Staged surgical results of functional single ventricle anomalies with pulmonary hypertension_Chinese Journal of Clinical Thoracic and Cardiovascular Surgery

Authors：

PAN Yanjun , HE Xiaomin , CHEN Huiwen , ZHENG Jinghao , ZHANG Haibo ,  ZHU Zhongqun

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

Corresponding author：

ZHU Zhongqun, Email: zzqheart@aliyun.com

Keywords：

Functional single ventricle anomalies; pulmonary hypertension; staged operations

DOI：

10.7507/1007-4848.201710066

Video：

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Objective To explore current results after staged operations in patients with functional single ventricle anomalies and pulmonary hypertension. Methods We retrospectively analyzed the clinical data of 129 patients with functional single ventricle anomalies and pulmonary hypertension undergoing pulmonary artery banding in our hospital between April 2008 and December 2015. There were 81 males and 48 females. There were 71 patients with double outlet of right ventricle, 17 patients with tricuspid atresia, 7 patients with transposition of great arteries, 33 patients with uni-ventricular heart, and one patient with complete atrio-ventricular septal defect. The surgical results, transition to Glenn procedure and subsequent transition to Fontan procedure were analyzed. Results The 129 patients underwent 159 operations of pulmonary artery banding totally. Hospital mortality was 4.7% (6/129). Nine patients were lost to follow-up. Eighty-seven (67.4%) patients underwent the second-stage Glenn procedure, and 43 patients(33.3%) underwent third-stage Fontan procedure. Two patients died after Glenn and 3 patients died after Fontan separately. There were 32 patients who accompanied with coarctation, interruption of aortic arch, heterotaxy, total anomalous pulmonary venous connection or atrio-ventricular valve regurgitation. Fifteen (46.9%) patients succeeded in transition to Glenn, and 6 (18.8%) patients succeeded in transition to Fontan. Fourteen patients developed obstruction of left ventricular outlet tract or bulbo-ventricular foramen. Conclusion Early pulmonary artery banding is an acceptable strategy for patients with single ventricle anomalies and pulmonary hypertension. Outcomes and results of subsequent Glenn and Fontan procedures are generally good. Accompanied complex anomalies are risk factors for lower ratio of transition to Glenn and Fontan procedure.

Citation： PAN Yanjun, HE Xiaomin, CHEN Huiwen, ZHENG Jinghao, ZHANG Haibo, ZHU Zhongqun. Staged surgical results of functional single ventricle anomalies with pulmonary hypertension. Chinese Journal of Clinical Thoracic and Cardiovascular Surgery, 2018, 25(3): 183-187. doi: 10.7507/1007-4848.201710066 Copy

1.	徐志伟, 苏肇伉, 丁文祥. 功能性单心室的外科治疗. 中国胸心血管外科临床杂志, 2002, 9(2): 77-80.
2.	吴清玉. 单心室外科治疗中的问题与策略. 中南大学学报 (医学版), 2008, 33(12): 1071-1075.
3.	Bradley SM, Simsic JM, Atz AM, et al. The infant with single ventricle and excessive pulmonary blood flow: results of a strategy of pulmonary artery division and shunt. Ann Thorac Surg, 2002, 74(3): 805-810.
4.	Alsoufi B, Manlhiot C, Ehrlich A, et al. Results of palliation with an initial pulmonary artery band in patients with single ventricle associated with unrestricted pulmonary blood flow. J Thorac Cardiovasc Surg, 2015, 149(1): 213-220.
5.	Rodefeld MD, Ruzmetov M, Schamberger MS, et al. Staged surgical repair of functional single ventricle in infants with unobstructed pulmonary blood flow. Eur J Cardiothorac Surg, 2005, 27(6): 949-955.
6.	Alsoufi B, McCracken C, Shashidharan S, et al. Palliation outcomes of neonates born with single-ventricle anomalies associated with aortic arch obstruction. Ann Thorac Surg, 2017, 103(2): 637-644.
7.	Kajihara N, Asou T, Takeda Y, et al. Staged surgical approach in neonates with a functionally single ventricle and arch obstruction: pulmonary artery banding and aortic arch reconstruction before placement of a bidirectional cavopulmonary shunt in infants. Pediatr Cardiol, 2010, 31(1): 33-39.
8.	Lee MG, Brizard CP, Galati JC, et al. Outcomes of patients born with single-ventricle physiology and aortic arch obstruction: the 26-year Melbourne experience. J Thorac Cardiovasc Surg, 2014, 148(1): 194-201.
9.	陈纲, 贾兵, 陈张根, 等. 肺动脉环缩术在儿童复杂先天性心脏病中的应用. 中华小儿外科杂志, 2008, 29(2): 70-73.
10.	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.
11.	Hess SL, Bricker JT, Garson A Jr, et al. Pulmonary artery banding and subaortic stenosis in patients with single ventricle: surgical alternatives and clinical outcome. Tex Heart Inst J, 1992, 19(1): 15-20.
12.	Freedom RM, Benson LN, Smallhorn JF, et al. Subaortic stenosis, the univentricular heart, and banding of the pulmonary artery: an analysis of the courses of 43 patients with univentricular heart palliated by pulmonary artery banding. Circulation, 1986, 73(4): 758-764.
13.	Lacour-Gayet F. Management of older single functioning ventricles with outlet obstruction due to a restricted " VSD” in double inlet left ventricle and in complex double outlet right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2009, 130-132.
14.	Fiore AC, Rodefeld M, Vijay P, et al. Subaortic obstruction in univentricular heart: results using the double barrel Damus-Kaye Stansel operation. Eur J Cardiothorac Surg, 2009, 35(1): 141-146.
15.	Alsoufi B, Al-Wadai A, Khan M, et al. Outcomes of Damus-Kaye-Stansel anastomosis at time of cavopulmonary connection in single ventricle patients at risk of developing systemic ventricular outflow tract obstruction. Eur J Cardiothorac Surg, 2014, 45(1): 77-82.
16.	Clarke AJ, Kasahara S, Andrews DR, et al. Mid-term results for double inlet left ventricle and similar morphologies: timing of Damus-Kaye-Stansel. Ann Thorac Surg, 2004, 78(2): 650-657.
17.	Heinle JS, Carberry KE, McKenzie ED, et al. Outcomes after the palliative arterial switch operation in neonates with single-ventricle anatomy. Ann Thorac Surg, 2013, 95(1): 212-218.

