for functional single ventricle with heterotaxy syndrome: a retrospective study of the clinical experience of a single tertiary center. J Cardiothorac Surg, 2013, 8: 93.
10.	Rubino M, Van Praagh S, Kadoba K, et al. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg, 1995, 110(3): 641-650.
11.	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.
12.	Serraf A, Bensari N, Houyel L, et al. Surgical management of congenital heart defects associated with heterotaxy syndrome. Eur J Cardiothorac Surg, 2010, 38(6): 721-727.
13.	Yu DC, Thiagarajan RR, Laussen PC, et al. Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation. J Pediatr Surg, 2009, 44(6): 1089-1095.
14.	Abbas PI, Dickerson HA, Wesson DE. Evaluating a management strategy for malrotation in heterotaxy patients. J Pediatr Surg, 2016, 51(5): 859-862.
15.	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.

1. 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.
2. 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.
3. 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.
4. Seale AN, Uemura H, Webber SA, et al. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation, 2010, 122(25): 2718-2726.
5. Morales DL, Braud BE, Booth JH, et al. Heterotaxy patients with total anomalous pulmonary venous return: improving surgical results. Ann Thorac Surg, 2006, 82(5): 1621-1627.
6. 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.
7. 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): 2134-2139.
8. Foerster SR, Gauvreau K, McElhinney DB, et al. Importance of totally anomalous pulmonary venous connection and postoperative pulmonary vein stenosis in outcomes of heterotaxy syndrome. Pediatr Cardiol, 2008, 29(3): 536-544.
9. Song J, Kang IS, Huh J, et al. Interstage mortality for functional single ventricle with heterotaxy syndrome: a retrospective study of the clinical experience of a single tertiary center. J Cardiothorac Surg, 2013, 8: 93.
10. Rubino M, Van Praagh S, Kadoba K, et al. Systemic and pulmonary venous connections in visceral heterotaxy with asplenia. Diagnostic and surgical considerations based on seventy-two autopsied cases. J Thorac Cardiovasc Surg, 1995, 110(3): 641-650.
11. 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.
12. Serraf A, Bensari N, Houyel L, et al. Surgical management of congenital heart defects associated with heterotaxy syndrome. Eur J Cardiothorac Surg, 2010, 38(6): 721-727.
13. Yu DC, Thiagarajan RR, Laussen PC, et al. Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation. J Pediatr Surg, 2009, 44(6): 1089-1095.
14. Abbas PI, Dickerson HA, Wesson DE. Evaluating a management strategy for malrotation in heterotaxy patients. J Pediatr Surg, 2016, 51(5): 859-862.
15. 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.

Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Surgical treatment strategy and outcome of functional univentricular heart with total anomalous pulmonary vein drainage

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