1. 徐志伟, 苏肇伉, 丁文祥. 功能性单心室的外科治疗. 中国胸心血管外科临床杂志, 2002, 9(2): 77-80.
2. 吴清玉. 单心室外科治疗中的问题与策略. 中南大学学报 (医学版), 2008, 33(12): 1071-1075.
3. Bradley SM, Simsic JM, Atz AM, et al. The infant with single ventricle and excessive pulmonary blood flow: results of a strategy of pulmonary artery division and shunt. Ann Thorac Surg, 2002, 74(3): 805-810.
4. Alsoufi B, Manlhiot C, Ehrlich A, et al. Results of palliation with an initial pulmonary artery band in patients with single ventricle associated with unrestricted pulmonary blood flow. J Thorac Cardiovasc Surg, 2015, 149(1): 213-220.
5. Rodefeld MD, Ruzmetov M, Schamberger MS, et al. Staged surgical repair of functional single ventricle in infants with unobstructed pulmonary blood flow. Eur J Cardiothorac Surg, 2005, 27(6): 949-955.
6. Alsoufi B, McCracken C, Shashidharan S, et al. Palliation outcomes of neonates born with single-ventricle anomalies associated with aortic arch obstruction. Ann Thorac Surg, 2017, 103(2): 637-644.
7. Kajihara N, Asou T, Takeda Y, et al. Staged surgical approach in neonates with a functionally single ventricle and arch obstruction: pulmonary artery banding and aortic arch reconstruction before placement of a bidirectional cavopulmonary shunt in infants. Pediatr Cardiol, 2010, 31(1): 33-39.
8. Lee MG, Brizard CP, Galati JC, et al. Outcomes of patients born with single-ventricle physiology and aortic arch obstruction: the 26-year Melbourne experience. J Thorac Cardiovasc Surg, 2014, 148(1): 194-201.
9. 陈纲, 贾兵, 陈张根, 等. 肺动脉环缩术在儿童复杂先天性心脏病中的应用. 中华小儿外科杂志, 2008, 29(2): 70-73.
10. 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.
11. Hess SL, Bricker JT, Garson A Jr, et al. Pulmonary artery banding and subaortic stenosis in patients with single ventricle: surgical alternatives and clinical outcome. Tex Heart Inst J, 1992, 19(1): 15-20.
12. Freedom RM, Benson LN, Smallhorn JF, et al. Subaortic stenosis, the univentricular heart, and banding of the pulmonary artery: an analysis of the courses of 43 patients with univentricular heart palliated by pulmonary artery banding. Circulation, 1986, 73(4): 758-764.
13. Lacour-Gayet F. Management of older single functioning ventricles with outlet obstruction due to a restricted " VSD” in double inlet left ventricle and in complex double outlet right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu, 2009, 130-132.
14. Fiore AC, Rodefeld M, Vijay P, et al. Subaortic obstruction in univentricular heart: results using the double barrel Damus-Kaye Stansel operation. Eur J Cardiothorac Surg, 2009, 35(1): 141-146.
15. Alsoufi B, Al-Wadai A, Khan M, et al. Outcomes of Damus-Kaye-Stansel anastomosis at time of cavopulmonary connection in single ventricle patients at risk of developing systemic ventricular outflow tract obstruction. Eur J Cardiothorac Surg, 2014, 45(1): 77-82.
16. Clarke AJ, Kasahara S, Andrews DR, et al. Mid-term results for double inlet left ventricle and similar morphologies: timing of Damus-Kaye-Stansel. Ann Thorac Surg, 2004, 78(2): 650-657.
17. Heinle JS, Carberry KE, McKenzie ED, et al. Outcomes after the palliative arterial switch operation in neonates with single-ventricle anatomy. Ann Thorac Surg, 2013, 95(1): 212-218.

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